Tuesday, November 04, 2014

The pathological and molecular but not clinical phenotypes are maintained after second passage of experimental atypical bovine spongiform encephalopathy in cattle

The pathological and molecular but not clinical phenotypes are maintained after second passage of experimental atypical bovine spongiform encephalopathy in cattle
 
Timm Konold, Laura J Phelan, [...], and Marion M Simmons

 
Additional article information


Abstract Background Atypical bovine spongiform encephalopathies (BSEs), classified as H-type and L-type BSE based on the Western immunoblot profiles, are naturally occurring diseases in cattle, which are phenotypically different to classical BSE. Transmission studies in cattle using the intracerebral route resulted in disease where the phenotypes were maintained irrespective of BSE type but clinically affected cattle with a shorter survival time displayed a nervous form whereas cattle with a longer survival time displayed a dull form. A second transmission study is reported here where four cattle were intracerebrally inoculated with brain tissue from experimentally infected cattle presenting with either the nervous or dull form of H- or L-type BSE to determine whether the phenotype is maintained.

 
Results The four inoculated cattle were culled at 16.5-19.5 months post inoculation after presenting with difficulty getting up, a positive scratch response (all) and dullness (three cattle), which was not observed in two non-inoculated control cattle, each housed with either group of inoculated cattle. Only the inoculated cattle had detectable prion protein in the brain based on immunohistochemical examination, and the Western immunoblot profile was consistent with the H-type or L-type BSE of the respective donor cattle.

 

Conclusions Second passage of H-type and L-type BSE in cattle produced a TSE where the majority of cattle displayed the dull form regardless of clinical disease form of the donor cattle. The pathological and molecular phenotypes of H- and L-type BSE were maintained.

 

Electronic supplementary material The online version of this article (doi:10.1186/s12917-014-0243-2) contains supplementary material, which is available to authorized users.


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Conclusions Second passage of H-type and L-type BSE in cattle by intracerebral inoculation produced a TSE where the majority displayed the dull disease form regardless of disease form of the donor cattle. The pathological and molecular phenotypes of H- and L-type BSE were maintained.

 


 

 ISSN 1999-4915

 

www.mdpi.com/journal/viruses Article

 

Molecular Modeling of Prion Transmission to Humans

 

Etienne Levavasseur 1, Nicolas Privat 1, Juan-Carlos Espinosa Martin 2, Steve Simoneau 3, Thierry Baron 4, Benoit Flan 3, Juan-Maria Torres 2 and Stéphane Haïk 1,5,6,* 1 Inserm U 1127, CNRS UMR 7225, Sorbonne Universités, UPMC Univ. Paris 06 UMR S 1127, Institut du Cerveau et de la Moelle épinière, ICM, 75013 Paris, France; E-Mails: etienne.levavasseur@inserm.fr (E.L.); nicolas.privat@inserm.fr (N.P.) 2 Centro de Investigacion en Sanidad Animal, Carretera de Algete a El Casar, 28130 Madrid, Spain; E-Mails: espinosa.juan@inia.es (J.-C.E.M.); jmtorres@inia.es (J.-M.T.) 3 LFB Biomédicaments, 91958 Les Ulis, France; E-Mails: simoneaus@lfb.fr (S.S.); flan@lfb.fr (B.F.) 4 Agence nationale de sécurité sanitaire de l’alimentation, de l’environnement et du travail (ANSES), Unité Maladies neurodégénératives, 69394 Lyon, France; E-Mail: thierry.baron@anses.fr 5 AP-HP, Hôpital de la Pitié-Salpêtrière, Cellule nationale de référence des MCJ, F-75013 Paris, France 6 AP-HP, Hôpital de la Pitié-Salpêtrière, Neuropathologie, 75013 Paris, France * Author to whom correspondence should be addressed; E-Mail: stephane.haik@courriel.upmc.fr; Tel.: +33-142-162-628; Fax: +33-142-161-899.

 

External Editor: Judd Aiken and Debbie McKenzie

 

Received: 24 July 2014; in revised form: 26 September 2014/ Accepted: 30 September 2014/ Published: 02 October 2014

 

Abstract: Using different prion strains, such as the variant Creutzfeldt-Jakob disease agent and the atypical bovine spongiform encephalopathy agents, and using transgenic mice expressing human or bovine prion protein, we assessed the reliability of protein misfolding cyclic amplification (PMCA) to model interspecies and genetic barriers to prion transmission. We compared our PMCA results with in vivo transmission data characterized by attack rates, i.e., the percentage of inoculated mice that developed the disease. Using 19 seed/substrate combinations, we observed that a significant PMCA amplification was only obtained when the mouse line used as substrate is susceptible to the corresponding strain. Our results suggest that PMCA provides a useful tool to study genetic barriers to transmission and to study the zoonotic potential of emerging prion strains.

 

1. Introduction

 

Prion diseases are fatal transmissible disorders affecting humans and animals. They are characterized by brain vacuolization, neuronal loss and accumulation of PrPsc, an abnormal isoform of the host-encoded cellular prion protein (PrPc). PrPsc has been proposed as the infectious agent, capable of converting PrPc into PrPsc in an autocatalytical manner [1]. In humans, prion diseases result from contamination, genetic inheritance or sporadic event. The host susceptibility is influenced by the prion protein-encoding gene PRNP. For example, the variant of Creutzfeldt-Jakob disease (vCJD), which has been associated to the classical bovine spongiform encephalopathy (C-BSE) epidemics in cattle through contaminated meat product consumption [2], has occurred so far only in individuals homozygous for methionine at codon 129 of PRNP [3]. However, this finding has been under debate [4,5]. Studies have investigated interspecies and genotypic barriers [6,7] using an in vitro PrPsc amplification system named protein misfolding cyclic amplification (PMCA) [8]. This method allows, in PCR tubes, the amplification of minute amounts of PrPsc in infected tissues (seed) in the presence of normal brain homogenate in excess (substrate), after cycles of incubation and sonication. Then, the final product of the reaction can be detected after proteinase K digestion by Western blot. While PMCA allows the amplification of PrPsc, it has also been demonstrated that infectivity was increased during the reaction [9], and that prion strain properties were maintained throughout the reaction [10–12]. Brains from humans or transgenic mice expressing a human PrP with methionine at codon 129 of PRNP provided the best substrates to amplify vCJD and BSE PrPsc [6,7], suggesting that PMCA may reproduce faithfully the genotypic transmission barrier. It was thus proposed as a means to evaluate the zoonotic risk associated with emerging prion strains (Nor98 in sheep, L-type BSE in cattle). Indeed, while classical BSE strain has been recognized to be at the origin of vCJD in humans, L-BSE is considered to be a sporadic form of prion disease in cattle, differing in many aspects (epidemiology, neuropathology, biochemical features) from the C-BSE strain.

 

***Moreover, L-BSE has been transmitted more easily to transgenic mice overexpressing a human PrP [13,14] or to primates [15,16] than C-BSE.

 

***It has been suggested that some sporadic CJD subtypes in humans may result from an exposure to the L-BSE agent. Lending support to this hypothesis, pathological and biochemical similarities have been observed between L-BSE and an sCJD subtype (MV genotype at codon 129 of PRNP) [17], and between L-BSE infected non-human primate and another sCJD subtype (MM genotype) [15].

 

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***P.170: Potential detection of oral transmission of H type atypical BSE in cattle using in vitro conversion

 

Sandor Dudas, John G Gray, Renee Clark, and Stefanie Czub Canadian Food Inspection Agency; Lethbridge, AB Canada

 

Keywords: Atypical BSE, oral transmission, RT-QuIC

 

The detection of bovine spongiform encephalopathy (BSE) has had a significant negative impact on the cattle industry worldwide. In response, governments took actions to prevent transmission and additional threats to animal health and food safety. While these measures seem to be effective for controlling classical BSE, the more recently discovered atypical BSE has presented a new challenge. To generate data for risk assessment and control measures, we have challenged cattle orally with atypical BSE to determine transmissibility and mis-folded prion (PrPSc) tissue distribution. Upon presentation of clinical symptoms, animals were euthanized and tested for characteristic histopathological changes as well as PrPSc deposition.

 

The H-type challenged animal displayed vacuolation exclusively in rostral brain areas but the L-type challenged animal showed no evidence thereof. To our surprise, neither of the animals euthanized, which were displaying clinical signs indicative of BSE, showed conclusive mis-folded prion accumulation in the brain or gut using standard molecular or immunohistochemical assays. To confirm presence or absence of prion infectivity, we employed an optimized real-time quaking induced conversion (RT-QuIC) assay developed at the Rocky Mountain Laboratory, Hamilton, USA.

 

Detection of PrPSc was unsuccessful for brain samples tests from the orally inoculated L type animal using the RT-QuIC. It is possible that these negative results were related to the tissue sampling locations or that type specific optimization is needed to detect PrPSc in this animal. We were however able to consistently detect the presence of mis-folded prions in the brain of the H-type inoculated animal. Considering the negative and inconclusive results with other PrPSc detection methods, positive results using the optimized RT-QuIC suggests the method is extremely sensitive for H-type BSE detection. This may be evidence of the first successful oral transmission of H type atypical BSE in cattle and additional investigation of samples from these animals are ongoing.

 

P.169: PrPSc distribution in brain areas of a natural German H-type BSE case

 

Anne Balkema-Buschmann, Grit Priemer, Markus Keller, and Martin H Groschup Friedrich Loeffler Institut, Institute for Novel and Emerging Infectious Diseases; Greifswald, Insel Riems, Germany

 

Keywords: BSE H-type, brain, muscle

 

Ten years after the initial description of atypical BSE cases of the H-type and L-type, the distribution of PrPSc in different brain areas and peripheral tissues of natural cases of these BSE forms is still not fully understood. Intracerebral challenge experiments have been performed with both atypical BSE forms in cattle, and the distribution of the abnormal prion protein and infectivity has been analysed in a variety of tissues, confirming the general restriction to the central nervous system as it was already generally acknowledged for classical BSE, but showing a slightly earlier and stronger involvement of the peripheral nervous system and the skeletal muscle. www.landesbioscience.com Prion 105

 

However, data from cattle orally challenged with atypical BSE, which might mimic the natural situation, are not yet available. Unfortunately, for most natural cases of atypical BSE, only the obex region is available for further analysis. The PrPSc distribution in the brains of natural L-type BSE cases in Italy has been described in some detail, but comparably few such data are yet available for natural H-type cases. Here we describe the analysis of different brain areas and muscle samples of a natural H-type BSE case diagnosed in Germany in 2014, and compare these data with those obtained from the respective samples collected from cattle challenged intracerebrally with H-type BSE.

 

P.159: Transgenic mice overexpressing rabbit prion protein are susceptible to BSE, BASE and scrapie prion strains but resistant to CWD and atypical scrapie

 

Natalia Fernández-Borges,1 Enric Vidal,2 Belén Pintado,4 Hasier Eraña,1 Montserrat Ordóñez,3 Mercedes Márquez,5 Francesca Chianini,6 Dolors Fondevila,5 Manuel A Sánchez-Martín,7 Olivier Andréoletti,8 Mark P Dagleish,6 Martí Pumarola,5 and Joaquín Castilla1,3 1CIC bioGUNE; Parque tecnológico de Bizkaia; Derio; Bizkaia, Spain; 2Centre de Recerca en Sanitat Animal (CReSA); UAB-IR TA, Campus de la Universitat Autònoma de Barcelona; Bellaterra; Barcelona, Catalonia, Spain; 3IKERBASQUE; Basque Foundation for Science; Bilbao, Bizkaia, Spain; 4Centro Nacional de Biotecnología (CNB), Campus de Cantoblanco; Cantoblanco; Madrid, Spain; 5Department of Animal Medicine and Surgery; Veterinary faculty; Universitat Autònoma de Barcelona (UAB); Bellaterra (Cerdanyola del Vallès); Barcelona, Catalonia, Spain; 6Moredun Research Institute; Bush Loan, Penicuik, Scotland, UK; 7Unidad de Generación de OMGs. S.E.A. Department of Medicine; University of Salamanca; Salamanca, Spain; 8Ecole Nationale du Veterinaire; Service de Pathologie du Bétail; Toulouse, France

 

Interspecies transmission of prions is a well established phenomenon, both experimentally and in field conditions. Upon passage through new hosts prion strains have proven their capacity to change their properties. It is, in fact, a source of strain diversity which needs to be considered when assessing the potential risks associated with consumption of prion contaminated protein sources.

 

Rabbits were considered for decades a prion resistant species until proven recently otherwise. To determine the extent of rabbit susceptibility to prions and to assess their effects on the passage of different prion strains through this species, a transgenic mouse model overexpressing rabbit PrPC was developed (TgRab). Intracerebral challenges with prion strains originating from a variety of species including field isolates (SSBP1 scrapie, Nor98-like scrapie, BSE, BASE and CWD), experimental murine strains (ME7 and RML), experimentally obtained strains (sheepBSE) and strains obtained by in vitro crossing of the species barrier using saPMCA (BSE-RabPrPres, SSBP1-RabPrPres and CWD-RabPrPres) have been performed.

 

Interestingly, on first passage, TgRab were susceptible to the majority of prions tested with the exception of SSBP1 scrapie, CWD and Nor98 scrapie. Furthermore TgRab were capable of propagating strain-specific features such as differences in incubation periods, brain lesion and PrPd deposition profiles and PK resistant western blotting band patterns. Our results confirm previous studies shattering the myth that rabbits are resistant to prion infection and this should be taken into account when choosing protein sources to feed rabbits.

 

P.168: Evolution of the biological properties of L-BSE after passage in sheep with susceptible and resistant PrP genotypes

 

Michele A Di Bari, Umberto Agrimi, Claudia D’Agostino, Geraldina Riccardi, Stefano Marcon, Elena Esposito, Paolo Frassanito, Flavio Torriani, Shimon Simson, and Romolo Nonno Istituto Superiore di Sanità (ISS) Department of Veterinary Public Health and Food Safety; Rome, Italy

 

Background. Cattle L-BSE was efficiently transmitted to sheep with susceptible (QQ171) and resistant (QR171) PrP genotypes. 1 Notably, the PrPSc signature of L-BSE was preserved in QQ171 sheep but not in QR171 sheep.2 Notwithstanding, bioassay in transgenic mice expressing bovine or ovine (ARQ) PrPC showed that L-BSE strain was preserved in both, QQ171 and QR171 sheep-passaged L-BSE.3

 

Here we studied the biological properties of sheep-passaged L-BSE by bioassay in bank voles and transgenic mice expressing the ovine VRQ PrP (tg338), both characterized by a comparatively low susceptibility to cattle L-BSE.

 

Material and Methods. Voles and tg338 mice were intracerebrally inoculated with cattle L-BSE and sheep-passaged (QQ171 and QR171) L-BSE isolates. Survival time, lesion profiles, Pet-blot and WB analysis were used for strain typing. Results. Cattle L-BSE transmitted quite inefficiently to tg338 mice, with survival time >400 days post-infection (d.p.i.), while sheep-passaged inocula were much more efficient and all gave terminal disease by ~140 d.p.i. However, after sub-passage all inocula converged to a survival time of ~145 d.p.i.. and showed overlapping pathological phenotypes.

 

In voles, cattle L-BSE transmitted with very long survival times (~800 d.p.i.) and was accompanied by an upward shift of the PrPSc type. Again, all sheep-passaged L-BSE isolates transmitted much more efficiently, with similar survival times of ~360 d.p.i.. Upon second passage, three different strains were isolated in vole, characterized by distinct pathological phenotypes. This divergence is epitomized by the different survival times of vole-adapted L-BSE strains, which were ~400 d.p.i. for cattle L-BSE, ~130 d.p.i. for QQ171-passaged L-BSE and ~225 d.p.i. for QR171-passaged L-BSE.

 

Conclusions. These findings, along with previously published data,3 show that the original L-BSE strain was recovered after passage in sheep when bioassay was performed in animal models expressing bovine or ovine PrPC. In contrast, strain changes were observed in both, QQ171- and QR171-passaged L-BSE by bioassay in vole, a species with divergent PrP sequence compared to ruminants. Importantly, QQ171- and QR171-passaged L-BSE were characterised by different PrPSc types and, accordingly, showed different biological properties when transmitted to voles, but not when transmitted to other animal models.

 

Overall, our work support the hypothesis that prion isolates are likely composed of multiple prion components, emphasizes the role of host PrP polymorphisms on strain selection and mutation, and highlights the risk for new potentially zoonotic strains that could emerge from prion evolution in animal reservoirs.

 

P.172: BSE exposure risk from bovine intestine and mesentery

 

Fulvio Barizzone,1 Herbert Budka,2 Christine Fast,3 John N Griffin,4 Giuseppe Ru,5 Pietro Stella1 and Olivier Andréoletti6 1European Food Safety Authority; Parma, Italy; 2Institute of Neuropathology; University Hospital Zurich; Zurich, Switzerland; 3Friedrich-Loeffler-Institut; Institute of Novel and Emerging Infectious Diseases; Isle of Riems, Germany; 4Department of Agriculture, Food and the Marine; Backweston, Celbridge, Co. Kildare, Ireland; 5Istituto Zooprofilattico Sperimentale del Piemonte; Liguria e Valle d’Aosta; Biostatistics Epidemiology and Analysis of Risk (BEAR) unit; Turin, Italy; 6UMR Interactions Hôtes Agents Pathogènes; Ecole Nationale Vétérinaire INR A; ENVT; Toulouse, France

 

Keywords: Bovine Spongiform Encephalopathy (BSE), cattle, intestine, mesentery, specified risk material (SRM), quantitative risk assessment (QRA)

 

Bovine intestines and mesenteries in the European Union (EU) are considered among the tissues potentially containing the highest level of BSE infectivity and have to be removed from the food and feed chain. A quantitative assessment of the BSE infectious load potentially entering the food and feed chain yearly in the European Union (EU) was developed. The evolution of the BSE infectious titre and of the weight of the structures accumulating infectivity was considered. The number of BSE infected cattle entering undetected in the food and feed chain yearly was estimated. A model (TSEi) was developed to estimates the evolution of the BSE infectious load in animals and the total yearly infectious load that could enter the food and feed chain. In a BSE infected bovine, the distribution of infectivity in intestines and mesentery varies with the age. Up to 36 months of age the infectivity is mainly associated (on average more than 90%) with the last 4 metres of small intestine and the caecum, over 36 and under 60 months of age, there is an inter-individual variability, from 60 months of age the infectivity is mainly associated (on average more than 90%) with the mesenteric nerves and the celiac and mesenteric ganglion complex. The total amount of infectivity peaks, about 15 BoID50, in animals younger than 18 months, it declines to 8-9 BoID50 (24–48 months of age) and it drops to 0.7 BoID50 in animals older than 60 months. The ileocaecal plate is the most infectious part of the intestine and it can be used to estimate the potential maximum level of exposure for an individual consumer.

 

In the EU, between 2007 and 2012, the yearly amount of BSE infectivity associated with intestine and mesentery from animals entering the food and feed chain was reduced by a factor of 10 (from about 23,000 to about 2,000 BoID50).

 

However, the maximum level of exposure to the BSE agent from intestine remained stable (on average about 1.5-1.6 BoID50 per meter).

 

In case of re-emergence of BSE in the EU there would be an increase of the potential maximum level of exposure to BSE from intestine. According to the TSEi model the removal of the last four metres of the small intestine and of the caecum from the food and feed chain would result in a major reduction of the BSE exposure risk associated with intestine and mesentery in cattle.

 

P.131: Transmission of sheep-bovine spongiform encephalopathy in pigs

 

Carlos Hedman,1 Belén Marín,1 Fabian Corbière,3 Hicham Filali,1 Francisco Vázquez, José Luis Pitarch,1 William Jirón,1 Rodrigo S Hernandez,1 Bernardino Moreno,1 Martí Pumarola,2 Olivier Andréoletti,3 Juan José Badiola,1 and Rosa Bolea1 1University of Zaragoza; Zaragoza, Spain; 2University of Barcelona; Barcelona, Spain; 3Institut National de la Recherche (INR A); Toulouse, France

 

Introduction. The transmissible spongiform encephalopathies (TSE) don´t occur in swine in natural conditions. However, the bovine spongiform encephalopathy (BSE) agent, inoculated by 3 simultaneous routes in pigs, is able to reproduce a neurological disease in these animals. On the other hand, the BSE agent after passage in sheep under experimental conditions (sheep- BSE) exhibits altered pathobiologic properties. This new agent is able to cross the cattle-pig transmission barrier more efficiently than BSE. The potential propagation of TSE in animals from the human food chain, including pigs, needs to be assessed regarding the risk for human infection by animals other than TSE-infected ruminants. The aim of this work was to determine the susceptibility of pigs to the Sheep-BSE agent and describe the pathological findings and PrPSc deposition in different tissues.

 

Material and Methods. Seven minipigs were challenged intracerebrally with sheep-BSE agent. Clinical observation and postmortem histopathology, immunohistochemistry (antibody 2G11) and Western blotting were performed on central nervous system (CNS), peripheral nervous system (PNS) and other tissues.

 

Results. One pig was culled in an early incubation stage, and remaining six were culled at the presence of clinical sings. Pigs developed a clinical disease with locomotor disorders in an average time of 23 months post inoculation, showing clinical findings in most of them earlier than those described in the BSE in pigs experimental infection. TSE wasn´t confirmed in the preclinical pig. In clinical pigs, the entire cerebral cortex showed severe neuropil vacuolation, extensive and severe vacuolar changes affecting the thalamus, hippocampus and cerebellum. PrPSc was found in CNS of all clinical pigs (6/6). Intracellular (intraneuronal and intraglial) and neuropil-associated PrPSc deposition was consistently observed in the brainstem, thalamus, and deeper layers of the cerebral cortex. Also, PrPSc was observed in PNS, mainly in the myenteric plexus and also in nerves belonging to the skeleton muscle. Moreover, the glycosylation profile showed a 3 band pattern with a predominant monoglycosylated band in positive pig samples.

 

This features concern on the potential risk of utilization of meat and bound meal of small ruminants in feeding pigs.

 

P.177: Elements modulating the prion species barrier and its passage consequences

 

Juan-Carlos Espinosa,1 Patricia Aguilar-Calvo,1 Ana Villa-Diaz,1 Olivier Andréoletti,2 and Juan María Torres1 1Centro de Investigación en Sanidad Animal (CISA-INI A); Valdeolmos, Madrid, Spain; 2UMR INR A-ENVT 1225; Interactions Hôte Agent Pathogène; École Nationale Vétérinaire de Toulouse; Toulouse, France

 

The phenotypic features of Transmissible Spongiform Encephalopathy (TSE) strains may be modified during passage across a species barrier. In this study we investigated the biochemical and biological characteristics of Bovine Spongiform Encephalopathy (BSE) infectious agent after transmission in both natural host species (cattle, sheep, pigs, and mice) and in transgenic mice overexpressing the corresponding cellular prion protein (PrPC) in comparison with other non-BSE related prions from the same species. After these passages, most characteristics of the BSE agent remained unchanged. BSE-derived agents only showed slight modifications in the biochemical properties of the accumulated PrPSc, which were demonstrated to be reversible upon re-inoculation into transgenic mice expressing bovine-PrPC. Transmission experiments in transgenic mice expressing bovine, porcine or human-PrP revealed that all BSE-derived agents were transmitted with no or a weak transmission barrier. In contrast, a high species barrier was observed for the non-BSE related prions that harboured an identical PrP amino acid sequence such as sheep-scrapie, mouse RML or human sCJD isolates, supporting the theory that the prion transmission barrier is modulated by strain properties (presumably conformation-dependent) rather than by PrP amino acid sequence differences between host and donor.

 

As identical results were observed with prions propagated either in natural hosts or in transgenic mouse models, we postulate that the species barrier and its passage consequences are uniquely governed by the host PrPC sequence and not influenced by the PrPC expression level or genetic factors other than the PrPC amino acid sequence. All these findings unequivocally demonstrate that the species barrier and its passage consequences are uniquely driven by the PrPC sequence, and not by other host genetic factors, demonstrating the validity of transgenic PrP animals as models for studies of the species barrier.

 

The results presented herein reinforce the idea that the BSE agent is highly promiscuous, infecting other species, maintaining its properties in the new species, and even increasing its capabilities to jump to other species including humans. These data are essential for the development of an accurate risk assessment for BSE.

 

P.150: Zoonotic potential of L-type BSE prions: A new prion disease in humans?

 

Emilie Jaumain,1 Stéphane Haïk,2 Isabelle Quadrio,3 Laetitia Herzog,1 Fabienne Reine,1 Armand Perret-Liaudet,3 Human Rezaei,1 Hubert Laude,1 Jean-Luc Vilotte,4 and Vincent Béringue1 1INR A (Institut National de la Recherche Agronomique); UR892; Virologie Immunologie Moléculaires; Jouy-en-Josas, France; 2IN SERM; Equipe maladie d’Alzheimer et maladies à Prions; CRicm; UMRS 1127; CNR S; UPMC. R.; ICM, Hôpital de la Salpêtrière; Paris, France; 3Neurobiologie, CMRR , Gériatrie, Hospices Civils de Lyon, Université Lyon 1-CNR S UMR5292-IN SERM U1028; Lyon, France; 3INR A; UMR1313; Génétique Animale et Biologie Intégrative; Jouy-en-Josas, France

 

Two novel prion strains, referred to as BSE-L and BSE-H, have been recognized in bovines through active prion surveillance programs, both being distinct from the epizootic, ‘classical’, BSE strain (C-BSE). Both H and L-types have been detected worldwide as rare cases occurring in aged animals. Like C-BSE prions, H- and L-types prions can propagate with relative ease in foreign species or in transgenic mouse lines expressing heterologous PrP sequences. A prion exhibiting biological properties similar to C-BSE agent sometimes emerged from these cross-species transmissions. Previously, L-type prions were shown to transmit to transgenic mice expressing human PrP with methionine at codon 129 with higher efficacy than C-BSE prions. Here, we examined whether L-type prions propagate without any apparent transmission barrier in these mice and whether such ‘humanised’ L-type prions share biological properties with CJD prions. L-type prions and a panel of human CJD cases with various genotypes at codon 129 and electrophoretic PrPres signatures were serially transmitted by intracerebral route to human PrP mice. The biological phenotypes induced by these agents were compared by all the standard methods currently used to distinguish between prion strains. At each passage, L-type prions were also transmitted back to bovine PrP mice to assess whether the agent has evolved upon passaging on the human PrP sequence. L-type prions transmitted to human PrP mice at 100% attack rate, without notable alteration in the mean incubation times over 5 passages. At each passage, ‘humanized’ L-type prions were able to transmit back to bovine PrP transgenic mice without apparent transmission barrier, as based on the survival time and the restoration of a L-type BSE phenotype. Comparison of mean incubation times on primary and subsequent passages in human PrP mice showed no overlap between L-type and sporadic CJD agents. While the electrophoretic signature and regional distribution of PrPres in L-type diseased mouse brains resembled that seen after transmission of MM2 CJD strain type, both agents exhibited distinct resistance of the associated PrPres molecules to protease denaturation.

 

In summary, L-type prions can be passaged on the human PrP sequence without any obvious transmission barrier. The phenotype obtained differs from the classical CJD prion types known so far. Careful extrapolation would suggest that the zoonotic transmission of this agent could establish a new prion disease type in humans.

 


 

Protein Structure and Biology

 


 

P.200: Clinical expression of BSE in mouse models is unrelated to hallmarks of prion diseases

 

Christelle Jas-Duval,1,2 Jacqueline Mikol,1 Sophie Luccantoni-Freire,1 Christine Defer,2 Jean-Jacques Huart,2 Paul Brown,1 Jean-Philippe Deslys,1 and Emmanuel E Comoy1 1CEA, Institute of Emerging Diseases and Innovative Therapies (iMETI), Division of Prions and Related Diseases (SEPIA); Fontenay-aux-Roses, France; 2EFS-Nord de France; Lille, France

 

Background. In 1997, we demonstrated that intracerebral inoculation of cattle BSE to C57Bl/6 mice induced neurological disease, even though half of the recipient animals were devoid of PrPres (Lasmezas et al., 1997). PrPres appeared with shortening incubation periods after subsequent passages, suggesting that detectable PrPres should be considered as an indicator of strain virulence rather than as a specific (and required) marker of prion diseases. Subsequent blood risk studies showed that cynomolgus macaques exposed to vCJD-contaminated blood products developed either classical vCJD or a novel neurological disease without detectable PrPres, In our continuing studies of this phenomenon, we now report the results of intravenous inoculations of several different wild-type mouse strains with bovine or primate BSE source material.

 

Materials and Methods. Brains from one bovine and one cynomolgus monkey showing clinical signs of BSE were sonicated and ultracentrifuged at 188,000 g for 1 hour. Resulting pellets and supernatants (equivalent to 2 mg of brain / mouse) were intravenously injected to PrP+/+ Swiss, PrP+/- and PrP+/+ C57Bl/6N mice, totalling 12 separate bioassays. PrPres was detected using conventional ELISA, western blotting and IHC procedures. Pathology was studied on formalin-fixed brain tissues.

 

Results. The transmission rate of BSE in those 12 experiments ranged from 0% (PrP+/- C57Bl/6N—cattle BSE—supernatant or pellet: no clinical sign, no spongiosis and no PrPres) to 100% (Swiss—primate BSE—supernatant or pellet: all mice exhibited clinical signs, spongiosis and PrPres). Overall, transmission was more efficient in (1) Swiss than C57Bl/6 mice, (2) PrP+/+ than PrP+/- mice, (3) primate than cattle BSE and (4) pellets than supernatant preparations. Among the 8 models exhibiting partial transmission ratios, 29 mice showed clinical neurological signs, of which only one-third (10) had detectable spongiosis and PrPres. The other animals exhibited only PrPres (7), only spongiosis (5) or neither (7).

 

Conclusion. Our results suggest that clinical neurotoxicity, spongiosis, and accumulation of PrPres are three interconnected but disparate phenomena. Spongiosis and PrPres are specific but not systematic hallmarks of the onset of prion diseases, notably upon first passage of non-adapted prion strains in a new host. These results question the universality of current human diagnostic criteria and the real prevalence of disease linked to BSE exposure.

 


 

***In addition, non-human primates are specifically susceptible for atypical BSE as demonstrated by an approximately 50% shortened incubation time for L-type BSE as compared to C-type. Considering the current scientific information available, it cannot be assumed that these different BSE types pose the same human health risks as C-type BSE or that these risks are mitigated by the same protective measures.

 


 

BSE prions propagate as either variant CJD-like or sporadic CJD-like prion strains in transgenic mice expressing human prion protein

 

*** Surprisingly, however, BSE transmission to these transgenic mice, in addition to producing a vCJD-like phenotype, can also result in a distinct molecular phenotype that is indistinguishable from that of sporadic CJD with PrPSc type 2.

 

These data suggest that more than one BSEderived prion strain might infect humans;

 

***it is therefore possible that some patients with a phenotype consistent with sporadic CJD may have a disease arising from BSE exposure.

 

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These studies further strengthen the evidence that vCJD is caused by a BSE-like prion strain.

 

Also, remarkably, the key neuropathological hallmark of vCJD, the presence of abundant florid PrP plaques, can be recapitulated on BSE or vCJD transmission to these mice.

 

***However, the most surprising aspect of the studies was the finding that an alternate pattern of disease can be induced in 129MM Tg35 mice from primary transmission of BSE, with a molecular phenotype indistinguishable from that of a subtype of sporadic CJD. This finding has important potential implications as it raises the possibility that some humans infected with BSE prions may develop a clinical disease indistinguishable from classical CJD associated with type 2 PrPSc. This is, in our experience, the commonest molecular sub-type of sporadic CJD. In this regard, it is of interest that the reported incidence of sporadic CJD has risen in the UK since the 1970s (Cousens et al., 1997)...

 


 

To date the OIE/WAHO assumes that the human and animal health standards set out in the BSE chapter for classical BSE (C-Type) applies to all forms of BSE which include the H-type and L-type atypical forms. This assumption is scientifically not completely justified and accumulating evidence suggests that this may in fact not be the case. Molecular characterization and the spatial distribution pattern of histopathologic lesions and immunohistochemistry (IHC) signals are used to identify and characterize atypical BSE. Both the L-type and H-type atypical cases display significant differences in the conformation and spatial accumulation of the disease associated prion protein (PrPSc) in brains of afflicted cattle. Transmission studies in bovine transgenic and wild type mouse models support that the atypical BSE types might be unique strains because they have different incubation times and lesion profiles when compared to C-type BSE. When L-type BSE was inoculated into ovine transgenic mice and Syrian hamster the resulting molecular fingerprint had changed, either in the first or a subsequent passage, from L-type into C-type BSE.

 

***In addition, non-human primates are specifically susceptible for atypical BSE as demonstrated by an approximately 50% shortened incubation time for L-type BSE as compared to C-type. Considering the current scientific information available, it cannot be assumed that these different BSE types pose the same human health risks as C-type BSE or that these risks are mitigated by the same protective measures.

 


 

-------- Original Message --------

 

Subject: re-BSE prions propagate as either variant CJD-like or sporadic CJD

 

Date: Thu, 28 Nov 2002 10:23:43 -0000

 

From: "Asante, Emmanuel A" e.asante@ic.ac.uk

 

To: "'flounder@wt.net'" flounder@wt.net

 

Dear Terry,

 

I have been asked by Professor Collinge to respond to your request. I am a Senior Scientist in the MRC Prion Unit and the lead author on the paper. I have attached a pdf copy of the paper for your attention.

 

Thank you for your interest in the paper.

 

In respect of your first question, the simple answer is, ***yes. As you will find in the paper, we have managed to associate the alternate phenotype to type 2 PrPSc, the commonest sporadic CJD. It is too early to be able to claim any further sub-classification in respect of Heidenhain variant CJD or Vicky Rimmer's version. It will take further studies, which are on-going, to establish if there are sub-types to our initial finding which we are now reporting. The main point of the paper is that, as well as leading to the expected new variant CJD phenotype, BSE transmission to the 129-methionine genotype can lead to an alternate phenotype which is indistinguishable from type 2 PrPSc.

 

I hope reading the paper will enlighten you more on the subject. If I can be of any further assistance please to not hesitate to ask. Best wishes.

 

Emmanuel Asante

 

<>

 

____________________________________

 

Dr. Emmanuel A Asante MRC Prion Unit & Neurogenetics Dept. Imperial College School of Medicine (St. Mary's) Norfolk Place, LONDON W2 1PG Tel: +44 (0)20 7594 3794 Fax: +44 (0)20 7706 3272 email: e.asante@ic.ac.uk (until 9/12/02) New e-mail: e.asante@prion.ucl.ac.uk (active from now)

 

____________________________________

 

Thursday, August 12, 2010

 

Seven main threats for the future linked to prions

 

First threat

 

The TSE road map defining the evolution of European policy for protection against prion diseases is based on a certain numbers of hypotheses some of which may turn out to be erroneous. In particular, a form of BSE (called atypical Bovine Spongiform Encephalopathy), recently identified by systematic testing in aged cattle without clinical signs, may be the origin of classical BSE and thus potentially constitute a reservoir, which may be impossible to eradicate if a sporadic origin is confirmed.

 

***Also, a link is suspected between atypical BSE and some apparently sporadic cases of Creutzfeldt-Jakob disease in humans. These atypical BSE cases constitute an unforeseen first threat that could sharply modify the European approach to prion diseases.

 

Second threat

 

snip...

 


 

Monday, October 10, 2011

 

EFSA Journal 2011 The European Response to BSE: A Success Story

 

snip...

 

EFSA and the European Centre for Disease Prevention and Control (ECDC) recently delivered a scientific opinion on any possible epidemiological or molecular association between TSEs in animals and humans (EFSA Panel on Biological Hazards (BIOHAZ) and ECDC, 2011). This opinion confirmed Classical BSE prions as the only TSE agents demonstrated to be zoonotic so far

 

*** but the possibility that a small proportion of human cases so far classified as "sporadic" CJD are of zoonotic origin could not be excluded. Moreover, transmission experiments to non-human primates suggest that some TSE agents in addition to Classical BSE prions in cattle (namely L-type Atypical BSE, Classical BSE in sheep, transmissible mink encephalopathy (TME) and chronic wasting disease (CWD) agents) might have zoonotic potential.

 

snip...

 


 


 

 

2014

 

***Moreover, L-BSE has been transmitted more easily to transgenic mice overexpressing a human PrP [13,14] or to primates [15,16] than C-BSE.

 

***It has been suggested that some sporadic CJD subtypes in humans may result from an exposure to the L-BSE agent. Lending support to this hypothesis, pathological and biochemical similarities have been observed between L-BSE and an sCJD subtype (MV genotype at codon 129 of PRNP) [17], and between L-BSE infected non-human primate and another sCJD subtype (MM genotype) [15].

 

snip...

 


 

 

Monday, June 23, 2014

 

PRION 2014 TYPICAL AND ATYPICAL BSE AND CJD REPORT UPDATES

 


 

Bovine Spongiform Encephalopathy BSE (typical and atypical strains) USDA

 

*** Saturday, November 2, 2013 ***

 

Exploring the risks of a putative transmission of BSE to new species

 


 

Wednesday, September 25, 2013

 

Presence of subclinical infection in gene-targeted human prion protein transgenic mice exposed to atypical BSE

 


 

I ask Professor Kong ; Thursday, December 04, 2008 3:37 PM

 

Subject: RE: re--Chronic Wating Disease (CWD) and Bovine Spongiform Encephalopathies (BSE): Public Health Risk Assessment

 

''IS the h-BSE more virulent than typical BSE as well, or the same as cBSE, or less virulent than cBSE? just curious.....'' Professor Kong reply ;

 

.....snip

 

*** ''As to the H-BSE, we do not have sufficient data to say one way or another, ***but we have found that H-BSE can infect humans. I hope we could publish these data once the study is complete. Thanks for your interest.''

 

Best regards, Qingzhong Kong, PhD Associate Professor Department of Pathology Case Western Reserve University Cleveland, OH 44106 USA

 

END...TSS

 

Thursday, December 04, 2008 2:37 PM

 

"we have found that H-BSE can infect humans."

 

personal communication with Professor Kong. ...TSS

 

BSE-H is also transmissible in our humanized Tg mice. The possibility of more than two atypical BSE strains will be discussed.

 

Supported by NINDS NS052319, NIA AG14359, and NIH AI 77774.

 


 


 

please see below from PRION2013 ;

 

*** This study imply the possibility that the novel BSE prions with high virulence in cattle will be emerged during intraspecies transmission.

 

AD.56: The emergence of novel BSE prions by serial passages of H-type BSE in bovinized mice

 

Kentaro Masujin, Naoko Tabeta, Ritsuko Miwa, Kohtaro Miyazawa, Hiroyuki Okada, Shirou Mohri and Takashi Yokoyama National Institute of Animal Health; Tsukuba, Japan

 

H-type bovine spongiform encephalopathy (BSE) is an atypical form of BSE, and has been detected in several European countries, and North America. Transmission studies of H-type BSE led to the emergence of the classical BSE (C-BSE) phenotypes during passages in inbred wild type and bovinized PrP-overexpressing transgenic mice. In this study, we conducted serial passages of Canadian H-type BSE isolate in bovinized PrP-overexpressing transgenic mice (TgBoPrP). H-type BSE isolate was transmitted to TgBoPrP with incubation periods of 320 ± 12.2 d at primary passage. The incubation period of 2nd and 3rd passage were constant (~= 220 d), no clear differences were observed in their biological and biochemical properties. However, at the forth passage, 2 different BSE phenotypes were confirmed; one is shorter survival times (109 ± 4 d) and the other is longer survival times. TgBoPrP mice with longer incubation period showed the H-type phenotype of PrPsc profile and pathology. However, those of shorter incubation period were different phenotypes from previously existed BSE prions (C-BSE, L-type BSE, and H-type BSE).

 

*** This study imply the possibility that the novel BSE prions with high virulence in cattle will be emerged during intraspecies transmission.

 


 


 

please see ;

 

Thursday, August 15, 2013

 

The emergence of novel BSE prions by serial passages of H-type BSE in bovinized mice

 


 

Saturday, August 14, 2010 BSE

 

Case Associated with Prion Protein Gene Mutation (g-h-BSEalabama) and VPSPr PRIONPATHY

 


 

Sunday, September 1, 2013

 

*** Evaluation of the Zoonotic Potential of Transmissible Mink Encephalopathy

 

We previously described the biochemical similarities between PrPres derived from L-BSE infected macaque and cortical MM2 sporadic CJD: those observations suggest a link between these two uncommon prion phenotypes in a primate model (it is to note that such a link has not been observed in other models less relevant from the human situation as hamsters or transgenic mice overexpressing ovine PrP [28]). We speculate that a group of related animal prion strains (L-BSE, c-BSE and TME) would have a zoonotic potential and lead to prion diseases in humans with a type 2 PrPres molecular signature (and more specifically type 2B for vCJD)

 

snip...

 

Together with previous experiments performed in ovinized and bovinized transgenic mice and hamsters [8,9] indicating similarities between TME and L-BSE, the data support the hypothesis that L-BSE could be the origin of the TME outbreaks in North America and Europe during the mid-1900s.

 


 

Saturday, November 2, 2013

 

*** APHIS Finalizes Bovine Import Regulations in Line with International Animal Health Standards while enhancing the spread of BSE TSE prion mad cow type disease around the Globe ***

 


 

Singeltary Response to USDA, and USDA RESPONSE TO SINGELTARY ON HARVARD BSE RISK ASSESSMENT

 

Owens, Julie

 

From: Terry S. Singeltary Sr. [flounder9@verizon.net]

 

Sent: Monday, July 24, 2006 1:09 PM

 

To: FSIS RegulationsComments

 

Subject: [Docket No. FSIS-2006-0011] FSIS Harvard Risk Assessment of Bovine Spongiform Encephalopathy (BSE) Page 1 of 98

 


 

FSIS, USDA, REPLY TO SINGELTARY

 


 

*** Singeltary reply ; Molecular, Biochemical and Genetic Characteristics of BSE in Canada Singeltary reply ;

 


 

10,000,000+ LBS. of PROHIBITED BANNED MAD COW FEED I.E. BLOOD LACED MBM IN COMMERCE USA 2007

 

Date: March 21, 2007 at 2:27 pm PST

 

RECALLS AND FIELD CORRECTIONS: VETERINARY MEDICINES -- CLASS II

 

PRODUCT

 

Bulk cattle feed made with recalled Darling's 85% Blood Meal, Flash Dried, Recall # V-024-2007

 

CODE

 

Cattle feed delivered between 01/12/2007 and 01/26/2007

 

RECALLING FIRM/MANUFACTURER

 

Pfeiffer, Arno, Inc, Greenbush, WI. by conversation on February 5, 2007.

 

Firm initiated recall is ongoing.

 

REASON

 

Blood meal used to make cattle feed was recalled because it was cross- contaminated with prohibited bovine meat and bone meal that had been manufactured on common equipment and labeling did not bear cautionary BSE statement.

 

VOLUME OF PRODUCT IN COMMERCE

 

42,090 lbs.

 

DISTRIBUTION

 

WI

 

___________________________________

 

PRODUCT

 

Custom dairy premix products: MNM ALL PURPOSE Pellet, HILLSIDE/CDL Prot- Buffer Meal, LEE, M.-CLOSE UP PX Pellet, HIGH DESERT/ GHC LACT Meal, TATARKA, M CUST PROT Meal, SUNRIDGE/CDL PROTEIN Blend, LOURENZO, K PVM DAIRY Meal, DOUBLE B DAIRY/GHC LAC Mineral, WEST PIONT/GHC CLOSEUP Mineral, WEST POINT/GHC LACT Meal, JENKS, J/COMPASS PROTEIN Meal, COPPINI - 8# SPECIAL DAIRY Mix, GULICK, L-LACT Meal (Bulk), TRIPLE J - PROTEIN/LACTATION, ROCK CREEK/GHC MILK Mineral, BETTENCOURT/GHC S.SIDE MK-MN, BETTENCOURT #1/GHC MILK MINR, V&C DAIRY/GHC LACT Meal, VEENSTRA, F/GHC LACT Meal, SMUTNY, A- BYPASS ML W/SMARTA, Recall # V-025-2007

 

CODE

 

The firm does not utilize a code - only shipping documentation with commodity and weights identified.

 

RECALLING FIRM/MANUFACTURER

 

Rangen, Inc, Buhl, ID, by letters on February 13 and 14, 2007. Firm initiated recall is complete.

 

REASON

 

Products manufactured from bulk feed containing blood meal that was cross contaminated with prohibited meat and bone meal and the labeling did not bear cautionary BSE statement.

 

VOLUME OF PRODUCT IN COMMERCE

 

9,997,976 lbs.

 

DISTRIBUTION

 

ID and NV

 

END OF ENFORCEMENT REPORT FOR MARCH 21, 2007

 


 

Sunday, December 15, 2013

 

*** FDA PART 589 -- SUBSTANCES PROHIBITED FROM USE IN ANIMAL FOOD OR FEED VIOLATIONS OFFICIAL ACTION INDICATED OIA UPDATE DECEMBER 2013 UPDATE ***

 


 

Tuesday, August 12, 2014

 

MAD COW USDA TSE PRION COVER UP or JUST IGNORANCE, for the record AUGUST 2014

 


 

Thursday, June 23, 2011

 

Experimental H-type bovine spongiform encephalopathy characterized by plaques and glial- and stellate-type prion protein deposits

 


 

Wednesday, April 25, 2012

 

4th MAD COW DISEASE U.S.A. CALIFORNIA ATYPICAL L-TYPE BSE 2012

 


 

 

Saturday, August 4, 2012

 

*** Final Feed Investigation Summary - California BSE Case - July 2012 (ATYPICAL L-TYPE BASE BSE)

 


 

Discussion: The C, L and H type BSE cases in Canada exhibit molecular characteristics similar to those described for classical and atypical BSE cases from Europe and Japan. *** This supports the theory that the importation of BSE contaminated feedstuff is the source of C-type BSE in Canada. *** It also suggests a similar cause or source for atypical BSE in these countries. ***

 

see page 176 of 201 pages...tss

 


 

*** Singeltary reply ; Molecular, Biochemical and Genetic Characteristics of BSE in Canada Singeltary reply ;

 


 

I think they still test the lowest possible by oie standards, to keep them in line for trade, which is about 40,000 a year, from 95 million or so...tss

 

USDA NEVER GOT STARTED GOOD BEFORE SHUTTING DOWN $$$

 

Why is USDA "only" testing 40,000 samples a year? USDA's surveillance strategy is to focus on the targeted populations where we are most likely to find disease if it is present. This is the most effective way to meet both OIE and our domestic surveillance standards. After completing our enhanced surveillance in 2006 and confirming that our BSE prevalence was very low, we concluded that 40,000 samples per year from these targeted, high risk populations would far exceed these standards. In fact, this sampling is ten times greater than OIE standards .

 


 

The total number of cattle and calves in the U.S. on January 1, 2014, was 87.73 million head, down 1.8% from January 2013 and 9.2% lower than at the last cyclical peak in 2007. This is the lowest January cattle inventory since 1951, _supposedly_, due to the drought, but I have my concern with that. the 2004 enhanced BSE surveillance program was shut down after finding two mad cows (atypical's), and in my opinion, that's why they shut it down. also, Dr. Paul Brown from the cdc said he trusted nothing from them after the Texas 2nd mad cow was covered up for months and months, on what was suppose to be a 48 hour turn around on testing. he said this ;

 

"Everything they did on the Texas cow makes everything USDA did before 2005 suspect," Brown said. (see link below).

 

the rest is history ;

 

An Arizona meat processing company and its owner pled guilty in February 2007 to charges of theft of Government funds, mail fraud, and wire fraud. The owner and his company defrauded the BSE Surveillance Program when they falsified BSE Surveillance Data Collection Forms and then submitted payment requests to USDA for the services. In addition to the targeted sample population (those cattle that were more than 30 months old or had other risk factors for BSE), the owner submitted to USDA, or caused to be submitted, BSE obex (brain stem) samples from healthy USDA-inspected cattle. As a result, the owner fraudulently received approximately $390,000. Sentencing is scheduled for May 2007.

 

snip...

 

Topics that will be covered in ongoing or planned reviews under Goal 1 include:

 

soundness of BSE maintenance sampling (APHIS),

 

implementation of Performance-Based Inspection System enhancements for specified risk material (SRM) violations and improved inspection controls over SRMs (FSIS and APHIS),

 

snip...

 

The findings and recommendations from these efforts will be covered in future semiannual reports as the relevant audits and investigations are completed.

 

4 USDA OIG SEMIANNUAL REPORT TO CONGRESS FY 2007 1st Half

 


 

Office of the United States Attorney District of Arizona FOR IMMEDIATE RELEASE For Information Contact Public Affairs February 16, 2007 WYN HORNBUCKLE Telephone: (602) 514-7625 Cell: (602) 525-2681

 

CORPORATION AND ITS PRESIDENT PLEAD GUILTY TO DEFRAUDING GOVERNMENT’S MAD COW DISEASE SURVEILLANCE PROGRAM

 

PHOENIX -- Farm Fresh Meats, Inc. and Roland Emerson Farabee, 55, of Maricopa, Arizona, pleaded guilty to stealing $390,000 in government funds, mail fraud and wire fraud, in federal district court in Phoenix. U.S. Attorney Daniel Knauss stated, “The integrity of the system that tests for mad cow disease relies upon the honest cooperation of enterprises like Farm Fresh Meats. Without that honest cooperation, consumers both in the U.S. and internationally are at risk.

 


 

WE can only hope that this is a single incident. BUT i have my doubts. I remember when the infamous TOKEN Purina Feed Mill in Texas was feeding up to 5.5 grams of potentially and probably tainted BANNED RUMINANT feed to cattle, and the FDA was bragging at the time that the amount of potentially BANNED product was so little and the cattle were so big ;

 

"It is important to note that the prohibited material was domestic in origin (therefore not likely to contain infected material because there is no evidence of BSE in U.S. cattle), fed at a very low level, and fed only once. The potential risk of BSE to such cattle is therefore exceedingly low, even if the feed were contaminated."

 


 

On Friday, April 30 th , the Food and Drug Administration learned that a cow with central nervous system symptoms had been killed and shipped to a processor for rendering into animal protein for use in animal feed. ... FDA's investigation showed that the animal in question had already been rendered into "meat and bone meal" (a type of protein animal feed). Over the weekend FDA was able to track down all the implicated material. That material is being held by the firm, which is cooperating fully with FDA.

 


 

WE now know all that was a lie. WE know that literally Thousands of TONS of BANNED and most likely tainted product is still going out to commerce. WE know now and we knew then that .005 to a gram was lethal. WE know that CWD infected deer and elk, scrapie infected sheep, BSE and BASE infected cattle have all been rendered and fed back to livestock (including cattle) for human and animal consumption.

 

Paul Brown, known and respected TSE scientist, former TSE expert for the CDC said he had ''absolutely no confidence in USDA tests before one year ago'', and this was on March 15, 2006 ;

 

"The fact the Texas cow showed up fairly clearly implied the existence of other undetected cases," Dr. Paul Brown, former medical director of the National Institutes of Health's Laboratory for Central Nervous System Studies and an expert on mad cow-like diseases, told United Press International. "The question was, 'How many?' and we still can't answer that."

 

Brown, who is preparing a scientific paper based on the latest two mad cow cases to estimate the maximum number of infected cows that occurred in the United States, said he has "absolutely no confidence in USDA tests before one year ago" because of the agency's reluctance to retest the Texas cow that initially tested positive.

 

USDA officials finally retested the cow and confirmed it was infected seven months later, but only at the insistence of the agency's inspector general.

 

"Everything they did on the Texas cow makes everything USDA did before 2005 suspect," Brown said. ...snip...end

 


 

CDC - Bovine Spongiform Encephalopathy and Variant Creutzfeldt ... Dr. Paul Brown is Senior Research Scientist in the Laboratory of Central Nervous System ... Address for correspondence: Paul Brown, Building 36, Room 4A-05, ...

 


 

PAUL BROWN COMMENT TO ME ON THIS ISSUE

 

Tuesday, September 12, 2006 11:10 AM

 

"Actually, Terry, I have been critical of the USDA handling of the mad cow issue for some years, and with Linda Detwiler and others sent lengthy detailed critiques and recommendations to both the USDA and the Canadian Food Agency."

 

OR, what the Honorable Phyllis Fong of the OIG found ;

 

Finding 2 Inherent Challenges in Identifying and Testing High-Risk Cattle Still Remain

 


 

*** Singeltary reply ; Molecular, Biochemical and Genetic Characteristics of BSE in Canada Singeltary reply ;

 


 

Tuesday, August 12, 2014

 

MAD COW USDA TSE PRION COVER UP or JUST IGNORANCE, for the record AUGUST 2014

 


 

Thursday, October 02, 2014

 

[Docket No. APHIS-2013-0064] Concurrence With OIE Risk Designations for Bovine Spongiform Encephalopathy

 


 

Saturday, August 14, 2010

 

BSE Case Associated with Prion Protein Gene Mutation (g-h-BSEalabama) and VPSPr PRIONPATHY

 


 

2009 UPDATE ON ALABAMA AND TEXAS MAD COWS 2005 and 2006

 


 

Transmissible Spongiform Encephalopathy TSE Prion Disease North America 2014

 

Transmissible Spongiform Encephalopathy TSE Prion Disease have now been discovered in a wide verity of species across North America. typical C-BSE, atypical L-type BASE BSE, atypical H-type BSE, atypical H-G BSE, of the bovine, typical and atypical Scrapie strains, in sheep and goats, with atypical Nor-98 Scrapie spreading coast to coast in about 5 years. Chronic Wasting Disease CWD in cervid is slowly spreading without any stopping it in Canada and the USA and now has mutated into many different strains. Transmissible Mink Encephalopathy TME outbreaks. These Transmissible Spongiform Encephalopathy TSE Prion Disease have been silently mutating and spreading in different species in North America for decades.

 

The USDA, FDA, et al have assured us of a robust Triple BSE TSE prion Firewall, of which we now know without a doubt, that it was nothing but ink on paper. Since the 1997 mad cow feed ban in the USA, literally tons and tons of banned mad cow feed has been put out into commerce, never to return, as late as December of 2013, serious, serious breaches in the FDA mad cow feed ban have been documented. The 2004 enhanced BSE surveillance program was so flawed, that one of the top TSE prion Scientist for the CDC, Dr. Paul Brown stated ; Brown, who is preparing a scientific paper based on the latest two mad cow cases to estimate the maximum number of infected cows that occurred in the United States, said he has "absolutely no confidence in USDA tests before one year ago" because of the agency's reluctance to retest the Texas cow that initially tested positive. see ; http://www.upi.com/Health_News/2006/03/15/Analysis-What-that-mad-cow-means/UPI-12841142465253/

 

The BSE surveillance and testing have also been proven to be flawed, and the GAO and OIG have both raised serious question as to just how flawed it has been (see GAO and OIG reports). North America has more documented TSE prion disease, in different documented species (excluding the Zoo BSE animals in the EU), then any other place on the Globe. This does not include the very likelihood that TSE prion disease in the domestic feline and canine have been exposed to high doses of the TSE prion disease vid pet food. To date, it’s still legal to include deer from cwd zone into pet food or deer food. Specified Risk Material i.e. SRM bans still being breach, as recently as just last month.

 

nvCJD or what they now call vCJD, another case documented in Texas last month, with very little information being released to the public on about this case? with still the same line of thought from federal officials, ‘it can’t happen here’, so another vCJD blamed on travel of a foreign animal disease from another country, while ignoring all the BSE TSE Prion risk factors we have here in the USA and Canada, and the time that this victim and others, do spend in the USA, and exposed to these risk factors, apparently do not count in any way with regard to risk factor. a flawed process of risk assessment.

 

sporadic CJD, along with new TSE prion disease in humans, of which the young are dying, of which long duration of illness from onset of symptoms to death have been documented, only to have a new name added to the pot of prion disease i.e. sporadic GSS, sporadic FFI, and or VPSPR. I only ponder how a familial type disease could be sporadic with no genetic link to any family member? when the USA is the only documented Country in the world to have documented two different cases of atypical H-type BSE, with one case being called atypical H-G BSE with the G meaning Genetic, with new science now showing that indeed atypical H-type BSE is very possible transmitted to cattle via oral transmission (Prion2014). sporadic CJD and VPSPR have been rising in Canada, USA, and the UK, with the same old excuse, better surveillance. You can only use that excuse for so many years, for so many decades, until one must conclude that CJD TSE prion cases are rising. a 48% incease in CJD in Canada is not just a blip or a reason of better surveillance, it is a mathematical rise in numbers. More and more we are seeing more humans exposed in various circumstance in the Hospital, Medical, Surgical arenas to the TSE Prion disease, and at the same time in North America, more and more humans are becoming exposed to the TSE prion disease via consumption of the TSE prion via deer and elk, cattle, sheep and goats, and for those that are exposed via or consumption, go on to further expose many others via the iatrogenic modes of transmission of the TSE prion disease i.e. friendly fire. I pondered this mode of transmission via the victims of sporadic FFI, sporadic GSS, could this be a iatrogenic event from someone sub-clinical with sFFI or sGSS ? what if?

 

Two decades have passed since Dr. Ironside first confirmed his first ten nvCJD victims in 1995. Ten years later, 2005, we had Dr. Gambetti and his first ten i.e. VPSPR in younger victims. now we know that indeed VPSPR is transmissible. yet all these TSE prion disease and victims in the USA and Canada are being pawned off as a spontaneous event, yet science has shown, the spontaneous theory has never been proven in any natural case of TSE prion disease, and scientist have warned, that they have now linked some sporadic CJD cases to atypical BSE, to atypical Scrapie, and to CWD, yet we don’t here about this in the public domain. We must make all human and animal TSE prion disease reportable in every age group, in ever state and internationally, we must have a serious re-evaluation and testing of the USA cattle herds, and we must ban interstate movement of all cervids. Any voluntary effort to do any of this will fail. Folks, we have let the industry run science far too long with regards to the TSE prion disease. While the industry and their lobbyist continues to funnel junk science to our decision policy makers, Rome burns. ...end

 

REFERENCES

 

[all scientific peer review studies and other scientific information I have put into blogs, to shorten reference data. I DO NOT advertise or make money from this, this information is for education use...lost my mom to the hvCJD, and just made a promise, never forget, and never let them forget. ...TSS]

 

Sunday, June 29, 2014

 

Transmissible Spongiform Encephalopathy TSE Prion Disease North America 2014

 


 

Monday, November 3, 2014

 

USA CJD TSE PRION UNIT, TEXAS, SURVEILLANCE UPDATE NOVEMBER 2014

 

National Prion Disease Pathology Surveillance Center Cases Examined1 (October 7, 2014)

 

***6 Includes 11 cases in which the diagnosis is pending, and 19 inconclusive cases; ***7 Includes 12 (11 from 2014) cases with type determination pending in which the diagnosis of vCJD has been excluded. ***The sporadic cases include 2660 cases of sporadic Creutzfeldt-Jakob disease (sCJD),

 

***50 cases of Variably Protease-Sensitive Prionopathy (VPSPr)

 

***and 21 cases of sporadic Fatal Insomnia (sFI).

 


 

Monday, November 3, 2014

 

The prion protein protease sensitivity, stability and seeding activity in variably protease sensitive prionopathy brain tissue suggests molecular overlaps with sporadic Creutzfeldt-Jakob disease

 


 


 

Saturday, August 14, 2010 BSE

 

Case Associated with Prion Protein Gene Mutation (g-h-BSEalabama) and VPSPr PRIONPATHY

 


 

CONFIRMED HUMAN BSE AKA MAD COW DISEASE vCJD TEXAS USA

 

CONFIRMED HUMAN BSE AKA MAD COW DISEASE vCJD TEXAS USA

 

Monday, June 02, 2014 Confirmed Variant CJD Case in Texas

 


 

SO, 4 months after the fact and still no word on this case. no information what so ever. the silence is deafening $$$

 

TSS

Thursday, October 02, 2014

[Docket No. APHIS-2013-0064] Concurrence With OIE Risk Designations for Bovine Spongiform Encephalopathy

[Federal Register Volume 79, Number 190 (Wednesday, October 1, 2014)] [Notices] [Pages 59207-59208] From the Federal Register Online via the Government Printing Office [www.gpo.gov] [FR Doc No: 2014-23407]
 
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[[Page 59207]]
 
 DEPARTMENT OF AGRICULTURE
 
Animal and Plant Health Inspection Service
 
[Docket No. APHIS-2013-0064]
 
Concurrence With OIE Risk Designations for Bovine Spongiform Encephalopathy
 
AGENCY: Animal and Plant Health Inspection Service, USDA.
 
ACTION: Notice.
 
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SUMMARY: We are advising the public of our decision to concur with the World Organization for Animal Health's (OIE) bovine spongiform encephalopathy (BSE) risk designations for 15 regions. The OIE recognizes these regions as being of either negligible risk for BSE or of controlled risk for BSE. We are taking this action based on our review of information supporting the OIE's risk designations for these regions.
 
FOR FURTHER INFORMATION CONTACT: Dr. Silvia Kreindel, Senior Staff Veterinarian, Regionalization Evaluation Services, National Import Export Services, VS, APHIS, 4700 River Road Unit 38, Riverdale, MD 20737-1231; (301) 851-3300.
 
SUPPLEMENTARY INFORMATION: The regulations in 9 CFR part 92 subpart B, ``Importation of Animals and Animal Products; Procedures for Requesting BSE Risk Status Classification With Regard to Bovines'' (referred to below as the regulations), set forth the process by which the Animal and Plant Health Inspection Service (APHIS) classifies regions for bovine spongiform encephalopathy (BSE) risk. Section 92.5 of the regulations provides that all countries of the world are considered by APHIS to be in one of three BSE risk categories: Negligible risk, controlled risk, or undetermined risk. These risk categories are defined in Sec. 92.1. Any region that is not classified by APHIS as presenting either negligible risk or controlled risk for BSE is considered to present an undetermined risk. The list of those regions classified by APHIS as having either negligible risk or controlled risk can be accessed on the APHIS Web site at http://www.aphis.usda.gov/ importexport/animals/ animaldiseasestatus.shtml. The list can also be obtained by writing to APHIS at National Import Export Services, 4700 River Road Unit 38, Riverdale, MD 20737. Under the regulations, APHIS may classify a region for BSE in one of two ways. One way is for countries that have not received a risk classification from the World Organization for Animal Health (OIE) to request classification by APHIS. The other way is for APHIS to concur with the classification given to a country by the OIE. If the OIE has recognized a country as either BSE negligible risk or BSE controlled risk, APHIS will seek information to support our concurrence with the OIE classification. This information may be publicly available information, or APHIS may request that countries supply the same information given to the OIE. APHIS will announce in the Federal Register, subject to public comment, its intent to concur with an OIE classification. In accordance with that process, we published a notice \1\ in the Federal Register on December 4, 2013 (78 FR 72859-72860, Docket No. APHIS-2013-0064), in which we announced our intent to concur with the OIE risk designations for 15 regions. In the notice we mistakenly stated that we intended to concur with the risk designations for 14 regions; the correct number is 15. The regions listed in the notice, however, were correct. The OIE recognizes these regions as being of either negligible risk for BSE or of controlled risk for BSE. We solicited comments on the notice for 60 days ending on February 3, 2014. We received three comments by that date, from two private citizens and a foreign industry association. ---------------------------------------------------------------------------
 
 \1\ To view the notice and the comments we received, go to http://www.regulations.gov/#!docketDetail;D=APHIS-2013-0064. ---------------------------------------------------------------------------
 
 One commenter expressed general concern that the risk designations did not accurately reflect the actual risk of BSE, but the commenter did not address the specific details of the OIE process or of any region's designation. Another commenter expressed concern that the OIE process is not transparent and there is insufficient detail in the OIE summaries to make an adequate determination of BSE risk. This commenter stated that APHIS should undertake its own assessment of BSE status rather than accepting the OIE risk designation. The summaries are the only information the OIE makes publicly available. Countries may make their BSE dossiers publicly available, in whole or in part, or they may share their dossiers with other countries upon request. For this reason, before announcing our intent to concur with the OIE classification, APHIS verifies that the information can be provided to us, or is publicly available, for review to support our concurrence with the OIE classification. APHIS' intention is to follow the OIE's BSE guidelines while ensuring that OIE-recognized countries apply adequate BSE risk mitigation measures assuring that bovines and bovine commodities destined for export pose a negligible risk for BSE, and that the country complies with OIE requirements for the specific BSE country recognition. If the information is not publicly available and the country does not provide the information, then we will not recognize the country's BSE status. APHIS thus has greater confidence in the outcomes of the evaluations and will have the necessary documentation to support or defend recognition decisions. The process we use is described in the regulations in Sec. 92.5. The information provided in the OIE dossier is more comprehensive than what appears in the summaries of the OIE Scientific Commission, and includes information about the likelihood that the disease could have been introduced into the country though the importation of bovine or bovine commodities in the last 7 years, the likelihood that the agent could have been recycled in as meat-and-bone meal or greaves for the last 8 years, the awareness, notification and laboratory capabilities of the region, BSE surveillance in the region, and the history of BSE in the region. One commenter stated that, according to the OIE summary reports, the evaluation for Brazil was provided by the OIE in February 2012. The commenter also stated that in December
 
[[Page 59208]]
 
2012, it was learned that a cow from Brazil that was sampled for testing in December 2010 tested positive for BSE. The commenter noted that immunohistochemistry (IHC) tests were not completed until June 2012, and it was another 6 months before a confirmatory test was completed at the Community Reference Laboratory in Weybridge, United Kingdom. The commenter stated that the lack of specific information regarding the OIE evaluation of the surveillance system made it difficult to determine if this was a one-time error or a failure of the system. APHIS agrees that the delays in the testing and reporting of the atypical BSE case detected in Brazil were problematic. In response to these concerns, the OIE Scientific Commission requested that Brazil provide all relevant information for their meeting in February 2013. At that meeting, the OIE Scientific Commission affirmed that the identification of this single case of BSE did not put Brazil's or its trading partners' animal and public health at risk because the animal was destroyed and no parts of it had entered the food or feed chain. However, the OIE was also concerned about the delay before Brazil sent the clinical samples for a confirmatory diagnosis and requested more detailed information on the procedures for processing samples and the improvement of the surveillance system in the country, so that they could further monitor compliance by Brazil with international standards.\2\ At a subsequent meeting in September 2013, the OIE assessed the additional information provided by Brazil.\3\ The OIE was satisfied with the evidence submitted but also concluded that Brazil should submit the results of the proficiency tests conducted for 2013 to the OIE as soon as they became available. ---------------------------------------------------------------------------
 
 \2\ The report of the OIE scientific commission meeting in February 2013 can be viewed at http://www.oie.int/fileadmin/Home/eng/InternationaStandardSetting/docs/pdf/SCAD/ASCADFeb2013.pdf. The discussion of the BSE case in Brazil appears on pages 13-14. \3\ The report of the OIE scientific commission meeting in September 2013 can be viewed at http://www.oie.int/fileadmin/Home/eng/InternationaStandardSetting/docs/pdf/SCAD/ASCADSept2013.pdf. The discussion of the BSE case in Brazil appears on page 7. ---------------------------------------------------------------------------
 
 In addition, representatives of APHIS and the United States Department of Agriculture's Food Safety and Inspection Service visited Brazil in February 2013 to evaluate the BSE laboratory infrastructure, emergency response, and BSE-related mitigations at the slaughter level. APHIS' review of the epidemiological and laboratory reports, including the report from the confirmatory tests conducted at Weybridge, shows that Brazil's first BSE case was most consistent with the atypical form of the disease. In addition, as a result of the delays in testing and reporting of this case, Brazil's Minist[eacute]rio da Agricultura, Pecu[aacute]ria e Abastecimento conducted audits of the laboratories to identify areas for change and improvement, and has implemented several new procedures to assure the timely testing of samples and reporting of results. Corrective actions include addition of a second lab to conduct IHC tests, expansion of testing capabilities to include Western Blot, and the development of an inter-laboratory data management system which will issue reports, record improper samples, and flag delays in sample receipt, completion, and notification of test results. Samples will be forwarded for IHC testing immediately after the immunofluorescence test for rabies is completed, rather than waiting for the animal inoculation tests to be completed. We note that Brazil detected a suspected case of BSE in a 12-year- old cow in April 2014. The Brazilian authorities carried out the required epidemiological investigation in accordance with OIE guidelines. In May 2014, tests at the OIE reference laboratory in Weybridge confirmed that it was an atypical case of BSE. Brazil still meets the criteria for a negligible risk region. In Article 11.5.3 of the Terrestrial Animal Health Code, the OIE requires, among other things, that if there has been an indigenous case of BSE in a region, every indigenous case was born more than 11 years ago. The cow in which BSE was detected was over 11 years of age. Therefore, this most recent case will not affect Brazil's negligible risk status. One commenter stated that India should be included in the list of regions of negligible risk for BSE. Our review of information in support of concurrence with the OIE designation for India is ongoing; we have requested the OIE dossier but have not yet received it. When our review is complete, if the findings support concurrence with the OIE designation, we will publish a notice in the Federal Register announcing our preliminary concurrence with the OIE's designation for India and provide the public with an opportunity to comment. One commenter stated that the United States should be included on this list of regions of negligible risk for BSE because some raw material may be exported from the United States and then reimported after processing abroad. When APHIS assesses the disease status of a region, it is to determine whether imports can be safely allowed from that region. For this reason we do not typically include the United States in the lists of regions recognized for any given disease status. In the event that raw material was exported for processing, we could allow it to be reimported under conditions that would be specified on the import permit. Therefore, in accordance with the regulations in Sec. 92.5, we are announcing our decision to concur with the OIE risk classifications of the following countries: Regions of negligible risk for BSE: Austria, Belgium, Brazil, Colombia, Israel, Italy, Japan, the Netherlands, Singapore, Slovenia. Regions of controlled risk for BSE: Bulgaria, Costa Rica, Croatia, Nicaragua, Taiwan.
 
 Authority: 7 U.S.C. 1622 and 8301-8317; 21 U.S.C. 136 and 136a; 31 U.S.C. 9701; 7 CFR 2.22, 2.80, and 371.4.
 
 Done in Washington, DC, this 26th day of September 2014. Kevin Shea, Administrator, Animal and Plant Health Inspection Service. [FR Doc. 2014-23407 Filed 9-30-14; 8:45 am] BILLING CODE 3410-34-P
 
 
From: Terry S. Singeltary Sr. Sent: Monday, September 29, 2014 9:28 PM To: Louis.Gagnon@fao.org Cc: FAO-Newsroom@fao.org ; kkm@onehealthinitiative.com ; globalhealth@hhs.gov Subject: FAO joins new global efforts targeting Ebola and other infectious diseases
 
Greetings Honorable FAO Director-General José Graziano da Silva et al,
 
I wish to kindly, and urgently address something I did not see in these reports, a long incubating, 100% fatal once clinical disease i.e. the Transmissible Spongiform Encephalopathy TSE Prion diseases aka mad cow type disease. They have been conveniently ignored, or covered up, take your pick, for way too long. I urge you to take further actions to address this issue as soon as possible. The INDUSTRY FRIENDLY trade agreements between Countries, via the OIE, is a sham, when considering the TSE prion disease, BSE, Scrapie, CWD, TME, FSE, and other animal TSE not yet documented such as canine spongiform encephalopathy CSE, and all the human TSE prion disease, and the many different names that follow, just making things much more complicated. the ramifications from friendly fire, or the iatrogenic modes from all the above is great. the longer global recognition, and surveillance there from is ignored, the worse it will get, and this is what the mad cow debacle has shown us, and please spare us what I term the UKBSEnvCJD only theory. that dog don’t hunt no more.
 
I lost my mother to the Heidenhain Variant of Creutzfeldt Jakob disease aka hvCJD aka sporadic CJD (just another name of the same disease), and no, please, how can a supposedly genetic TSE prion disease, such as FFI or GSS, suddenly become a sporadic disease, such as now sporadic FFI, or sporadic GSS, or VPSPr, but not be connected to any family genetically? please tell me this.
 
I have wasted almost 18 years of my life, daily, searching for answers, as are many others, to what the government calls a spontaneous event. ...that dog don’t hunt no more either.
 
THE TSE prion disease, aka mad cow type disease, they are more of a political disease than anything else$
 
I just mad a promise to mom, never forget, and never let them forget.
 
I submit the following information on the USA and it’s state of the TSE prion disease aka mad cow type disease. for what’s it worth, please use as you wish.
 
I kind submitt ;
 
FAO joins new global efforts targeting Ebola and other infectious diseases
 
In talks hosted by U.S. President Barack Obama, FAO Director-General outlines FAO's role in Global Health Security Agenda
 
Photo: ©FAO
 
FAO Director-General José Graziano da Silva speaks at the Global Health Security Summit at the White House.
 
26 September 2014, Washington, D.C. - FAO Director-General José Graziano da Silva today stressed the need for controls on animal health to help curb the spread of Ebola and other infectious diseases dangerous to humans, during discussions hosted by U.S. President Barack Obama.
 
The FAO chief joined leaders of the World Health Organization (WHO), the World Organisation for Animal Health (OIE) and representatives from more than 40 countries at the Global Health Security Agenda (GHSA) event held at the White House in Washington, D.C.
 
President Obama underscored the importance of dealing with the breakout of infectious diseases collectively, highlighting that in a world that is deeply interconnected, outbreaks have the potential to impact every country. "No nation can meet this challenge on its own. Nobody is that isolated," he said, stressing the need for global cooperation.
 
The U.S. government-initiated GHSA is an international partnership to strengthen health systems with the objective to prevent, detect and respond to emerging disease threats. It is estimated that 70 percent of new infectious diseases that have emerged in humans over recent decades have animal origin, mostly from wildlife.
 
Graziano da Silva underlined that "controlling zoonotic diseases and emerging threats at the human, animal and ecosystems interface needs an integrated and multidisciplinary approach that brings different sectors to work closely together to attain the health of people, animals and the environment."
 
He noted how this is echoed in the One Health agenda which has seen FAO, as part of its tripartite partnership (FAO-WHO-OIE), integrate this approach in its vision for sustainable livestock development "to attain a healthier and more prosperous world."
 
By focusing on prevention, countries can minimize loss of human life when diseases cross over from animal to human populations and thus become harder to manage - a fact illustrated by the current Ebola outbreak in West Africa, the FAO Director-General said.
 
He expressed "great concern" over the possible impact of this epidemic on "food security and livelihoods of affected communities, with a potential to cause long-term food insecurity in West Africa, as a result of prolonged disruption of crop harvesting and subsequent planting."
 
The FAO Director-General in his address also mentioned other recent emerging diseases of animal origin that affect humans such as H5N1 avian influenza, Severe Acute Respiratory Syndrome (SARS) and Middle East respiratory syndrome (MERS).
 
"There is a need to set up global preparedness, surveillance and response programmes," Graziano da Silva told participants at the White House event. He welcomed the GHSA's focus on prevention, detection and response, and noted how FAO shared this approach.
 
Graziano da Silva reiterated FAO's commitment, alongside WHO and OIE, to further support countries in tackling threats to health from animal sources. FAO works with country and regional partners to assist them to develop preparedness and contingency plans for animal health-related events, and these capacities serve both public health and food security aims.
 
FAO also contributes to health protection through its work on the Joint FAO/WHO Codex Alimentarius Commission, that provides guidance on food safety and public health taking into account the entire food chain.
 
The Director-General, in his address at the White House event, emphasized the strong link between nutrition and human health. He noted how the upcoming Second International Conference on Nutrition (ICN2), jointly organized by FAO and WHO, will establish a framework of actions on nutrition that will be part of the Post-2015 Agenda.
 
 
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Any allied health scientist, physician, osteopath, veterinarian or interested individual citizen may be added to one and/or both lists by contacting us at kkm@onehealthinitiative.com c/o Contents Manager. Please include your curriculum vitae or brief biography, title, degree(s), affiliation, and address consistent with those currently acknowledged as "One Health/One Medicine" supporters. There are no obligations attached to joining this group and you may have your name removed at any time upon request.
 
The preparers of this message and the two lists act independently of any other entity or organization; however where feasible we attempt to augment and support those organizations’ efforts to recognize, promote and implement this initiative such as the American Academy of Pediatrics, American Association of Public Health Physicians, American College of Preventive Medicine, American Association of Veterinary Laboratory Diagnosticians, American Association of Wildlife Veterinarians, American College of Veterinary Microbiologists, American College of Veterinary Pathologists, American College of Veterinary Preventive Medicine, American Medical Association, American Nurses Association, American Physiological Society, American Phytopathological Society, American Society for Microbiology, American Society of Tropical Medicine and Hygiene, American Veterinary Medical Association, Association of Academic Health Centers, Association of American Medical Colleges, Association of American Veterinary Medical Colleges, Association of Schools of Public Health, Auburn University’s College of Veterinary Medicine, Auburn, Alabama (USA), Bella Moss Foundation, United Kingdom Biomedical Technology, Epidemiology and Food Safety Global Network: Brno, Czech Republic, CAB International (CABI) http://www.cabi.org, College of Veterinary Medicine and Biomedical Sciences, Texas A&M University (USA), Conservation through Public Health (CTPH) - http://www.ctph.org/index.php, Corporation Red SPVet, Bogota-Columbia, Council for Agricultural Science and Technology (CAST), Council of State and Territorial Epidemiologists, Croatian Society for Infectious Diseases, Delta Society, Department of Molecular and Comparative Pathobiology, Johns Hopkins University School of Medicine, Exuberant Animal, Federation of Veterinarians of Europe (FVE), Global Alliance for Rabies Control, Immuno Valley Consortium in The Netherlands, Indian Veterinary Public Health Association, Institute of Tropical Medicine, Department of Animal Health, Antwerp, Belgium, Italian Society of Preventive Medicine, National Association of State Public Health Veterinarians, National Environmental Health Association (NEHA), National Park Service (USA), New Zealand Centre for Conservation Medicine (NZCCM) – Auckland, Nigerian Biomedical and Life Scientists, Nigerian Veterinary Medical Association, One Health in Epidemiology, Massey University, New Zealand, Praecipio International, SAPUVET III Project, Silent Heroes Foundation, Society for Tropical Veterinary Medicine, SpayFIRST, Inc., State Environmental Health Directors, United States Animal Health Association (USAHA), Veterinarians without Borders/ Vétérinaires sans Frontières – Canada, Veterinarni Medicina, the international journal for biomedical and veterinary sciences http://vetmed.vri.cz/, Volunteers for Intercultural and Definitive Adventures (VIDA) - www.vidavolunteertravel.org, WiLDCOAST/COSTASALVAjE http://www.wildcoast.net/, Wildlife Disease Association www.wildlifedisease.org, World Association of Veterinary Laboratory Diagnosticians, and Zoonotic and Emerging Diseases, Edinburgh, UK.
 
This autonomous endeavor is maintained pro bono due to our firm conviction regarding the enormous value of the "One Health/One Medicine" concept. Thank you for your support.
 
Disclaimer
 
The views expressed in this One Health Initiative website do not necessarily represent those of the Kahn-Kaplan-Monath-Woodall-Conti One Health team or the general contents manager/editor Bruce Kaplan or ProMED contents manager/editor Jack Woodall. The truth of the information in the materials reproduced in this publication have not been independently verified by the Kahn-Kaplan-Monath-Woodall-Conti One Health team or the contents managers/editors and therefore the aforementioned individuals do not accept legal responsibility for the truth or accuracy of the information and accept no liability for the contents, or any consequences that may result from the use of any information contained herein. This provision is intended to exclude all participant parties from all liability for negligence in the reproduction of the materials set out herein.
 
Please note [February 9, 2012]: It has been brought to our attention that this One Health Initiative website material has been unethically expropriated and reproduced on another website domain without authorization. Be advised that there is NO OTHER individual, group, business or organization authorized to lay claim to information posted on the One Health Initiative website other than “One Health Initiative” by the One Health Initiative team of Laura H. Kahn, MD, MPH, MPP, Bruce Kaplan, DVM, Thomas P. Monath, MD, Jack Woodall, PhD and Lisa A. Conti, DVM, MPH.
 
Laura H Kahn, MD,MPH, MPP Bruce Kaplan, DVM Thomas P. Monath, MD
 
email: kkm@onehealthinitiative.com
 
 
>>> It is estimated that 70 percent of new infectious diseases that have emerged in humans over recent decades have animal origin, mostly from wildlife.
 
USA 4TH CASE VCJD (aka nvCJD) HUMAN MAD COW, THE SILENCE IS DEAFENING BSE, CWD, AND SCRAPIE TSE PRION DISEASE
 
Greetings DSHS, Dr. Fishcer, et al,
 
I know that most in the USA could care less about the CJD TSE prion disease aka mad cow type disease. but there are some of us here that will never forget.
 
you can cover up what ever you want. we all know. I have seen it happen too many times here in Texas with BSE TSE prion, either the typical or the atypical strains, or with the feed, or, with cwd, or scrapie as that goes, but we are still here, and we will never forget...
 
kind regards, terry
 
Creutzfeld-Jacob Disease (CJD) Emerging & Acute Infectious Disease Branch Michael Fischer
 
Marilyn Felkner
 
512-776-7676
 
512-776-7676
 
Chronic Wasting Disease Zoonosis Control Branch Eric Fonken
 
512-776-2155
 
Lab tests have confirmed a diagnosis of variant Creutzfeldt-Jakob Disease (CJD) in a patient who recently died in Texas. Variant CJD is a rare, fatal brain disorder, first described in 1996 in the United Kingdom and associated with beef consumption overseas.
 
This is the fourth case ever reported in the United States. In each of the three previous cases, infection likely occurred outside the United States, including the United Kingdom and Saudi Arabia. The history of this fourth patient includes extensive travel to Europe and the Middle East, and infection likely occurred outside the United States. The CDC and DSHS continue to investigate the case.
 
There are no Texas public health concerns or threats associated with this case.
 
 
 
 
Last updated June 02, 2014
 
 
CONFIRMED HUMAN BSE AKA MAD COW DISEASE vCJD TEXAS USA
 
CONFIRMED HUMAN BSE AKA MAD COW DISEASE vCJD TEXAS USA
 
Monday, June 02, 2014 Confirmed Variant CJD Case in Texas
 
 
SO, 4 months after the fact and still no word on this case. no information what so ever. the silence is deafening $$$
 
Transmissible Spongiform Encephalopathy TSE Prion Disease North America 2014
 
Transmissible Spongiform Encephalopathy TSE Prion Disease have now been discovered in a wide verity of species across North America. typical C-BSE, atypical L-type BASE BSE, atypical H-type BSE, atypical H-G BSE, of the bovine, typical and atypical Scrapie strains, in sheep and goats, with atypical Nor-98 Scrapie spreading coast to coast in about 5 years. Chronic Wasting Disease CWD in cervid is slowly spreading without any stopping it in Canada and the USA and now has mutated into many different strains. Transmissible Mink Encephalopathy TME outbreaks. These Transmissible Spongiform Encephalopathy TSE Prion Disease have been silently mutating and spreading in different species in North America for decades.
 
The USDA, FDA, et al have assured us of a robust Triple BSE TSE prion Firewall, of which we now know without a doubt, that it was nothing but ink on paper. Since the 1997 mad cow feed ban in the USA, literally tons and tons of banned mad cow feed has been put out into commerce, never to return, as late as December of 2013, serious, serious breaches in the FDA mad cow feed ban have been documented. The 2004 enhanced BSE surveillance program was so flawed, that one of the top TSE prion Scientist for the CDC, Dr. Paul Brown stated ; Brown, who is preparing a scientific paper based on the latest two mad cow cases to estimate the maximum number of infected cows that occurred in the United States, said he has "absolutely no confidence in USDA tests before one year ago" because of the agency's reluctance to retest the Texas cow that initially tested positive.
 
see ;
 
 
The BSE surveillance and testing have also been proven to be flawed, and the GAO and OIG have both raised serious question as to just how flawed it has been (see GAO and OIG reports). North America has more documented TSE prion disease, in different documented species (excluding the Zoo BSE animals in the EU), then any other place on the Globe. This does not include the very likelihood that TSE prion disease in the domestic feline and canine have been exposed to high doses of the TSE prion disease vid pet food. To date, it’s still legal to include deer from cwd zone into pet food or deer food. Specified Risk Material i.e. SRM bans still being breach, as recently as just last month.
 
nvCJD or what they now call vCJD, another case documented in Texas last month, with very little information being released to the public on about this case? with still the same line of thought from federal officials, ‘it can’t happen here’, so another vCJD blamed on travel of a foreign animal disease from another country, while ignoring all the BSE TSE Prion risk factors we have here in the USA and Canada, and the time that this victim and others, do spend in the USA, and exposed to these risk factors, apparently do not count in any way with regard to risk factor. a flawed process of risk assessment.
 
sporadic CJD, along with new TSE prion disease in humans, of which the young are dying, of which long duration of illness from onset of symptoms to death have been documented, only to have a new name added to the pot of prion disease i.e. sporadic GSS, sporadic FFI, and or VPSPR. I only ponder how a familial type disease could be sporadic with no genetic link to any family member? when the USA is the only documented Country in the world to have documented two different cases of atypical H-type BSE, with one case being called atypical H-G BSE with the G meaning Genetic, with new science now showing that indeed atypical H-type BSE is very possible transmitted to cattle via oral transmission (Prion2014). sporadic CJD and VPSPR have been rising in Canada, USA, and the UK, with the same old excuse, better surveillance. You can only use that excuse for so many years, for so many decades, until one must conclude that CJD TSE prion cases are rising. a 48% incease in CJD in Canada is not just a blip or a reason of better surveillance, it is a mathematical rise in numbers. More and more we are seeing more humans exposed in various circumstance in the Hospital, Medical, Surgical arenas to the TSE Prion disease, and at the same time in North America, more and more humans are becoming exposed to the TSE prion disease via consumption of the TSE prion via deer and elk, cattle, sheep and goats, and for those that are exposed via or consumption, go on to further expose many others via the iatrogenic modes of transmission of the TSE prion disease i.e. friendly fire. I pondered this mode of transmission via the victims of sporadic FFI, sporadic GSS, could this be a iatrogenic event from someone sub-clinical with sFFI or sGSS ? what if?
 
Two decades have passed since Dr. Ironside first confirmed his first ten nvCJD victims in 1995. Ten years later, 2005, we had Dr. Gambetti and his first ten i.e. VPSPR in younger victims. now we know that indeed VPSPR is transmissible. yet all these TSE prion disease and victims in the USA and Canada are being pawned off as a spontaneous event, yet science has shown, the spontaneous theory has never been proven in any natural case of TSE prion disease, and scientist have warned, that they have now linked some sporadic CJD cases to atypical BSE, to atypical Scrapie, and to CWD, yet we don’t here about this in the public domain. We must make all human and animal TSE prion disease reportable in every age group, in ever state and internationally, we must have a serious re-evaluation and testing of the USA cattle herds, and we must ban interstate movement of all cervids. Any voluntary effort to do any of this will fail. Folks, we have let the industry run science far too long with regards to the TSE prion disease. While the industry and their lobbyist continues to funnel junk science to our decision policy makers, Rome burns. ...end
 
REFERENCES
 
Sunday, June 29, 2014
 
Transmissible Spongiform Encephalopathy TSE Prion Disease North America 2014
 
 
Tuesday, August 12, 2014
 
MAD COW USDA TSE PRION COVER UP or JUST IGNORANCE, for the record AUGUST 2014
 
 
Singeltary Response to USDA, and USDA
 
RESPONSE TO SINGELTARY ON HARVARD BSE RISK ASSESSMENT
 
Owens, Julie
 
From: Terry S. Singeltary Sr. [flounder9@verizon.net]
 
Sent: Monday, July 24, 2006 1:09 PM
 
To: FSIS RegulationsComments
 
Subject: [Docket No. FSIS-2006-0011] FSIS Harvard Risk Assessment of Bovine Spongiform Encephalopathy (BSE) Page 1 of 98
 
 
FSIS, USDA, REPLY TO SINGELTARY
 
 
IT is of my opinion, that the OIE and the USDA et al, are the soul reason, and responsible parties, for Transmissible Spongiform Encephalopathy TSE prion diseases, including typical and atypical BSE, typical and atypical Scrapie, and all strains of CWD, and human TSE there from, spreading around the globe.
 
 
I have lost all confidence of this organization as a regulatory authority on animal disease, and consider it nothing more than a National Trading Brokerage for all strains of animal TSE, just to satisfy there commodity. AS i said before, OIE should hang up there jock strap now, since it appears they will buckle every time a country makes some political hay about trade protocol, commodities and futures. IF they are not going to be science based, they should do everyone a favor and dissolve there organization.
 
JUST because of low documented human body count with nvCJD and the long incubation periods, the lack of sound science being replaced by political and corporate science in relations with the fact that science has now linked some sporadic CJD with atypical BSE and atypical scrapie, and the very real threat of CWD being zoonosis, I believed the O.I.E. has failed terribly and again, I call for this organization to be dissolved. ...
 
 
Tuesday, July 17, 2012
 
O.I.E. BSE, CWD, SCRAPIE, TSE PRION DISEASE Final Report of the 80th General Session, 20 - 25 May 2012
 
 
 
Thursday, December 20, 2012
 
OIE GROUP RECOMMENDS THAT SCRAPE PRION DISEASE BE DELISTED AND SAME OLD BSe WITH BOVINE MAD COW DISEASE
 
 
Monday, November 30, 2009
 
*** USDA AND OIE COLLABORATE TO EXCLUDE ATYPICAL SCRAPIE NOR-98 ANIMAL HEALTH CODE, DOES NOT SURPRISE ME $
 
 
 
IN A NUT SHELL ;
 
(Adopted by the International Committee of the OIE on 23 May 2006)
 
11. Information published by the OIE is derived from appropriate declarations made by the official Veterinary Services of Member Countries. The OIE is not responsible for inaccurate publication of country disease status based on inaccurate information or changes in epidemiological status or other significant events that were not promptly reported to the Central Bureau,
 
 
 
Thursday, May 30, 2013
 
World Organization for Animal Health (OIE) has upgraded the United States' risk classification for mad cow disease to "negligible" from "controlled", and risk further exposing the globe to the TSE prion mad cow type disease
 
U.S. gets top mad-cow rating from international group and risk further exposing the globe to the TSE prion mad cow type disease
 
 
 
 
Creutzfeldt-Jakob Disease CJD cases rising North America updated report August 2013
 
*** Creutzfeldt-Jakob Disease CJD cases rising North America with Canada seeing an extreme increase of 48% between 2008 and 2010 ***
 
 
 
Sunday, October 13, 2013
 
*** CJD TSE Prion Disease Cases in Texas by Year, 2003-2012
 
 
 
*** When people talk about 1 per million, often they interpret that as thinking it is incredibly rare. They think they have a 1-in-a-million chance of developing this disease. You haven’t. You’ve got about a 1-in-30,000 chance of developing it.
 
 
 
Tuesday, April 01, 2014
 
*** Questions linger in U.S. CJD cases 2005, and still do in 2014 ***
 
 
 
Sent: Sunday, September 28, 2014 5:14 PM
Subject: USA 4TH CASE VCJD (aka nvCJD) HUMAN MAD COW, THE SILENCE IS DEAFENING BSE, CWD, AND SCRAPIE TSE PRION DISEASE
 
USA 4TH CASE VCJD (aka nvCJD) HUMAN MAD COW, THE SILENCE IS DEAFENING BSE, CWD, AND SCRAPIE TSE PRION DISEASE
 
Greetings DSHS, Dr. Fishcer, et al,
 
I know that most in the USA could care less about the CJD TSE prion disease aka mad cow type disease. but there are some of us here that will never forget.
 
you can cover up what ever you want. we all know. I have seen it happen too many times here in Texas with BSE TSE prion, either the typical or the atypical strains, or with the feed, or, with cwd, or scrapie as that goes, but we are still here, and we will never forget...
 
kind regards, terry
 
Creutzfeld-Jacob Disease (CJD) Emerging & Acute Infectious Disease Branch Michael Fischer
 
Marilyn Felkner
 
512-776-7676
 
512-776-7676
 
Chronic Wasting Disease Zoonosis Control Branch Eric Fonken
 
512-776-2155
 
Lab tests have confirmed a diagnosis of variant Creutzfeldt-Jakob Disease (CJD) in a patient who recently died in Texas. Variant CJD is a rare, fatal brain disorder, first described in 1996 in the United Kingdom and associated with beef consumption overseas.
 
This is the fourth case ever reported in the United States. In each of the three previous cases, infection likely occurred outside the United States, including the United Kingdom and Saudi Arabia. The history of this fourth patient includes extensive travel to Europe and the Middle East, and infection likely occurred outside the United States. The CDC and DSHS continue to investigate the case.
 
There are no Texas public health concerns or threats associated with this case.
 
 
 
 
Last updated June 02, 2014
 
 
CONFIRMED HUMAN BSE AKA MAD COW DISEASE vCJD TEXAS USA
 
CONFIRMED HUMAN BSE AKA MAD COW DISEASE vCJD TEXAS USA
 
Monday, June 02, 2014 Confirmed Variant CJD Case in Texas
 
 
 
SO, 4 months after the fact and still no word on this case. no information what so ever. the silence is deafening $$$
 
 
SO, 4 months after the fact and still no word on this case.
 
no information what so ever. the silence is deafening $$$
 
*** CONFIRMED HUMAN BSE AKA MAD COW DISEASE vCJD TEXAS USA
 
Monday, June 02, 2014 Confirmed Variant CJD Case in Texas
 
 
 
MAD DEER AND ELK DISEASE
 
CHRONIC WASTING DISEASE CWD
 
TRANSMISSIBLE SPONGIFORM ENCEPHALOPATHY TSE
 
PRION DISEASE
 
 
*** The potential impact of prion diseases on human health was greatly magnified by the recognition that interspecies transfer of BSE to humans by beef ingestion resulted in vCJD. While changes in animal feed constituents and slaughter practices appear to have curtailed vCJD, there is concern that CWD of free-ranging deer and elk in the U.S. might also cross the species barrier. Thus, consuming venison could be a source of human prion disease. Whether BSE and CWD represent interspecies scrapie transfer or are newly arisen prion diseases is unknown. Therefore, the possibility of transmission of prion disease through other food animals cannot be ruled out. There is evidence that vCJD can be transmitted through blood transfusion. There is likely a pool of unknown size of asymptomatic individuals infected with vCJD, and there may be asymptomatic individuals infected with the CWD equivalent. These circumstances represent a potential threat to blood, blood products, and plasma supplies.
 
 
NOW, what is the latest on human risk factors to CWD strains ???
 
*** PPo3-7: Prion Transmission from Cervids to Humans is Strain-dependent
 
*** Here we report that a human prion strain that had adopted the cervid prion protein (PrP) sequence through passage in cervidized transgenic mice efficiently infected transgenic mice expressing human PrP,
 
*** indicating that the species barrier from cervid to humans is prion strain-dependent and humans can be vulnerable to novel cervid prion strains.
 
PPo2-27:
 
Generation of a Novel form of Human PrPSc by Inter-species Transmission of Cervid Prions
 
*** Our findings suggest that CWD prions have the capability to infect humans, and that this ability depends on CWD strain adaptation, implying that the risk for human health progressively increases with the spread of CWD among cervids.
 
PPo2-7:
 
Biochemical and Biophysical Characterization of Different CWD Isolates
 
*** The data presented here substantiate and expand previous reports on the existence of different CWD strains.
 
 
Envt.07:
 
Pathological Prion Protein (PrPTSE) in Skeletal Muscles of Farmed and Free Ranging White-Tailed Deer Infected with Chronic Wasting Disease
 
***The presence and seeding activity of PrPTSE in skeletal muscle from CWD-infected cervids suggests prevention of such tissue in the human diet as a precautionary measure for food safety, pending on further clarification of whether CWD may be transmissible to humans.
 
 
>>>CHRONIC WASTING DISEASE , THERE WAS NO ABSOLUTE BARRIER TO CONVERSION OF THE HUMAN PRION PROTEIN<<<
 
*** PRICE OF CWD TSE PRION POKER GOES UP 2014 ***
 
Transmissible Spongiform Encephalopathy TSE PRION update January 2, 2014
 
Wednesday, January 01, 2014
 
Molecular Barriers to Zoonotic Transmission of Prions
 
*** chronic wasting disease, there was no absolute barrier to conversion of the human prion protein.
 
*** Furthermore, the form of human PrPres produced in this in vitro assay when seeded with CWD, resembles that found in the most common human prion disease, namely sCJD of the MM1 subtype.
 
 
 
PRION2013 CONGRESSIONAL ABSTRACTS CWD
 
Sunday, August 25, 2013
 
HD.13: CWD infection in the spleen of humanized transgenic mice
 
***These results indicate that the CWD prion may have the potential to infect human peripheral lymphoid tissues.
 
Oral.15: Molecular barriers to zoonotic prion transmission: Comparison of the ability of sheep, cattle and deer prion disease isolates to convert normal human prion protein to its pathological isoform in a cell-free system ***However, they also show that there is no absolute barrier to conversion of human prion protein in the case of chronic wasting disease.
 
PRION2013 CONGRESSIONAL ABSTRACTS CWD
 
Sunday, August 25, 2013
 
***Chronic Wasting Disease CWD risk factors, humans, domestic cats, blood, and mother to offspring transmission
 
 
Friday, November 09, 2012
 
*** Chronic Wasting Disease CWD in cervidae and transmission to other species
 
 
there is in fact evidence that the potential for cwd transmission to humans can NOT be ruled out.
 
I thought your readers and hunters and those that consume the venison, should have all the scientific facts, personally, I don’t care what you eat, but if it effects me and my family down the road, it should then concern everyone, and the potential of iatrogenic transmission of the TSE prion is real i.e. ‘friendly fire’, medical, surgical, dental, blood, tissue, and or products there from...like deer antler velvet and TSE prions and nutritional supplements there from, all a potential risk factor that should not be ignored or silenced. ...
 
the prion gods at the cdc state that there is ;
 
''no strong evidence''
 
but let's see exactly what the authors of this cwd to human at the cdc state ;
 
now, let’s see what the authors said about this casual link, personal communications years ago. see where it is stated NO STRONG evidence. so, does this mean there IS casual evidence ????
 
“Our conclusion stating that we found no strong evidence of CWD transmission to humans”
 
From: TSS (216-119-163-189.ipset45.wt.net)
 
Subject: CWD aka MAD DEER/ELK TO HUMANS ???
 
Date: September 30, 2002 at 7:06 am PST
 
From: "Belay, Ermias"
 
To:
 
Cc: "Race, Richard (NIH)" ; ; "Belay, Ermias"
 
Sent: Monday, September 30, 2002 9:22 AM
 
Subject: RE: TO CDC AND NIH - PUB MED- 3 MORE DEATHS - CWD - YOUNG HUNTERS
 
Dear Sir/Madam,
 
In the Archives of Neurology you quoted (the abstract of which was attached to your email), we did not say CWD in humans will present like variant CJD.
 
That assumption would be wrong. I encourage you to read the whole article and call me if you have questions or need more clarification (phone: 404-639-3091). Also, we do not claim that "no-one has ever been infected with prion disease from eating venison." Our conclusion stating that we found no strong evidence of CWD transmission to humans in the article you quoted or in any other forum is limited to the patients we investigated.
 
Ermias Belay, M.D. Centers for Disease Control and Prevention
 
-----Original Message-----
 
From:
 
Sent: Sunday, September 29, 2002 10:15 AM
 
To: rr26k@nih.gov; rrace@niaid.nih.gov; ebb8@CDC.GOV
 
Subject: TO CDC AND NIH - PUB MED- 3 MORE DEATHS - CWD - YOUNG HUNTERS
 
Sunday, November 10, 2002 6:26 PM ......snip........end..............TSS
 
Thursday, April 03, 2008
 
A prion disease of cervids: Chronic wasting disease
 
2008 1: Vet Res. 2008 Apr 3;39(4):41
 
A prion disease of cervids: Chronic wasting disease
 
Sigurdson CJ.
 
snip...
 
*** twenty-seven CJD patients who regularly consumed venison were reported to the Surveillance Center***,
 
snip...
 
full text ;
 
 
 
 
***********CJD REPORT 1994 increased risk for consumption of veal and venison and lamb***********
 
CREUTZFELDT JAKOB DISEASE SURVEILLANCE IN THE UNITED KINGDOM THIRD ANNUAL REPORT AUGUST 1994
 
Consumption of venison and veal was much less widespread among both cases and controls. For both of these meats there was evidence of a trend with increasing frequency of consumption being associated with increasing risk of CJD. (not nvCJD, but sporadic CJD...tss)
 
These associations were largely unchanged when attention was restricted to pairs with data obtained from relatives. ...
 
Table 9 presents the results of an analysis of these data.
 
There is STRONG evidence of an association between ‘’regular’’ veal eating and risk of CJD (p = .0.01).
 
Individuals reported to eat veal on average at least once a year appear to be at 13 TIMES THE RISK of individuals who have never eaten veal.
 
There is, however, a very wide confidence interval around this estimate. There is no strong evidence that eating veal less than once per year is associated with increased risk of CJD (p = 0.51).
 
The association between venison eating and risk of CJD shows similar pattern, with regular venison eating associated with a 9 FOLD INCREASE IN RISK OF CJD (p = 0.04).
 
There is some evidence that risk of CJD INCREASES WITH INCREASING FREQUENCY OF LAMB EATING (p = 0.02).
 
The evidence for such an association between beef eating and CJD is weaker (p = 0.14). When only controls for whom a relative was interviewed are included, this evidence becomes a little STRONGER (p = 0.08).
 
snip...
 
It was found that when veal was included in the model with another exposure, the association between veal and CJD remained statistically significant (p = < 0.05 for all exposures), while the other exposures ceased to be statistically significant (p = > 0.05).
 
snip...
 
In conclusion, an analysis of dietary histories revealed statistical associations between various meats/animal products and INCREASED RISK OF CJD. When some account was taken of possible confounding, the association between VEAL EATING AND RISK OF CJD EMERGED AS THE STRONGEST OF THESE ASSOCIATIONS STATISTICALLY. ...
 
snip...
 
In the study in the USA, a range of foodstuffs were associated with an increased risk of CJD, including liver consumption which was associated with an apparent SIX-FOLD INCREASE IN THE RISK OF CJD. By comparing the data from 3 studies in relation to this particular dietary factor, the risk of liver consumption became non-significant with an odds ratio of 1.2 (PERSONAL COMMUNICATION, PROFESSOR A. HOFMAN. ERASMUS UNIVERSITY, ROTTERDAM). (???...TSS)
 
snip...see full report ;
 
 
Thursday, October 10, 2013
 
*************CJD REPORT 1994 increased risk for consumption of veal and venison and lamb**************
 
 
CJD9/10022
 
October 1994
 
Mr R.N. Elmhirst Chairman British Deer Farmers Association Holly Lodge Spencers Lane BerksWell Coventry CV7 7BZ
 
Dear Mr Elmhirst,
 
CREUTZFELDT-JAKOB DISEASE (CJD) SURVEILLANCE UNIT REPORT
 
Thank you for your recent letter concerning the publication of the third annual report from the CJD Surveillance Unit. I am sorry that you are dissatisfied with the way in which this report was published.
 
The Surveillance Unit is a completely independant outside body and the Department of Health is committed to publishing their reports as soon as they become available. In the circumstances it is not the practice to circulate the report for comment since the findings of the report would not be amended. In future we can ensure that the British Deer Farmers Association receives a copy of the report in advance of publication.
 
The Chief Medical Officer has undertaken to keep the public fully informed of the results of any research in respect of CJD. This report was entirely the work of the unit and was produced completely independantly of the the Department.
 
The statistical results reqarding the consumption of venison was put into perspective in the body of the report and was not mentioned at all in the press release. Media attention regarding this report was low key but gave a realistic presentation of the statistical findings of the Unit. This approach to publication was successful in that consumption of venison was highlighted only once by the media ie. in the News at one television proqramme.
 
I believe that a further statement about the report, or indeed statistical links between CJD and consumption of venison, would increase, and quite possibly give damaging credence, to the whole issue. From the low key media reports of which I am aware it seems unlikely that venison consumption will suffer adversely, if at all.
 
 
DOCKET-- 03D-0186 -- FDA Issues Draft Guidance on Use of Material From Deer and Elk in Animal Feed; Availability
 
Date: Fri, 16 May 2003 11:47:37 –0500
 
EMC 1 Terry S. Singeltary Sr. Vol #: 1
 
 
 
 
*** We hypothesize that both BSE prions and CWD prions passaged through felines will seed human recPrP more efficiently than BSE or CWD from the original hosts, evidence that the new host will dampen the species barrier between humans and BSE or CWD. The new host effect is particularly relevant as we investigate potential means of trans-species transmission of prion disease.
 
 
Monday, August 8, 2011
 
*** Susceptibility of Domestic Cats to CWD Infection ***
 
Oral.29: Susceptibility of Domestic Cats to CWD Infection
 
Amy Nalls, Nicholas J. Haley, Jeanette Hayes-Klug, Kelly Anderson, Davis M. Seelig, Dan S. Bucy, Susan L. Kraft, Edward A. Hoover and Candace K. Mathiason†
 
Colorado State University; Fort Collins, CO USA†Presenting author; Email: ckm@lamar.colostate.edu
 
Domestic and non-domestic cats have been shown to be susceptible to one prion disease, feline spongiform encephalopathy (FSE), thought to be transmitted through consumption of bovine spongiform encephalopathy (BSE) contaminated meat. Because domestic and free ranging felids scavenge cervid carcasses, including those in CWD affected areas, we evaluated the susceptibility of domestic cats to CWD infection experimentally. Groups of n = 5 cats each were inoculated either intracerebrally (IC) or orally (PO) with CWD deer brain homogenate. Between 40–43 months following IC inoculation, two cats developed mild but progressive symptoms including weight loss, anorexia, polydipsia, patterned motor behaviors and ataxia—ultimately mandating euthanasia. Magnetic resonance imaging (MRI) on the brain of one of these animals (vs. two age-matched controls) performed just before euthanasia revealed increased ventricular system volume, more prominent sulci, and T2 hyperintensity deep in the white matter of the frontal hemisphere and in cortical grey distributed through the brain, likely representing inflammation or gliosis. PrPRES and widely distributed peri-neuronal vacuoles were demonstrated in the brains of both animals by immunodetection assays. No clinical signs of TSE have been detected in the remaining primary passage cats after 80 months pi. Feline-adapted CWD was sub-passaged into groups (n=4 or 5) of cats by IC, PO, and IP/SQ routes. Currently, at 22 months pi, all five IC inoculated cats are demonstrating abnormal behavior including increasing aggressiveness, pacing, and hyper responsiveness.
 
*** Two of these cats have developed rear limb ataxia. Although the limited data from this ongoing study must be considered preliminary, they raise the potential for cervid-to-feline transmission in nature.
 
 
 
AD.63:
 
Susceptibility of domestic cats to chronic wasting disease
 
Amy V.Nalls,1 Candace Mathiason,1 Davis Seelig,2 Susan Kraft,1 Kevin Carnes,1 Kelly Anderson,1 Jeanette Hayes-Klug1 and Edward A. Hoover1 1Colorado State University; Fort Collins, CO USA; 2University of Minnesota; Saint Paul, MN USA
 
Domestic and nondomestic cats have been shown to be susceptible to feline spongiform encephalopathy (FSE), almost certainly caused by consumption of bovine spongiform encephalopathy (BSE)-contaminated meat. Because domestic and free-ranging nondomestic felids scavenge cervid carcasses, including those in areas affected by chronic wasting disease (CWD), we evaluated the susceptibility of the domestic cat (Felis catus) to CWD infection experimentally. Cohorts of 5 cats each were inoculated either intracerebrally (IC) or orally (PO) with CWD-infected deer brain. At 40 and 42 mo post-inoculation, two IC-inoculated cats developed signs consistent with prion disease, including a stilted gait, weight loss, anorexia, polydipsia, patterned motor behaviors, head and tail tremors, and ataxia, and progressed to terminal disease within 5 mo. Brains from these two cats were pooled and inoculated into cohorts of cats by IC, PO, and intraperitoneal and subcutaneous (IP/SC) routes. Upon subpassage, feline-adapted CWD (FelCWD) was transmitted to all IC-inoculated cats with a decreased incubation period of 23 to 27 mo. FelCWD was detected in the brains of all the symptomatic cats by western blotting and immunohistochemistry and abnormalities were seen in magnetic resonance imaging, including multifocal T2 fluid attenuated inversion recovery (FLAIR) signal hyper-intensities, ventricular size increases, prominent sulci, and white matter tract cavitation. Currently, 3 of 4 IP/SQ and 2 of 4 PO inoculared cats have developed abnormal behavior patterns consistent with the early stage of feline CWD.
 
*** These results demonstrate that CWD can be transmitted and adapted to the domestic cat, thus raising the issue of potential cervid-to- feline transmission in nature.
 
 
www.landesbioscience.com
 
PO-081: Chronic wasting disease in the cat— Similarities to feline spongiform encephalopathy (FSE)
 
 
 
FELINE SPONGIFORM ENCEPHALOPATHY FSE
 
 
 
Sunday, November 10, 2013
 
LARGE CJD TSE PRION POTENTIAL CASE STUDY AMONG HUMANS WHO TAKE DEER ANTLER VELVET WILL BE ONGOING FOR YEARS IF NOT DECADES, but who's cares $
 
 
Singeltary submission ;
 
Program Standards: Chronic Wasting Disease Herd Certification Program and Interstate Movement of Farmed or Captive Deer, Elk, and Moose
 
DOCUMENT ID: APHIS-2006-0118-0411
 
***Singeltary submission
 
Docket No. 00-108-10 Chronic Wasting Disease Herd Certification Program and Interstate Movement of Farmed or Captive Deer, Elk, and Moose; Program Standards
 
>>>The CWD herd certification program is a voluntary, cooperative program that establishes minimum requirements for the interstate movement of farmed or captive cervids, provisions for participating States to administer Approved State CWD Herd Certification Programs, and provisions for participating herds to become certified as having a low risk of being infected with CWD<<<
 
Greetings USDA/APHIS et al,
 
I kindly would like to comment on Docket No. 00-108-10 Chronic Wasting Disease Herd Certification Program and Interstate Movement of Farmed or Captive Deer, Elk, and Moose; Program Standards.
 
I believe, and in my opinion, and this has been proven by scientific facts, that without a validated and certified test for chronic wasting disease cwd, that is 100% sensitive, and in use, any voluntary effort will be futile. the voluntary ban on mad cow feed and SRMs have failed terribly, the bse mad cow surveillance program has failed terribly, as well as the testing for bse tse prion in cattle, this too has failed terrible. all this has been proven time and time again via OIG reports and GOA reports.
 
I believe that until this happens, 100% cwd testing with validated test, ALL MOVEMENT OF CERVIDS BETWEEN STATES MUST BE BANNED, AND THE BORDERS CLOSED TO INTERSTATE MOVEMENT OF CERVIDS. there is simply to much at risk.
 
In my opinion, and the opinions of many scientists and DNR officials, that these so called game farms are the cause of the spreading of chronic wasting disease cwd through much negligence. the game farms in my opinion are not the only cause, but a big factor. I kindly wish to submit the following to show what these factors are, and why interstate movement of cervids must be banned. ...
 
snip...see full text and PDF ATTACHMENT HERE ;
 
 
 
Sunday, June 23, 2013
 
National Animal Health Laboratory Network Reorganization Concept Paper (Document ID APHIS-2012-0105-0001)
 
***Terry S. Singeltary Sr. submission
 
 
Friday, November 22, 2013
 
Wasting disease is threat to the entire UK deer population CWD TSE PRION disease in cervids
 
***SINGELTARY SUBMISSION
 
The Scottish Parliament’s Rural Affairs, Climate Change and Environment Committee has been looking into deer management, as you can see from the following press release,
 
***and your email has been forwarded to the committee for information:
 
 
 
Friday, November 22, 2013
 
Wasting disease is threat to the entire UK deer population
 
 
Sunday, July 21, 2013
 
Welsh Government and Food Standards Agency Wales Joint Public Consultation on the Proposed Transmissible Spongiform Encephalopathies (Wales) Regulations 2013
 
*** Singeltary Submission WG18417
 
 
Thursday, July 03, 2014
 
*** How Chronic Wasting Disease is affecting deer population and what’s the risk to humans and pets?
 
 
=============================================================================
 
Tuesday, July 01, 2014
 
*** CHRONIC WASTING DISEASE CWD TSE PRION DISEASE, GAME FARMS, AND POTENTIAL RISK FACTORS THERE FROM ***
 
 
=============================================================================
 
Ronnie Dunn Cross Examination, slaughtering cattle, or killing deer ?
 
IN THE UNITED STATES DISTRICT COURT FOR THE NORTHERN DISTRICT OF INDIANA SOUTH BEND DIVISION UNITED STATES OF AMERICA, vs. RUSSELL G. BELLAR, Defendant.
 
___________________________
 
)))))))))
 
Cause No.: 3:04cr00068-AS South Bend, Indiana January 4, 2005 9:30 a.m.
 
TRANSCRIPT EXCERPT OF JURY TRIAL (TESTIMONY OF: RONNIE DUNN AND RUSTY CAMP) BEFORE THE HONORABLE ALLEN SHARP
 
snip...
 
Ronnie Dunn Cross Examination
 
Q. Mr. Dunn, at one point I believe you told the federal agents that Mr. Bellar told you that this was a private deer farm and shooting deer on that farm was like slaughtering cattle; is that correct?
 
A. I don't know if I used the word "slaughter," but it was, yeah, like that.
 
Q. You don't know if that was your word, "slaughtering cattle"?
 
A. I don't know that.
 
Q. Well, did he give you the idea of killing cattle?
 
A. Yes, it was the same principle.
 
snip...
 
see full text ;
 
 
 
 
BUCK FEVER
 
 
Thursday, September 18, 2014
 
*** Risk behaviors in a rural community with a known point-source exposure to chronic wasting disease
 
 
Saturday, September 20, 2014
 
*** North Carolina Captive cervid licenses and permits Senate Bill 744 Singeltary Submission
 
Description The proposed changes to 15A NACA 10H .0301 would allow the Commission to issue new captivity licenses and permits for the purpose of holding cervids in captivity and allow certified herd owners to sell or transfer cervids to any licensed facility. Also, mandatory testing for CWD will be raised from all cervids that die at age 6 months or older to all cervids that die at age 12 months or older.
 
 
North Carolina Captive cervid licenses and permits Senate Bill 744 Singeltary Submission
 
*** p.s. please add this to my submission, very important information...
 
Saturday, February 04, 2012
 
*** Wisconsin 16 age limit on testing dead deer Game Farm CWD Testing Protocol Needs To Be Revised
 
Approximately 4,200 fawns, defined as deer under 1 year of age, were sampled from the eradication zone over the last year. The majority of fawns sampled were between the ages of 5 to 9 months, though some were as young as 1 month.
 
*** Two of the six fawns with CWD detected were 5 to 6 months old.
 
All six of the positive fawns were taken from the core area of the CWD eradication zone where the highest numbers of positive deer have been identified.
 
Saturday, February 04, 2012
 
*** Wisconsin 16 age limit on testing dead deer Game Farm CWD Testing Protocol Needs To Be Revised
 
 
*** Conclusion. CWD prions (as inferred by prion seeding activity by RT-QuIC) are shed in urine of infected deer as early as 6 months post inoculation and throughout the subsequent disease course. Further studies are in progress refining the real-time urinary prion assay sensitivity and we are examining more closely the excretion time frame, magnitude, and sample variables in relationship to inoculation route and prionemia in naturally and experimentally CWD-infected cervids.
 
 
SNIP...SEE FULL TEXT ;
 
Saturday, September 20, 2014
 
*** North Carolina Captive cervid licenses and permits Senate Bill 744 Singeltary Submission
 
 
Thursday, September 11, 2014
 
Missouri Nixon's Veto Stands Overide Fails on Agriculture Legislation
 
How they voted: attempt to override veto of ag bill fails in the House
 
 
Wednesday, September 17, 2014
 
Wyoming GAME AND FISH CONTINUES CWD SAMPLING
 
 
Pennsylvania
 
>>> Thank you for your comments, which I will share with the Board Thank You kindly !
 
>>> Please note that in Pennsylvania, the responsibility for regulating captive cervids falls to the Pennsylvania Department of Agriculture.
 
please note;
 
*** Spraker suggested an interesting explanation for the occurrence of CWD. The deer pens at the Foot Hills Campus were built some 30-40 years ago by a Dr. Bob Davis. At or abut that time, allegedly, some scrapie work was conducted at this site. When deer were introduced to the pens they occupied ground that had previously been occupied by sheep. ...
 
also, see where even decades back, the USDA had the same thought as they do today with CWD, not their problem...see page 27 below as well, where USDA stated back then, the same thing they stated in the state of Pennsylvania, not their damn business, once they escape, and they said the same thing about CWD in general back then ;
 
”The occurrence of CWD must be viewed against the contest of the locations in which it occurred. It was an incidental and unwelcome complication of the respective wildlife research programmes. Despite it’s subsequent recognition as a new disease of cervids, therefore justifying direct investigation, no specific research funding was forthcoming.
 
***The USDA veiwed it as a wildlife problem and consequently not their province!” ...page 26.
 
 
”The occurrence of CWD must be viewed against the contest of the locations in which it occurred. It was an incidental and unwelcome complication of the respective wildlife research programmes. Despite it’s subsequent recognition as a new disease of cervids, therefore justifying direct investigation, no specific research funding was forthcoming. The USDA veiwed it as a wildlife problem and consequently not their province!” ...page 26.
 
sound familiar $$$
 
Sunday, January 06, 2013
 
USDA TO PGC ONCE CAPTIVES ESCAPE
 
*** "it‘s no longer its business.”
 
 
Saturday, June 29, 2013
 
PENNSYLVANIA CAPTIVE CWD INDEX HERD MATE YELLOW *47 STILL RUNNING LOOSE IN INDIANA, YELLOW NUMBER 2 STILL MISSING, AND OTHERS ON THE RUN STILL IN LOUISIANA
 
 
Tuesday, May 28, 2013
 
Chronic Wasting Disease CWD quarantine Louisiana via CWD index herd Pennsylvania Update May 28, 2013
 
*** 6 doe from Pennsylvania CWD index herd still on the loose in Louisiana, quarantine began on October 18, 2012, still ongoing, Lake Charles premises.
 
 
Thursday, September 11, 2014
 
*** TEXAS ANIMAL HEALTH COMMISSION 390th COMMISSION MEETING AGENDA (CWD movement restriction zone) September 16, 2014 8:30 A.M.
 
 
Wednesday, September 17, 2014
 
*** Cervid Health Business Plan Fiscal Years 2014 to 2018 Animal and Plant Health Inspection Service Veterinary Services ***
 
 
Friday, September 05, 2014
 
*** CFIA CWD and Grain Screenings due to potential risk factor of spreading via contamination of grain, oil seeds, etc. ***
 
 
Wednesday, September 17, 2014
 
*** Cervid Health Business Plan Fiscal Years 2014 to 2018 Animal and Plant Health Inspection Service Veterinary Services ***
 
 
Sunday, September 21, 2014
 
INFORM: Cervid Health and States Indemnity FY 2015
 
 
Wednesday, September 17, 2014
 
Cost benefit analysis of the development and use of ante-mortem tests for transmissible spongiform encephalopathies
 
 
Sunday, August 24, 2014
 
*** USAHA 117TH ANNUAL MEETING USDA-APHIS–VS CWD Herd Certification Program Goals TSE PRION October 17 – 23, 2013
 
 
*** Ronnie Dunn Cross Examination, slaughtering cattle, or killing deer ? ***
 
IN THE UNITED STATES DISTRICT COURT FOR THE NORTHERN DISTRICT OF INDIANA SOUTH BEND DIVISION UNITED STATES OF AMERICA, vs. RUSSELL G. BELLAR, Defendant.
 
___________________________
 
)))))))))
 
Cause No.: 3:04cr00068-AS South Bend, Indiana January 4, 2005 9:30 a.m.
 
TRANSCRIPT EXCERPT OF JURY TRIAL (TESTIMONY OF: RONNIE DUNN AND RUSTY CAMP) BEFORE THE HONORABLE ALLEN SHARP
 
snip...
 
Ronnie Dunn Cross Examination
 
Q. Mr. Dunn, at one point I believe you told the federal agents that Mr. Bellar told you that this was a private deer farm and shooting deer on that farm was like slaughtering cattle; is that correct?
 
A. I don't know if I used the word "slaughter," but it was, yeah, like that.
 
Q. You don't know if that was your word, "slaughtering cattle"?
 
A. I don't know that.
 
Q. Well, did he give you the idea of killing cattle?
 
A. Yes, it was the same principle.
 
snip...
 
see full text ;
 
 
 
 
BUCK FEVER
 
 
Thursday, September 18, 2014
 
*** Risk behaviors in a rural community with a known point-source exposure to chronic wasting disease
 
 
*** We conclude that TSE infectivity is likely to survive burial for long time periods with minimal loss of infectivity and limited movement from the original burial site. However PMCA results have shown that there is the potential for rainwater to elute TSE related material from soil which could lead to the contamination of a wider area. These experiments reinforce the importance of risk assessment when disposing of TSE risk materials.
 
*** The results show that even highly diluted PrPSc can bind efficiently to polypropylene, stainless steel, glass, wood and stone and propagate the conversion of normal prion protein. For in vivo experiments, hamsters were ic injected with implants incubated in 1% 263K-infected brain homogenate. Hamsters, inoculated with 263K-contaminated implants of all groups, developed typical signs of prion disease, whereas control animals inoculated with non-contaminated materials did not.
 
PRION 2014 CONFERENCE
 
CHRONIC WASTING DISEASE CWD
 
A FEW FINDINGS ;
 
Conclusions. To our knowledge, this is the first established experimental model of CWD in TgSB3985. We found evidence for co-existence or divergence of two CWD strains adapted to Tga20 mice and their replication in TgSB3985 mice. Finally, we observed phenotypic differences between cervid-derived CWD and CWD/Tg20 strains upon propagation in TgSB3985 mice. Further studies are underway to characterize these strains.
 
We conclude that TSE infectivity is likely to survive burial for long time periods with minimal loss of infectivity and limited movement from the original burial site. However PMCA results have shown that there is the potential for rainwater to elute TSE related material from soil which could lead to the contamination of a wider area. These experiments reinforce the importance of risk assessment when disposing of TSE risk materials.
 
The results show that even highly diluted PrPSc can bind efficiently to polypropylene, stainless steel, glass, wood and stone and propagate the conversion of normal prion protein. For in vivo experiments, hamsters were ic injected with implants incubated in 1% 263K-infected brain homogenate. Hamsters, inoculated with 263K-contaminated implants of all groups, developed typical signs of prion disease, whereas control animals inoculated with non-contaminated materials did not.
 
Our data establish that meadow voles are permissive to CWD via peripheral exposure route, suggesting they could serve as an environmental reservoir for CWD. Additionally, our data are consistent with the hypothesis that at least two strains of CWD circulate in naturally-infected cervid populations and provide evidence that meadow voles are a useful tool for CWD strain typing.
 
Conclusion. CWD prions are shed in saliva and urine of infected deer as early as 3 months post infection and throughout the subsequent >1.5 year course of infection. In current work we are examining the relationship of prionemia to excretion and the impact of excreted prion binding to surfaces and particulates in the environment.
 
Conclusion. CWD prions (as inferred by prion seeding activity by RT-QuIC) are shed in urine of infected deer as early as 6 months post inoculation and throughout the subsequent disease course. Further studies are in progress refining the real-time urinary prion assay sensitivity and we are examining more closely the excretion time frame, magnitude, and sample variables in relationship to inoculation route and prionemia in naturally and experimentally CWD-infected cervids.
 
Conclusions. Our results suggested that the odds of infection for CWD is likely controlled by areas that congregate deer thus increasing direct transmission (deer-to-deer interactions) or indirect transmission (deer-to-environment) by sharing or depositing infectious prion proteins in these preferred habitats. Epidemiology of CWD in the eastern U.S. is likely controlled by separate factors than found in the Midwestern and endemic areas for CWD and can assist in performing more efficient surveillance efforts for the region.
 
Conclusions. During the pre-symptomatic stage of CWD infection and throughout the course of disease deer may be shedding multiple LD50 doses per day in their saliva. CWD prion shedding through saliva and excreta may account for the unprecedented spread of this prion disease in nature.
 
see full text and more ;
 
Monday, June 23, 2014
 
*** PRION 2014 CONFERENCE CHRONIC WASTING DISEASE CWD
 
 
 
*** Infectious agent of sheep scrapie may persist in the environment for at least 16 years***
 
Gudmundur Georgsson1, Sigurdur Sigurdarson2 and Paul Brown3
 
 
New studies on the heat resistance of hamster-adapted scrapie agent: Threshold survival after ashing at 600°C suggests an inorganic template of replication
 
 
Prion Infected Meat-and-Bone Meal Is Still Infectious after Biodiesel Production
 
 
Detection of protease-resistant cervid prion protein in water from a CWD-endemic area
 
 
A Quantitative Assessment of the Amount of Prion Diverted to Category 1 Materials and Wastewater During Processing
 
 
Rapid assessment of bovine spongiform encephalopathy prion inactivation by heat treatment in yellow grease produced in the industrial manufacturing process of meat and bone meals
 
 
PPo4-4:
 
Survival and Limited Spread of TSE Infectivity after Burial
 
PPo4-4:
 
Survival and Limited Spread of TSE Infectivity after Burial
 
Karen Fernie, Allister Smith and Robert A. Somerville The Roslin Institute and R(D)SVS; University of Edinburgh; Roslin, Scotland UK
 
Scrapie and chronic wasting disease probably spread via environmental routes, and there are also concerns about BSE infection remaining in the environment after carcass burial or waste 3disposal. In two demonstration experiments we are determining survival and migration of TSE infectivity when buried for up to five years, as an uncontained point source or within bovine heads. Firstly boluses of TSE infected mouse brain were buried in lysimeters containing either sandy or clay soil. Migration from the boluses is being assessed from soil cores taken over time. With the exception of a very small amount of infectivity found 25 cm from the bolus in sandy soil after 12 months, no other infectivity has been detected up to three years. Secondly, ten bovine heads were spiked with TSE infected mouse brain and buried in the two soil types. Pairs of heads have been exhumed annually and assessed for infectivity within and around them. After one year and after two years, infectivity was detected in most intracranial samples and in some of the soil samples taken from immediately surrounding the heads. The infectivity assays for the samples in and around the heads exhumed at years three and four are underway. These data show that TSE infectivity can survive burial for long periods but migrates slowly. Risk assessments should take into account the likely long survival rate when infected material has been buried.
 
The authors gratefully acknowledge funding from DEFRA.
 
 
 
*** Susceptibility of UK red deer (Cervus alaphus elaphus) to oral BSE transmission Project Code: M03024 ***
 
02/08/2011
 
The project confirmed that U.K red deer are susceptible to both oral and intra-cerebral inoculation with the cattle BSE agent. Six clinically positive (from 26-42 months post inoculation) i.c inoculated and one (56 months post inoculation) orally dosed deer that tested positive for TSE by immunohistochemistry and Western blotting using several primary antibodies demonstrated widespread accumulation of disease specific prion protein in the central nervous system, peripheral nervous system and enteric nervous system but none in lymphoreticular system. All showed several brain sites positive for disease specific prion protein and presented immunohistochemistry and Western blotting phenotypes with similarities to BSE in sheep, goats and cattle but unlike those seen in chronic wasting disease (CWD) in elk or scrapie in sheep. The vacuolar pathology and distribution of disease specific prion protein in red deer resembled that of CWD in most major respects however we have shown that BSE can be clearly differentiated from CWD by existing immunohistochemical and biochemical methods that are in routine use.
 
The knowledge gained as a result of this work will permit rapid and accurate diagnosis should a TSE ever be detected in European red deer and will also enable effective disease control methods to be quickly put in place.
 
 
Results
 
We confirmed that U.K red deer are susceptible to both oral and intra-cerebral inoculation with the cattle BSE agent. Six clinically positive (from 26-42mpi) i.c inoculated and one (56mpi) orally dosed deer that tested positive for TSE by IHC and WB using several primary antibodies demonstrated widespread accumulation of disease specific PrP in CNS, PNS and ENS but none in LRS. All showed several brain sites positive for disease specific PrP and presented IHC and WB phenotypes with similarities to BSE in sheep, goats and cattle but unlike those seen in CWD in elk or scrapie in sheep. The vacuolar pathology and distribution of PrPd BSE in red deer resembled that of CWD in most major respects however we have shown that BSE can be clearly differentiated from CWD by existing immunohistochemical and biochemical methods that are in routine use.
 
Final technical report MO3024 01/04/2003 – 31/03/2010 Susceptibility of UK red deer (Cervus elaphus elaphus) to oral BSE transmission. Stuart Martin - VLA Lasswade Pentlands Science Park Bush Loan Penicuik EH26 0PZ Page 2 of 21 Further work undertaken August 2009 – March 2010. Genetic analysis - Wilfred Goldmann; Roslin NPD.
 
Negative controls and the remaining 5 orally dosed deer culled at 72mpi tested negative by IHC and Western blot however analysis of the PrP ORF of these deer (kindly carried out by Wilfred Goldmann of the Roslin NPD) identified a Q to E polymorphism at codon 226 that may influence the efficiency of oral transmission (not published).
 
In the experimental BSE challenge of red deer six out of six deer succumbed to BSE when challenged by intracerebral routes but only one of six deer challenged by the oral route succumbed to infection. Deer killed at 190 days or 365 days post oral challenge showed no evidence of abnormal PrP accumulation when tested by immunocytochemistry. The PrP gene of red deer includes a Q to E polymorphism at codon 226. The table shows the distribution of these codon 226 polymorphisms within experimental challenge groups.
 
snip...
 
 
Research article Open Access
 
Immunohistochemical and biochemical characteristics of BSE and CWD in experimentally infected European red deer (Cervus elaphus elaphus)
 
Stuart Martin*1, Martin Jeffrey1, Lorenzo González1, Sílvia Sisó1, Hugh W Reid2, Philip Steele2, Mark P Dagleish2, Michael J Stack3, Melanie J Chaplin3 and Aru Balachandran4 Address: 1Veterinary Laboratories Agency (VLA-Lasswade), Pentlands Science Park, Bush Loan, Penicuik, Midlothian, EH26 0PZ, UK, 2Moredun Research Institute, Pentlands Science Park, Bush Loan, Penicuik, Midlothian, EH26 0PZ, UK, 3VLA-Weybridge, Addlestone, Surrey, KT15 3NB, UK and 4Animal Diseases Research Institute, Canadian Food Inspection Agency, Ottawa, Ontario, K2H 8P9, Canada
 
Abstract
 
Background: The cause of the bovine spongiform encephalopathy (BSE) epidemic in the United Kingdom (UK) was the inclusion of contaminated meat and bone meal in the protein rations fed to cattle. Those rations were not restricted to cattle but were also fed to other livestock including farmed and free living deer. Although there are no reported cases to date of natural BSE in European deer, BSE has been shown to be naturally or experimentally transmissible to a wide range of different ungulate species. Moreover, several species of North America's cervids are highly susceptible to chronic wasting disease (CWD), a transmissible spongiform encephalopathy (TSE) that has become endemic. Should BSE infection have been introduced into the UK deer population, the CWD precedent could suggest that there is a danger for spread and maintenance of the disease in both free living and captive UK deer populations. This study compares the immunohistochemical and biochemical characteristics of BSE and CWD in experimentally-infected European red deer (Cervus elpahus elaphus).
 
Results: After intracerebral or alimentary challenge, BSE in red deer more closely resembled natural infection in cattle rather than experimental BSE in small ruminants, due to the lack of accumulation of abnormal PrP in lymphoid tissues. In this respect it was different from CWD, and although the neuropathological features of both diseases were similar, BSE could be clearly differentiated from CWD by immunohistochemical and Western blotting methods currently in routine use.
 
Conclusion: Red deer are susceptible to both BSE and CWD infection, but the resulting disease phenotypes are distinct and clearly distinguishable.
 
SNIP...
 
Results
 
Clinical disease
 
All six deer challenged i.c. with BSE developed clinical disease between 794 and 1260 days post-inoculation with a mean incubation period of 1027 days. A detailed description of the clinical signs was provided in an earlier report [8]. Briefly, affected deer showed variable degrees of ataxia, anorexia, circling and apparent blindness, together with failure of seasonal change of coat, weight loss and 'panic attacks'. In addition, one of six red deer orally dosed with BSE developed clinical disease 1740 days after challenge, and this animal presented with a short clinical duration of two days; the other five deer from this group remain healthy at the time of writing (65 months after challenge). Sequential rectal biopsies taken at five different time points from orally and i.c. inoculated deer were negative for PrPd.
 
All four deer orally challenged with CWD started to show behavioural changes between 577 and 586 days post challenge;
 
these progressed to definite neurological disease between 742 and 760 days post-challenge (Table 1).
 
Clinical signs were similar to the BSE challenged deer and included nervousness, weight loss, excessive salivation, roughness of coat, and progressive ataxia. All these CWD inoculated deer showed PrPd accumulation in the secondary follicles of rectal biopsies taken at 7 months post infection.
 
Conclusion
 
European red deer are susceptible to infection with the cattle BSE agent, not only by the intra-cerebral but also by the oral route, and although the clinical signs and spong- iform change are similar to those of CWD in the same species, these two infections can be easily differentiated. The lack of lymphoid involvement, the PrPd truncation pattern both "in vivo" and "in vitro", and the predominantly intracellular accumulation of PrPd are features of deer BSE that are in contrast with those of deer CWD. However, only one of six deer developed disease after alimentary exposure to 25 g of a BSE brain pool homogenate after an incubation period of nearly 5 years; this suggests a strong species barrier but if a TSE in European red deer should ever be identified then BSE/CWD discrimination would be an urgent priority. To determine whether there are potential naturally occurring BSE-like strains and to determine the degree to which there is strain variation, it would be necessary to examine many more naturally occurring CWD cases. These results will support the ongoing European surveillance for natural TSEs in red deer and the further assessment of potential risk to human health.
 
Published: 27 July 2009 BMC Veterinary Research 2009, 5:26 doi:10.1186/1746-6148-5-26 Received: 12 February 2009 Accepted: 27 July 2009 This article is available from: http://www.biomedcentral.com/1746-6148/5/26 © 2009 Martin et al; licensee BioMed Central Ltd. This is an Open Access article distributed under the terms of the Creative Commons Attribution License (http://creativecommons.org/licenses/by/2.0), which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.
 
 
Monday, May 05, 2014
 
*** Member Country details for listing OIE CWD 2013 against the criteria of Article 1.2.2., the Code Commission recommends consideration for listing ***
 
 
snip...
 
Friday, December 14, 2012
 
DEFRA U.K. What is the risk of Chronic Wasting Disease CWD being introduced into Great Britain? A Qualitative Risk Assessment October 2012
 
snip...
 
In the USA, under the Food and Drug Administration’s BSE Feed Regulation (21 CFR 589.2000) most material (exceptions include milk, tallow, and gelatin) from deer and elk is prohibited for use in feed for ruminant animals. With regards to feed for non-ruminant animals, under FDA law, CWD positive deer may not be used for any animal feed or feed ingredients. For elk and deer considered at high risk for CWD, the FDA recommends that these animals do not enter the animal feed system. However, this recommendation is guidance and not a requirement by law.
 
Animals considered at high risk for CWD include:
 
1) animals from areas declared to be endemic for CWD and/or to be CWD eradication zones and
 
2) deer and elk that at some time during the 60-month period prior to slaughter were in a captive herd that contained a CWD-positive animal.
 
Therefore, in the USA, materials from cervids other than CWD positive animals may be used in animal feed and feed ingredients for non-ruminants.
 
The amount of animal PAP that is of deer and/or elk origin imported from the USA to GB can not be determined, however, as it is not specified in TRACES. It may constitute a small percentage of the 8412 kilos of non-fish origin processed animal proteins that were imported from US into GB in 2011.
 
*** Overall, therefore, it is considered there is a __greater than negligible risk___ that (nonruminant) animal feed and pet food containing deer and/or elk protein is imported into GB.
 
There is uncertainty associated with this estimate given the lack of data on the amount of deer and/or elk protein possibly being imported in these products.
 
snip...
 
 
2003D-0186 Guidance for Industry: Use of Material From Deer and Elk In Animal Feed
 
EMC 1 Terry S. Singeltary Sr. Vol #: 1
 
 
 
see my full text submission here ;
 
 
Sunday, December 15, 2013
 
*** FDA PART 589 -- SUBSTANCES PROHIBITED FROM USE IN ANIMAL FOOD OR FEED VIOLATIONS OFFICIAL ACTION INDICATED OIA UPDATE DECEMBER 2013 UPDATE
 
 
Tuesday, September 16, 2014
 
mad cow scaremongers consumerfreedom.com December 20, 2003 article and a 2014 review
 
 
The detection of low levels of BSE-PrPSc in the presence of an excess of scrapie-PrPSc
 
It was previously thought that any given prion disease had a very limited natural host range. However, the BSE agent has affected species other than cattle. This study aims to provide a novel, high sensitivity assay to monitor for the presence of very low levels of BSE in small ruminants, even with concurrent scrapie infections. Study Duration: October 2012 to December 2013 Contractor: ADAS Project Code: FS101077 Background Research Approach Background Prion diseases are fatal brain diseases with no effective treatment. Until the emergence of BSE in the mid-1980s it was thought that any given prion disease had a very limited natural host range. For example, scrapie affecting sheep/goats and Creutzfeldt Jakob disease (CJD) affecting humans. However, the BSE agent not only affected cattle, but was also the causal agent of feline spongiform encephalopathy and human variant CJD (vCJD), both arising from the consumption of BSE contaminated bovine products.
 
BSE-contaminated bovine-derived meat and bone meal was extensively fed not only to cows, giving rise to the UK cattle BSE epidemic, but also to small ruminants. Yet extensive surveillance has failed to uncover BSE infections in sheep populations throughout Europe, including the UK. This is surprising given that sheep are susceptible to BSE when fed the agent under experimental conditions. Two cases of BSE infections have been identified in goats. The UK small ruminant population has had endemic scrapie and whilst the incidence of this disease has been significantly reduced, it is estimated that approximately 67,000 scrapie infected animals still enter the human food chain each year. One concern is that low levels of BSE cases may be harboured within the small ruminant population and be masked by concurrent scrapie infections.
 
Research Approach The presence of mixed strains of prion agents within an individual host is known to exist for both natural infections and experimental models. Importantly, it is also known that hosts with mixed infections displaying a single pathology can harbour low levels of a distinct strain which can emerge upon further passage. There are limited studies on mixed infections with BSE and scrapie, however within a mouse model this scenario led to the presentation of a scrapie-associated pathology, even when the inoculum had much greater levels of BSE agent compared to scrapie agent. Together, data suggests that similar mixed infections of sheep or goats may present as scrapie even though the BSE agent is present.
 
An in vitro prion amplification assay will be applied to the detection of very low levels of BSE agent in the presence of an excess of scrapie agent. The contractor has previously demonstrated that this assay is exquisitely sensitive for the BSE agent and does not amplify any scrapie agents. The aim of this study is to establish this novel amplification-assay for the detection of BSE in the presence of scrapie agent and to compare this method with conventional BSE tests applied in routine surveillance. The aim is to provide a novel, high sensitivity assay to monitor for the presence of very low levels of BSE in small ruminants, even with concurrent scrapie infections. It will help to inform policy as to the potential risk of BSE agent being present in sheep or goats but not being detected when measured by conventional assays.
 
 
Overall, all Nor98 isolates contained highly PK resistant PrPres aggregates, with the main PrPres being a non-glycosylated internal fragment, cleaved at both the N and C termini, which represent the distinctive biochemical feature of Nor98. This biochemical signature, unique among animal TSEs, is reminiscent of PrPres observed in human prion disorders such as GSS and VPSPr.
 
snip...
 
At present the only epidemiological link between animal and human TSEs has been demonstrated for classical BSE and variant CJD [16], [78], showing for the first time the zoonotic potential of TSEs. Since then, the implementation of active surveillance in livestock has led to the identification of Nor98 and other previously unrecognised animal prion strains, mainly with a sporadic occurrence, whose origin and zoonotic potential are still poorly understood [79]. It has been previously shown that peripheral tissues of sheep with Nor98 might harbour detectable levels of infectivity [49], [50], indicating that infectious material might enter the food chain. On the other hand, the well known genetic aetiology of GSS suggests that the similar PrPSc conformations found in Nor98 and GSS P102L are unlikely to indicate a common infectious source, but might derive from a similar molecular mechanisms involved in PrPC-to-PrPSc conversion.
 
snip...
 
Citation: Pirisinu L, Nonno R, Esposito E, Benestad SL, Gambetti P, et al. (2013) Small Ruminant Nor98 Prions Share Biochemical Features with Human Gerstmann-Sträussler-Scheinker Disease and Variably Protease-Sensitive Prionopathy. PLoS ONE 8(6): e66405. doi:10.1371/journal.pone.0066405
 
Editor: Corinne Ida Lasmezas, The Scripps Research Institute Scripps Florida, United States of America
 
Received: January 24, 2013; Accepted: May 6, 2013; Published: June 24, 2013
 
Copyright: © 2013 Pirisinu et al. This is an open-access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited.
 
Funding: This work was supported by grants from the Italian Ministry of Health (RF-2009-1474624); the European Union (Neuroprion Network of Excellence CT-2004–506579); the National Institutes of Health (NIH) NS062787, NIH AG-08012, AG-14359; Alliance BioSecure, as well as the Center for Disease Control and Prevention Contract UR8/CCU515004. The funders had no role in study design, data collection and analysis, decision to publish, or preparation of the manuscript.
 
Competing interests: The authors have declared that no competing interests exist.
 
 
*** The discovery of previously unrecognized prion diseases in both humans and animals (i.e., Nor98 in small ruminants) demonstrates that the range of prion diseases might be wider than expected and raises crucial questions about the epidemiology and strain properties of these new forms. We are investigating this latter issue by molecular and biological comparison of VPSPr, GSS and Nor98.
 
VARIABLY PROTEASE-SENSITVE PRIONOPATHY IS TRANSMISSIBLE ...price of prion poker goes up again $
 
OR-10: Variably protease-sensitive prionopathy is transmissible in bank voles
 
Romolo Nonno,1 Michele Di Bari,1 Laura Pirisinu,1 Claudia D’Agostino,1 Stefano Marcon,1 Geraldina Riccardi,1 Gabriele Vaccari,1 Piero Parchi,2 Wenquan Zou,3 Pierluigi Gambetti,3 Umberto Agrimi1 1Istituto Superiore di Sanità; Rome, Italy; 2Dipartimento di Scienze Neurologiche, Università di Bologna; Bologna, Italy; 3Case Western Reserve University; Cleveland, OH USA
 
Background. Variably protease-sensitive prionopathy (VPSPr) is a recently described “sporadic”neurodegenerative disease involving prion protein aggregation, which has clinical similarities with non-Alzheimer dementias, such as fronto-temporal dementia. Currently, 30 cases of VPSPr have been reported in Europe and USA, of which 19 cases were homozygous for valine at codon 129 of the prion protein (VV), 8 were MV and 3 were MM. A distinctive feature of VPSPr is the electrophoretic pattern of PrPSc after digestion with proteinase K (PK). After PK-treatment, PrP from VPSPr forms a ladder-like electrophoretic pattern similar to that described in GSS cases. The clinical and pathological features of VPSPr raised the question of the correct classification of VPSPr among prion diseases or other forms of neurodegenerative disorders. Here we report preliminary data on the transmissibility and pathological features of VPSPr cases in bank voles.
 
Materials and Methods. Seven VPSPr cases were inoculated in two genetic lines of bank voles, carrying either methionine or isoleucine at codon 109 of the prion protein (named BvM109 and BvI109, respectively). Among the VPSPr cases selected, 2 were VV at PrP codon 129, 3 were MV and 2 were MM. Clinical diagnosis in voles was confirmed by brain pathological assessment and western blot for PK-resistant PrPSc (PrPres) with mAbs SAF32, SAF84, 12B2 and 9A2.
 
Results. To date, 2 VPSPr cases (1 MV and 1 MM) gave positive transmission in BvM109. Overall, 3 voles were positive with survival time between 290 and 588 d post inoculation (d.p.i.). All positive voles accumulated PrPres in the form of the typical PrP27–30, which was indistinguishable to that previously observed in BvM109 inoculated with sCJDMM1 cases.
 
In BvI109, 3 VPSPr cases (2 VV and 1 MM) showed positive transmission until now. Overall, 5 voles were positive with survival time between 281 and 596 d.p.i.. In contrast to what observed in BvM109, all BvI109 showed a GSS-like PrPSc electrophoretic pattern, characterized by low molecular weight PrPres. These PrPres fragments were positive with mAb 9A2 and 12B2, while being negative with SAF32 and SAF84, suggesting that they are cleaved at both the C-terminus and the N-terminus. Second passages are in progress from these first successful transmissions.
 
Conclusions. Preliminary results from transmission studies in bank voles strongly support the notion that VPSPr is a transmissible prion disease. Interestingly, VPSPr undergoes divergent evolution in the two genetic lines of voles, with sCJD-like features in BvM109 and GSS-like properties in BvI109.
 
The discovery of previously unrecognized prion diseases in both humans and animals (i.e., Nor98 in small ruminants) demonstrates that the range of prion diseases might be wider than expected and raises crucial questions about the epidemiology and strain properties of these new forms. We are investigating this latter issue by molecular and biological comparison of VPSPr, GSS and Nor98.
 
 
Wednesday, March 28, 2012
 
VARIABLY PROTEASE-SENSITVE PRIONOPATHY IS TRANSMISSIBLE, price of prion poker goes up again $
 
 
Thursday, October 10, 2013
 
CJD REPORT 1994 increased risk for consumption of veal and venison and lamb
 
 
*** The discovery of previously unrecognized prion diseases in both humans and animals (i.e., Nor98 in small ruminants) demonstrates that the range of prion diseases might be wider than expected and raises crucial questions about the epidemiology and strain properties of these new forms. We are investigating this latter issue by molecular and biological comparison of VPSPr, GSS and Nor98.
 
Increased Atypical Scrapie Detections
 
Press reports indicate that increased surveillance is catching what otherwise would have been unreported findings of atypical scrapie in sheep. In 2009, five new cases have been reported in Quebec, Ontario, Alberta, and Saskatchewan. With the exception of Quebec, all cases have been diagnosed as being the atypical form found in older animals. Canada encourages producers to join its voluntary surveillance program in order to gain scrapie-free status. The World Animal Health will not classify Canada as scrapie-free until no new cases are reported for seven years. The Canadian Sheep Federation is calling on the government to fund a wider surveillance program in order to establish the level of prevalence prior to setting an eradication date. Besides long-term testing, industry is calling for a compensation program for farmers who report unusual deaths in their flocks.
 
 
Thursday, March 29, 2012
 
atypical Nor-98 Scrapie has spread from coast to coast in the USA 2012
 
NIAA Annual Conference April 11-14, 2011San Antonio, Texas
 
 
Monday, April 25, 2011
 
Experimental Oral Transmission of Atypical Scrapie to Sheep
 
Volume 17, Number 5-May 2011 However, work with transgenic mice has demonstrated the potential susceptibility of pigs, with the disturbing finding that the biochemical properties of the resulting PrPSc have changed on transmission (40).
 
 
Monday, December 14, 2009
 
Similarities between Forms of Sheep Scrapie and Creutzfeldt-Jakob Disease Are Encoded by Distinct Prion Types
 
(hmmm, this is getting interesting now...TSS)
 
Sporadic CJD type 1 and atypical/ Nor98 scrapie are characterized by fine (reticular) deposits,
 
see also ;
 
All of the Heidenhain variants were of the methionine/ methionine type 1 molecular subtype.
 
 
see full text ;
 
Monday, December 14, 2009
 
Similarities between Forms of Sheep Scrapie and Creutzfeldt-Jakob Disease Are Encoded by Distinct Prion Types
 
 
P03.141
 
Aspects of the Cerebellar Neuropathology in Nor98
 
Gavier-Widén, D1; Benestad, SL2; Ottander, L1; Westergren, E1 1National Veterinary Insitute, Sweden; 2National Veterinary Institute,
 
Norway Nor98 is a prion disease of old sheep and goats. This atypical form of scrapie was first described in Norway in 1998. Several features of Nor98 were shown to be different from classical scrapie including the distribution of disease associated prion protein (PrPd) accumulation in the brain. The cerebellum is generally the most affected brain area in Nor98. The study here presented aimed at adding information on the neuropathology in the cerebellum of Nor98 naturally affected sheep of various genotypes in Sweden and Norway. A panel of histochemical and immunohistochemical (IHC) stainings such as IHC for PrPd, synaptophysin, glial fibrillary acidic protein, amyloid, and cell markers for phagocytic cells were conducted. The type of histological lesions and tissue reactions were evaluated. The types of PrPd deposition were characterized. The cerebellar cortex was regularly affected, even though there was a variation in the severity of the lesions from case to case. Neuropil vacuolation was more marked in the molecular layer, but affected also the granular cell layer. There was a loss of granule cells. Punctate deposition of PrPd was characteristic. It was morphologically and in distribution identical with that of synaptophysin, suggesting that PrPd accumulates in the synaptic structures. PrPd was also observed in the granule cell layer and in the white matter. The pathology features of Nor98 in the cerebellum of the affected sheep showed similarities with those of sporadic Creutzfeldt-Jakob disease in humans.
 
***The pathology features of Nor98 in the cerebellum of the affected sheep showed similarities with those of sporadic Creutzfeldt-Jakob disease in humans.
 
 
PR-26
 
NOR98 SHOWS MOLECULAR FEATURES REMINISCENT OF GSS
 
R. Nonno1, E. Esposito1, G. Vaccari1, E. Bandino2, M. Conte1, B. Chiappini1, S. Marcon1, M. Di Bari1, S.L. Benestad3, U. Agrimi1 1 Istituto Superiore di Sanità, Department of Food Safety and Veterinary Public Health, Rome, Italy (romolo.nonno@iss.it); 2 Istituto Zooprofilattico della Sardegna, Sassari, Italy; 3 National Veterinary Institute, Department of Pathology, Oslo, Norway
 
Molecular variants of PrPSc are being increasingly investigated in sheep scrapie and are generally referred to as "atypical" scrapie, as opposed to "classical scrapie". Among the atypical group, Nor98 seems to be the best identified. We studied the molecular properties of Italian and Norwegian Nor98 samples by WB analysis of brain homogenates, either untreated, digested with different concentrations of proteinase K, or subjected to enzymatic deglycosylation. The identity of PrP fragments was inferred by means of antibodies spanning the full PrP sequence. We found that undigested brain homogenates contain a Nor98-specific PrP fragment migrating at 11 kDa (PrP11), truncated at both the C-terminus and the N-terminus, and not N-glycosylated. After mild PK digestion, Nor98 displayed full-length PrP (FL-PrP) and N-glycosylated C-terminal fragments (CTF), along with increased levels of PrP11. Proteinase K digestion curves (0,006-6,4 mg/ml) showed that FL-PrP and CTF are mainly digested above 0,01 mg/ml, while PrP11 is not entirely digested even at the highest concentrations, similarly to PrP27-30 associated with classical scrapie. Above 0,2 mg/ml PK, most Nor98 samples showed only PrP11 and a fragment of 17 kDa with the same properties of PrP11, that was tentatively identified as a dimer of PrP11. Detergent solubility studies showed that PrP11 is insoluble in 2% sodium laurylsorcosine and is mainly produced from detergentsoluble, full-length PrPSc. Furthermore, among Italian scrapie isolates, we found that a sample with molecular and pathological properties consistent with Nor98 showed plaque-like deposits of PrPSc in the thalamus when the brain was analysed by PrPSc immunohistochemistry. Taken together, our results show that the distinctive pathological feature of Nor98 is a PrP fragment spanning amino acids ~ 90-155. This fragment is produced by successive N-terminal and C-terminal cleavages from a full-length and largely detergent-soluble PrPSc, is produced in vivo and is extremely resistant to PK digestion.
 
*** Intriguingly, these conclusions suggest that some pathological features of Nor98 are reminiscent of Gerstmann-Sträussler-Scheinker disease.
 
119
 
 
A newly identified type of scrapie agent can naturally infect sheep with resistant PrP genotypes
 
Annick Le Dur*,?, Vincent Béringue*,?, Olivier Andréoletti?, Fabienne Reine*, Thanh Lan Laï*, Thierry Baron§, Bjørn Bratberg¶, Jean-Luc Vilotte?, Pierre Sarradin**, Sylvie L. Benestad¶, and Hubert Laude*,?? +Author Affiliations
 
*Virologie Immunologie Moléculaires and ?Génétique Biochimique et Cytogénétique, Institut National de la Recherche Agronomique, 78350 Jouy-en-Josas, France; ?Unité Mixte de Recherche, Institut National de la Recherche Agronomique-Ecole Nationale Vétérinaire de Toulouse, Interactions Hôte Agent Pathogène, 31066 Toulouse, France; §Agence Française de Sécurité Sanitaire des Aliments, Unité Agents Transmissibles Non Conventionnels, 69364 Lyon, France; **Pathologie Infectieuse et Immunologie, Institut National de la Recherche Agronomique, 37380 Nouzilly, France; and ¶Department of Pathology, National Veterinary Institute, 0033 Oslo, Norway
 
***Edited by Stanley B. Prusiner, University of California, San Francisco, CA (received for review March 21, 2005)
 
Abstract Scrapie in small ruminants belongs to transmissible spongiform encephalopathies (TSEs), or prion diseases, a family of fatal neurodegenerative disorders that affect humans and animals and can transmit within and between species by ingestion or inoculation. Conversion of the host-encoded prion protein (PrP), normal cellular PrP (PrPc), into a misfolded form, abnormal PrP (PrPSc), plays a key role in TSE transmission and pathogenesis. The intensified surveillance of scrapie in the European Union, together with the improvement of PrPSc detection techniques, has led to the discovery of a growing number of so-called atypical scrapie cases. These include clinical Nor98 cases first identified in Norwegian sheep on the basis of unusual pathological and PrPSc molecular features and "cases" that produced discordant responses in the rapid tests currently applied to the large-scale random screening of slaughtered or fallen animals. Worryingly, a substantial proportion of such cases involved sheep with PrP genotypes known until now to confer natural resistance to conventional scrapie. Here we report that both Nor98 and discordant cases, including three sheep homozygous for the resistant PrPARR allele (A136R154R171), efficiently transmitted the disease to transgenic mice expressing ovine PrP, and that they shared unique biological and biochemical features upon propagation in mice. *** These observations support the view that a truly infectious TSE agent, unrecognized until recently, infects sheep and goat flocks and may have important implications in terms of scrapie control and public health.
 
 
Monday, December 1, 2008
 
When Atypical Scrapie cross species barriers
 
Authors
 
Andreoletti O., Herva M. H., Cassard H., Espinosa J. C., Lacroux C., Simon S., Padilla D., Benestad S. L., Lantier F., Schelcher F., Grassi J., Torres, J. M., UMR INRA ENVT 1225, Ecole Nationale Veterinaire de Toulouse.France; ICISA-INlA, Madrid, Spain; CEA, IBiTec-5, DSV, CEA/Saclay, Gif sur Yvette cedex, France; National Veterinary Institute, Postboks 750 Sentrum, 0106 Oslo, Norway, INRA IASP, Centre INRA de Tours, 3738O Nouzilly, France.
 
Content
 
Atypical scrapie is a TSE occurring in small ruminants and harbouring peculiar clinical, epidemiological and biochemical properties. Currently this form of disease is identified in a large number of countries. In this study we report the transmission of an atypical scrapie isolate through different species barriers as modeled by transgenic mice (Tg) expressing different species PRP sequence.
 
The donor isolate was collected in 1995 in a French commercial sheep flock. inoculation into AHQ/AHQ sheep induced a disease which had all neuro-pathological and biochemical characteristics of atypical scrapie. Transmitted into Transgenic mice expressing either ovine or PrPc, the isolate retained all the described characteristics of atypical scrapie.
 
Surprisingly the TSE agent characteristics were dramatically different v/hen passaged into Tg bovine mice. The recovered TSE agent had biological and biochemical characteristics similar to those of atypical BSE L in the same mouse model. Moreover, whereas no other TSE agent than BSE were shown to transmit into Tg porcine mice, atypical scrapie was able to develop into this model, albeit with low attack rate on first passage.
 
Furthermore, after adaptation in the porcine mouse model this prion showed similar biological and biochemical characteristics than BSE adapted to this porcine mouse model. Altogether these data indicate.
 
(i) the unsuspected potential abilities of atypical scrapie to cross species barriers
 
(ii) the possible capacity of this agent to acquire new characteristics when crossing species barrier
 
These findings raise some interrogation on the concept of TSE strain and on the origin of the diversity of the TSE agents and could have consequences on field TSE control measures.
 
 
Friday, February 11, 2011
 
Atypical/Nor98 Scrapie Infectivity in Sheep Peripheral Tissues
 
 
Saturday, August 14, 2010
 
BSE Case Associated with Prion Protein Gene Mutation (g-h-BSEalabama) and VPSPr PRIONPATHY
 
(see mad cow feed in COMMERCE IN ALABAMA...TSS)
 
 
why do we not want to do TSE transmission studies on chimpanzees $
 
5. A positive result from a chimpanzee challenged severly would likely create alarm in some circles even if the result could not be interpreted for man. I have a view that all these agents could be transmitted provided a large enough dose by appropriate routes was given and the animals kept long enough. Until the mechanisms of the species barrier are more clearly understood it might be best to retain that hypothesis.
 
snip...
 
R. BRADLEY
 
 
1: J Infect Dis 1980 Aug;142(2):205-8
 
Oral transmission of kuru, Creutzfeldt-Jakob disease, and scrapie to nonhuman primates.
 
Gibbs CJ Jr, Amyx HL, Bacote A, Masters CL, Gajdusek DC.
 
Kuru and Creutzfeldt-Jakob disease of humans and scrapie disease of sheep and goats were transmitted to squirrel monkeys (Saimiri sciureus) that were exposed to the infectious agents only by their nonforced consumption of known infectious tissues. The asymptomatic incubation period in the one monkey exposed to the virus of kuru was 36 months; that in the two monkeys exposed to the virus of Creutzfeldt-Jakob disease was 23 and 27 months, respectively; and that in the two monkeys exposed to the virus of scrapie was 25 and 32 months, respectively. Careful physical examination of the buccal cavities of all of the monkeys failed to reveal signs or oral lesions. One additional monkey similarly exposed to kuru has remained asymptomatic during the 39 months that it has been under observation.
 
snip...
 
The successful transmission of kuru, Creutzfeldt-Jakob disease, and scrapie by natural feeding to squirrel monkeys that we have reported provides further grounds for concern that scrapie-infected meat may occasionally give rise in humans to Creutzfeldt-Jakob disease.
 
PMID: 6997404
 
 
Recently the question has again been brought up as to whether scrapie is transmissible to man. This has followed reports that the disease has been transmitted to primates. One particularly lurid speculation (Gajdusek 1977) conjectures that the agents of scrapie, kuru, Creutzfeldt-Jakob disease and transmissible encephalopathy of mink are varieties of a single "virus". The U.S. Department of Agriculture concluded that it could "no longer justify or permit scrapie-blood line and scrapie-exposed sheep and goats to be processed for human or animal food at slaughter or rendering plants" (ARC 84/77)" The problem is emphasised by the finding that some strains of scrapie produce lesions identical to the once which characterise the human dementias"
 
Whether true or not. the hypothesis that these agents might be transmissible to man raises two considerations. First, the safety of laboratory personnel requires prompt attention. Second, action such as the "scorched meat" policy of USDA makes the solution of the acrapie problem urgent if the sheep industry is not to suffer grievously.
 
snip...
 
76/10.12/4.6
 
 
Nature. 1972 Mar 10;236(5341):73-4.
 
Transmission of scrapie to the cynomolgus monkey (Macaca fascicularis).
 
Gibbs CJ Jr, Gajdusek DC.
 
Nature 236, 73 - 74 (10 March 1972); doi:10.1038/236073a0
 
Transmission of Scrapie to the Cynomolgus Monkey (Macaca fascicularis)
 
C. J. GIBBS jun. & D. C. GAJDUSEK
 
National Institute of Neurological Diseases and Stroke, National Institutes of Health, Bethesda, Maryland
 
SCRAPIE has been transmitted to the cynomolgus, or crab-eating, monkey (Macaca fascicularis) with an incubation period of more than 5 yr from the time of intracerebral inoculation of scrapie-infected mouse brain. The animal developed a chronic central nervous system degeneration, with ataxia, tremor and myoclonus with associated severe scrapie-like pathology of intensive astroglial hypertrophy and proliferation, neuronal vacuolation and status spongiosus of grey matter. The strain of scrapie virus used was the eighth passage in Swiss mice (NIH) of a Compton strain of scrapie obtained as ninth intracerebral passage of the agent in goat brain, from Dr R. L. Chandler (ARC, Compton, Berkshire).
 
 
 
Suspect symptoms
 
What if you can catch old-fashioned CJD by eating meat from a sheep infected with scrapie?
 
28 Mar 01
 
Like lambs to the slaughter 31 March 2001 by Debora MacKenzie Magazine issue 2284. Subscribe and get 4 free issues. FOUR years ago, Terry Singeltary watched his mother die horribly from a degenerative brain disease. Doctors told him it was Alzheimer's, but Singeltary was suspicious. The diagnosis didn't fit her violent symptoms, and he demanded an autopsy. It showed she had died of sporadic Creutzfeldt-Jakob disease.
 
Most doctors believe that sCJD is caused by a prion protein deforming by chance into a killer. But Singeltary thinks otherwise. He is one of a number of campaigners who say that some sCJD, like the variant CJD related to BSE, is caused by eating meat from infected animals. Their suspicions have focused on sheep carrying scrapie, a BSE-like disease that is widespread in flocks across Europe and North America.
 
Now scientists in France have stumbled across new evidence that adds weight to the campaigners' fears. To their complete surprise, the researchers found that one strain of scrapie causes the same brain damage in mice as sCJD.
 
"This means we cannot rule out that at least some sCJD may be caused by some strains of scrapie," says team member Jean-Philippe Deslys of the French Atomic Energy Commission's medical research laboratory in Fontenay-aux-Roses, south-west of Paris. Hans Kretschmar of the University of Göttingen, who coordinates CJD surveillance in Germany, is so concerned by the findings that he now wants to trawl back through past sCJD cases to see if any might have been caused by eating infected mutton or lamb.
 
Scrapie has been around for centuries and until now there has been no evidence that it poses a risk to human health. But if the French finding means that scrapie can cause sCJD in people, countries around the world may have overlooked a CJD crisis to rival that caused by BSE.
 
Deslys and colleagues were originally studying vCJD, not sCJD. They injected the brains of macaque monkeys with brain from BSE cattle, and from French and British vCJD patients. The brain damage and clinical symptoms in the monkeys were the same for all three. Mice injected with the original sets of brain tissue or with infected monkey brain also developed the same symptoms.
 
As a control experiment, the team also injected mice with brain tissue from people and animals with other prion diseases: a French case of sCJD; a French patient who caught sCJD from human-derived growth hormone; sheep with a French strain of scrapie; and mice carrying a prion derived from an American scrapie strain. As expected, they all affected the brain in a different way from BSE and vCJD. But while the American strain of scrapie caused different damage from sCJD, the French strain produced exactly the same pathology.
 
"The main evidence that scrapie does not affect humans has been epidemiology," says Moira Bruce of the neuropathogenesis unit of the Institute for Animal Health in Edinburgh, who was a member of the same team as Deslys. "You see about the same incidence of the disease everywhere, whether or not there are many sheep, and in countries such as New Zealand with no scrapie." In the only previous comparisons of sCJD and scrapie in mice, Bruce found they were dissimilar.
 
But there are more than 20 strains of scrapie, and six of sCJD. "You would not necessarily see a relationship between the two with epidemiology if only some strains affect only some people," says Deslys. Bruce is cautious about the mouse results, but agrees they require further investigation. Other trials of scrapie and sCJD in mice, she says, are in progress.
 
People can have three different genetic variations of the human prion protein, and each type of protein can fold up two different ways. Kretschmar has found that these six combinations correspond to six clinical types of sCJD: each type of normal prion produces a particular pathology when it spontaneously deforms to produce sCJD.
 
But if these proteins deform because of infection with a disease-causing prion, the relationship between pathology and prion type should be different, as it is in vCJD. "If we look at brain samples from sporadic CJD cases and find some that do not fit the pattern," says Kretschmar, "that could mean they were caused by infection."
 
There are 250 deaths per year from sCJD in the US, and a similar incidence elsewhere. Singeltary and other US activists think that some of these people died after eating contaminated meat or "nutritional" pills containing dried animal brain. Governments will have a hard time facing activists like Singeltary if it turns out that some sCJD isn't as spontaneous as doctors have insisted.
 
Deslys's work on macaques also provides further proof that the human disease vCJD is caused by BSE. And the experiments showed that vCJD is much more virulent to primates than BSE, even when injected into the bloodstream rather than the brain. This, says Deslys, means that there is an even bigger risk than we thought that vCJD can be passed from one patient to another through contaminated blood transfusions and surgical instruments.
 
 
Sunday, December 12, 2010
 
EFSA reviews BSE/TSE infectivity in small ruminant tissues News Story 2 December 2010
 
 
Wednesday, January 18, 2012
 
Selection of Distinct Strain Phenotypes in Mice Infected by Ovine Natural Scrapie Isolates Similar to CH1641 Experimental Scrapie
 
Journal of Neuropathology & Experimental Neurology: February 2012 - Volume 71 - Issue 2 - p 140–147
 
 
Thursday, July 14, 2011
 
Histopathological Studies of "CH1641-Like" Scrapie Sources Versus Classical Scrapie and BSE Transmitted to Ovine Transgenic Mice (TgOvPrP4)
 
 
Wednesday, January 18, 2012
 
BSE IN GOATS CAN BE MISTAKEN FOR SCRAPIE
 
February 1, 2012
 
 
Thursday, December 23, 2010
 
Molecular Typing of Protease-Resistant Prion Protein in Transmissible Spongiform Encephalopathies of Small Ruminants, France, 2002-2009
 
Volume 17, Number 1 January 2011
 
 
Thursday, November 18, 2010
 
Increased susceptibility of human-PrP transgenic mice to bovine spongiform encephalopathy following passage in sheep
 
 
Monday, December 14, 2009
 
Similarities between Forms of Sheep Scrapie and Creutzfeldt-Jakob Disease Are Encoded by Distinct Prion Types
 
(hmmm, this is getting interesting now...TSS)
 
Sporadic CJD type 1 and atypical/ Nor98 scrapie are characterized by fine (reticular) deposits,
 
see also ;
 
All of the Heidenhain variants were of the methionine/ methionine type 1 molecular subtype.
 
 
see full text ;
 
Monday, December 14, 2009
 
Similarities between Forms of Sheep Scrapie and Creutzfeldt-Jakob Disease Are Encoded by Distinct Prion Types
 
 
Thursday, July 21, 2011
 
A Second Case of Gerstmann-Sträussler-Scheinker Disease Linked to the G131V Mutation in the Prion Protein Gene in a Dutch Patient Journal of Neuropathology & Experimental Neurology:
 
August 2011 - Volume 70 - Issue 8 - pp 698-702
 
 
Monday, April 25, 2011
 
Experimental Oral Transmission of Atypical Scrapie to Sheep
 
Volume 17, Number 5-May 2011
 
 
Sunday, April 18, 2010
 
SCRAPIE AND ATYPICAL SCRAPIE TRANSMISSION STUDIES A REVIEW 2010
 
 
Thursday, November 18, 2010
 
Increased susceptibility of human-PrP transgenic mice to bovine spongiform encephalopathy following passage in sheep
 
 
Wednesday, January 19, 2011
 
EFSA and ECDC review scientific evidence on possible links between TSEs in animals and humans Webnachricht 19 Januar 2011
 
 
Monday, June 27, 2011
 
Comparison of Sheep Nor98 with Human Variably Protease-Sensitive Prionopathy and Gerstmann-Sträussler-Scheinker Disease
 
 
Thursday, November 18, 2010
 
Increased susceptibility of human-PrP transgenic mice to bovine spongiform encephalopathy following passage in sheep
 
 
Wednesday, November 13, 2013
 
Atypical Scrapie Prions from Sheep and Lack of Disease in Transgenic Mice Overexpressing Human Prion Protein
 
 
REPORT OF THE COMMITTEE 344 Ewes were experimentally inoculated with brain homogenate obtained from a U.S. sheep with clinical Nor98-like scrapie.
 
Recipient ewes are bred annually to examine the placenta for evidence of a transmissible agent. Placentas shed 2009-2013 were negative.
 
*** In 2013, one recipient ewe developed an unrelated disease. At postmortem examination, abundant accumulation of PrPSc was observed only in the cerebellum of this ewe with much less accumulation in the hindbrain obex. This confirms that initial inoculation of these ewes has been successful. Monitoring continues in the remaining ewes of this study.
 
PROCEEDINGS ONE HUNDRED AND SEVENTEENTH ANNUAL MEETING of the UNITED STATES ANIMAL HEALTH ASSOCIATION Town and Country Hotel San Diego, California October 17 – 23, 2013
 
 
snip...see ;
 
Sunday, August 24, 2014
 
USAHA 117TH ANNUAL MEETING USDA-APHIS–VS CWD Herd Certification Program Goals TSE PRION October 17 – 23, 2013
 
 
*** OIE GROUP RECOMMENDS THAT SCRAPE PRION DISEASE BE DELISTED, WISHES TO CONTINUE SPREADING IT AROUND THE GLOBE
 
 
Monday, November 30, 2009
 
*** USDA AND OIE COLLABORATE TO EXCLUDE ATYPICAL SCRAPIE NOR-98 ANIMAL HEALTH CODE, DOES NOT SURPRISE ME $
 
 
Sunday, September 1, 2013
 
Evaluation of the Zoonotic Potential of Transmissible Mink Encephalopathy
 
We previously described the biochemical similarities between PrPres derived from L-BSE infected macaque and cortical MM2 sporadic CJD: those observations suggest a link between these two uncommon prion phenotypes in a primate model (it is to note that such a link has not been observed in other models less relevant from the human situation as hamsters or transgenic mice overexpressing ovine PrP [28]). We speculate that a group of related animal prion strains (L-BSE, c-BSE and TME) would have a zoonotic potential and lead to prion diseases in humans with a type 2 PrPres molecular signature (and more specifically type 2B for vCJD)
 
snip...
 
Together with previous experiments performed in ovinized and bovinized transgenic mice and hamsters [8,9] indicating similarities between TME and L-BSE, the data support the hypothesis that L-BSE could be the origin of the TME outbreaks in North America and Europe during the mid-1900s.
 
 
Tuesday, July 21, 2009
 
Transmissible mink encephalopathy - review of the etiology
 
 
Saturday, December 01, 2007
 
Phenotypic Similarity of Transmissible Mink Encephalopathy in Cattle and L-type Bovine Spongiform Encephalopathy in a Mouse Model
 
 
Sunday, December 10, 2006
 
Transmissible Mink Encephalopathy TME
 
 
TSE have been around for decades.
 
it’s not out of the question to think that CWD might have been here much sooner than it was documented.
 
have cwd and these other TSE been around for eons, or forever, I cannot answer that.
 
I know scarpie was first documented around 1947 here in the USA, how long it was here before that, I don’t know, some say over 250 years.
 
 
TME in mink was documented in the early 1960s. it was first thought that the TME out break was from scrapie infected sheep, until a investigation was done on feed practices at these mink facilities, and it was later found that the mink had been fed 95%+ dead stock downer cows. and later, the Late Richard Marsh tried to warn the feds of the pending mad cow debacle. they refused to listen. ...
 
some interesting reading on pages 26 to 33
 
 
 
1979
 
TME originates from feeding mink, scrapie infected materials...
 
 
Evidence That Transmissible Mink Encephalopathy Results from Feeding Infected Cattle
 
*** Over the next 8-10 weeks, approximately 40% of all the adult mink on the farm died from TME. ***
 
snip...
 
*** The rancher was a ''dead stock'' feeder using mostly (>95%) downer or dead dairy cattle ***
 
 
 
 
 
Wednesday, September 10, 2014
 
Creutzfeldt-Jakob disease (CJD) biannual update (August 2014), with updated guidance on decontamination of gastrointestinal endoscopy equipment
 
Research and analysis
 
 
CWD STRAINS TO HUMANS ???
 
*** These results would seem to suggest that CWD does indeed have zoonotic potential, at least as judged by the compatibility of CWD prions and their human PrPC target. Furthermore, extrapolation from this simple in vitro assay suggests that if zoonotic CWD occurred, it would most likely effect those of the PRNP codon 129-MM genotype and that the PrPres type would be similar to that found in the most common subtype of sCJD (MM1).
 
 
as I said, what if ?
 
*** our results raise the possibility that CJD cases classified as VV1 may include cases caused by iatrogenic transmission of sCJD-MM1 prions or food-borne infection by type 1 prions from animals, e.g., chronic wasting disease prions in cervid. In fact, two CJD-VV1 patients who hunted deer or consumed venison have been reported (40, 41). The results of the present study emphasize the need for traceback studies and careful re-examination of the biochemical properties of sCJD-VV1 prions. ***
 
 
===========================================
 
Thursday, January 2, 2014
 
*** CWD TSE Prion in cervids to hTGmice, Heidenhain Variant Creutzfeldt-Jacob Disease MM1 genotype, and iatrogenic CJD ??? ***
 
WHAT IF ?
 
 
Saturday, April 19, 2014
 
Exploring the zoonotic potential of animal prion diseases: In vivo and in vitro approaches
 
 
Creutzfeldt-Jakob Disease CJD cases rising North America updated report August 2013
 
*** Creutzfeldt-Jakob Disease CJD cases rising North America with Canada seeing an extreme increase of 48% between 2008 and 2010 ***
 
 
Sunday, October 13, 2013
 
*** CJD TSE Prion Disease Cases in Texas by Year, 2003-2012
 
 
Tuesday, April 01, 2014
 
*** Questions linger in U.S. CJD cases 2005, and still do in 2014
 
 
Monday, March 29, 2010
 
CJD TEXAS 38 YEAR OLD FEMALE WORKED SLAUGHTERING CATTLE EXPOSED TO BRAIN AND SPINAL CORD MATTER
 
URGENT, PLEASE NOTE ;
 
>>> Up until about 6 years ago, the pt worked at Tyson foods where she worked on the assembly line, slaughtering cattle and preparing them for packaging. She was exposed to brain and spinal cord matter when she would euthanize the cattle. <<<
 
 
 
CJD NE TEXAS CLUSTER
 
Creutzfeldt-Jakob Disease in Northeast Texas
 
J.A. Rawlings,*1 K.A. Hendricks1, O.M. Nuno1, D.A. Brown1, D.A. Evans2, Texas Department of Health, 1Austin and 2Tyler, Texas Creutzfeldt-Jacob Disease (CJD), a transmissible spongiform encephalopathy, is caused by prions composed of proteinaceous material devoid of nucleic acid. CJD occurs sporadically (generally 1 case/1,000,000 population per year) in older patients (average age of 65) and is characterized by rapidly progressive dementia, accompanied by severe muscle spasms and incoordination. Death usually occurs within 3 to 12 months (average 7 months). CJD activity in Texas, which has a population of nearly 19 million, appeared to be typical. The statewide death rate for 1995 and 1996 was just under 1/1,000,000. In April of 1997, the Texas Department of Health became aware of an increased number of possible CJD cases in a 23-county area of NE Texas with a population of just over one million. After review of medical and pathology records, four patients were identified with definite classic CJD and three were identified with probable CJD. Dates of death for the eight patients were from April, 1996 through mid-July 1997. The patients were from 46 through 65 years of age; four were male and three were female. A case-control study to identify risks for CJD in NE Texas has been initiated. http://www.jifsan.umd.edu/tse/Rawlings.htm
 
we get them young cases of tse prion disease in Texas, that is not related to anything $$$ money and politics will buy anything, especially junk science... sporadic ffi and sporadic gss ;
 
NOT THIS CASE !!! but another one a while back in Texas...see ;
 
We report a case of a 33-year-old female who died of a prion disease for whom the diagnosis of sFI or FFI was not considered clinically. Following death of this patient, an interview with a close family member indicated the patient's illness included a major change in her sleep pattern, corroborating the reported autopsy diagnosis of sFI.
 
 
sporadic FFI or nvCJD Texas style ???
 
 
Creutzfeldt-Jakob Disease Surveillance in Texas
 
 
Sunday, July 11, 2010
 
CJD or prion disease 2 CASES McLennan County Texas population 230,213 both cases in their 40s
 
 
 
2009 UPDATE ON ALABAMA AND TEXAS MAD COWS 2005 and 2006
 
 
Thursday, August 12, 2010
 
Seven main threats for the future linked to prions
 
***Also, a link is suspected between atypical BSE and some apparently sporadic cases of Creutzfeldt-Jakob disease in humans. These atypical BSE cases constitute an unforeseen first threat that could sharply modify the European approach to prion diseases.
 
Second threat
 
snip...
 
 
Monday, October 10, 2011
 
EFSA Journal 2011 The European Response to BSE: A Success Story
 
snip...
 
*** but the possibility that a small proportion of human cases so far classified as "sporadic" CJD are of zoonotic origin could not be excluded. Moreover, transmission experiments to non-human primates suggest that some TSE agents in addition to Classical BSE prions in cattle (namely L-type Atypical BSE, Classical BSE in sheep, transmissible mink encephalopathy (TME) and chronic wasting disease (CWD) agents) might have zoonotic potential.
 
snip...
 
 
 
***In addition, non-human primates are specifically susceptible for atypical BSE as demonstrated by an approximately 50% shortened incubation time for L-type BSE as compared to C-type. Considering the current scientific information available, it cannot be assumed that these different BSE types pose the same human health risks as C-type BSE or that these risks are mitigated by the same protective measures.
 
 
Monday, May 19, 2014
 
Variant CJD: 18 years of research and surveillance
 
 
Terry S. Singeltary Sr. on the Creutzfeldt-Jakob Disease Public Health Crisis
 
 
 
 
full text with source references ;
 
 
re-Human Prion Diseases in the United States
 
Posted by flounder on 01 Jan 2010 at 18:11 GMT
 
 
Views & Reviews
 
Monitoring the occurrence of emerging forms of Creutzfeldt-Jakob disease in the United States
 
Ermias D. Belay, MD, Ryan A. Maddox, MPH, Pierluigi Gambetti, MD and Lawrence B. Schonberger, MD
 
+ Author Affiliations
 
From the Division of Viral and Rickettsial Diseases (Drs. Belay and Schonberger and R.A. Maddox), National Center for Infectious Diseases, Centers for Disease Control and Prevention, Atlanta, GA; and National Prion Disease Pathology Surveillance Center (Dr. Gambetti), Division of Neuropathology, Institute of Pathology, Case Western Reserve University, Cleveland, OH.
 
Address correspondence and reprint requests to Dr. Ermias D. Belay, 1600 Clifton Road, Mailstop A-39, Atlanta, GA 30333.
 
 
26 March 2003
 
Terry S. Singeltary, retired (medically) CJD WATCH
 
I lost my mother to hvCJD (Heidenhain Variant CJD). I would like to comment on the CDC's attempts to monitor the occurrence of emerging forms of CJD. Asante, Collinge et al [1] have reported that BSE transmission to the 129-methionine genotype can lead to an alternate phenotype that is indistinguishable from type 2 PrPSc, the commonest sporadic CJD. However, CJD and all human TSEs are not reportable nationally. CJD and all human TSEs must be made reportable in every state and internationally. I hope that the CDC does not continue to expect us to still believe that the 85%+ of all CJD cases which are sporadic are all spontaneous, without route/source. We have many TSEs in the USA in both animal and man. CWD in deer/elk is spreading rapidly and CWD does transmit to mink, ferret, cattle, and squirrel monkey by intracerebral inoculation. With the known incubation periods in other TSEs, oral transmission studies of CWD may take much longer. Every victim/family of CJD/TSEs should be asked about route and source of this agent. To prolong this will only spread the agent and needlessly expose others. In light of the findings of Asante and Collinge et al, there should be drastic measures to safeguard the medical and surgical arena from sporadic CJDs and all human TSEs. I only ponder how many sporadic CJDs in the USA are type 2 PrPSc?
 
 
Diagnosis and Reporting of Creutzfeldt-Jakob Disease Singeltary, Sr et al. JAMA.2001; 285: 733-734. Vol. 285 No. 6, February 14, 2001 JAMA
 
Diagnosis and Reporting of Creutzfeldt-Jakob Disease
 
To the Editor: In their Research Letter, Dr Gibbons and colleagues1 reported that the annual US death rate due to Creutzfeldt-Jakob disease (CJD) has been stable since 1985. These estimates, however, are based only on reported cases, and do not include misdiagnosed or preclinical cases. It seems to me that misdiagnosis alone would drastically change these figures. An unknown number of persons with a diagnosis of Alzheimer disease in fact may have CJD, although only a small number of these patients receive the postmortem examination necessary to make this diagnosis. Furthermore, only a few states have made CJD reportable. Human and animal transmissible spongiform encephalopathies should be reportable nationwide and internationally.
 
Terry S. Singeltary, Sr Bacliff, Tex
 
1. Gibbons RV, Holman RC, Belay ED, Schonberger LB. Creutzfeldt-Jakob disease in the United States: 1979-1998. JAMA. 2000;284:2322-2323. FREE FULL TEXT
 
 
 
2 January 2000
 
British Medical Journal
 
U.S. Scientist should be concerned with a CJD epidemic in the U.S., as well
 
 
15 November 1999
 
British Medical Journal
 
vCJD in the USA * BSE in U.S.
 
 
Saturday, January 2, 2010
 
Human Prion Diseases in the United States January 1, 2010 ***FINAL***
 
 
14th ICID International Scientific Exchange Brochure -
 
Final Abstract Number: ISE.114
 
Session: International Scientific Exchange
 
Transmissible Spongiform encephalopathy (TSE) animal and human TSE in North America update October 2009
 
T. Singeltary
 
Bacliff, TX, USA
 
Background:
 
An update on atypical BSE and other TSE in North America. Please remember, the typical U.K. c-BSE, the atypical l-BSE (BASE), and h-BSE have all been documented in North America, along with the typical scrapie's, and atypical Nor-98 Scrapie, and to date, 2 different strains of CWD, and also TME. All these TSE in different species have been rendered and fed to food producing animals for humans and animals in North America (TSE in cats and dogs ?), and that the trading of these TSEs via animals and products via the USA and Canada has been immense over the years, decades.
 
Methods:
 
12 years independent research of available data
 
Results:
 
I propose that the current diagnostic criteria for human TSEs only enhances and helps the spreading of human TSE from the continued belief of the UKBSEnvCJD only theory in 2009. With all the science to date refuting it, to continue to validate this old myth, will only spread this TSE agent through a multitude of potential routes and sources i.e. consumption, medical i.e., surgical, blood, dental, endoscopy, optical, nutritional supplements, cosmetics etc.
 
Conclusion:
 
I would like to submit a review of past CJD surveillance in the USA, and the urgent need to make all human TSE in the USA a reportable disease, in every state, of every age group, and to make this mandatory immediately without further delay. The ramifications of not doing so will only allow this agent to spread further in the medical, dental, surgical arena's. Restricting the reporting of CJD and or any human TSE is NOT scientific. Iatrogenic CJD knows NO age group, TSE knows no boundaries. I propose as with Aguzzi, Asante, Collinge, Caughey, Deslys, Dormont, Gibbs, Gajdusek, Ironside, Manuelidis, Marsh, et al and many more, that the world of TSE Transmissible Spongiform Encephalopathy is far from an exact science, but there is enough proven science to date that this myth should be put to rest once and for all, and that we move forward with a new classification for human and animal TSE that would properly identify the infected species, the source species, and then the route.
 
 
*** Singeltary reply ; Molecular, Biochemical and Genetic Characteristics of BSE in Canada Singeltary reply ;
 
 
re-Human Prion Diseases in the United States
 
Posted by flounder on 01 Jan 2010 at 18:11 GMT
 
I kindly disagree with your synopsis for the following reasons ;
 
snip...
 
I would kindly like to add to my initial concerns, something I brought up years ago, and I believe that still hold true today, more so even than when I first stated these concerns in 2003 ;
 
routine passive mortality CJD surveillance USA ?
 
THIS has been proven not to be very useful in the U.K.;
 
THE EPIDEMIOLOGY OF CJD RG WILL 1984 (182 PAGES)
 
snip...
 
One reason for this was the _inaccuracy_ in coding of cases correctly certified as CJD Coding is carried out by staff who are not medically qualified and it is not surprising that coding errors occur in the processing of large numbers of certificates. In 1982, 12,000 certificates per week were processed at the office of population censuses and surveys by 15 coders and 6 checkers (Alderson et al., 1983). The occurrence of both inter- and intra-observer coding errors has been described (Curb et al., 1983) and the _inaccuracies_ of BOTH certification and coding discovered in this study _support_ the introduction of a more accurate system of death certificates and a more detailed and specific coding system...
 
snip...
 
 
Draft Proposal For The Monitoring of Creutzfeldt-Kakob Disease 1989 Dr. R. Will
 
snip...
 
IDENTIFICATION OF CASES
 
Cases of CJD may be identified from death certificates, but this alone is unlikely to provide adequate monitoring. ERRORS are made in certification and diagnosis; in the Oxford study death certificates were obtained on a series of known confirmed cases and CJD was mentioned in only 66% of certificates. In another series of 175 certified cases, 42 patients were judged not to have suffered from CJD after examination of case notes (7)...
 
full text;
 
 
snip...see my full text submission here ;
 
re-Human Prion Diseases in the United States
 
Posted by flounder on 01 Jan 2010 at 18:11 GMT
 
 
2001-2002ish
 
greetings TSE PRION WORLD,
 
i am reminded of a few things deep throat told me years ago;
 
*** The most frightening thing I have read all day is the report of Gambetti's finding of a new strain of sporadic cjd in young people.........
 
Dear God, what in the name of all that is holy is that!!! If the US has different strains of scrapie..... why???? than the UK... then would the same mechanisms that make different strains of scrapie here make different strains of BSE... if the patterns are different in sheep and mice for scrapie..... could not the BSE be different in the cattle, in the mink, in the humans....... I really think the slides or tissues and everything from these young people with the new strain of sporadic cjd should be put up to be analyzed by many, many experts in cjd........ bse..... scrapie
 
Scrape the damn slide and put it into mice..... wait..... chop up the mouse brain and and spinal cord........ put into some more mice..... dammit amplify the thing and start the damned research..... This is NOT rocket science... we need to use what we know and get off our butts and move.... the whining about how long everything takes..... well it takes a whole lot longer if you whine for a year and then start the research!!!
 
Not sure where I read this but it was a recent press release or something like that: I thought I would fall out of my chair when I read about how there was no worry about infectivity from a histopath slide or tissues because they are preserved in formic acid, or formalin or formaldehyde..... for God's sake........ Ask any pathologist in the UK what the brain tissues in the formalin looks like after a year....... it is a big fat sponge... the agent continues to eat the brain ...... you can't make slides anymore because the agent has never stopped........ and the old slides that are stained with Hemolysin and Eosin...... they get holier and holier and degenerate and continue... what you looked at 6 months ago is not there........ Gambetti better be photographing every damned thing he is looking at.....
 
***Okay, you need to know. You don't need to pass it on as nothing will come of it and there is not a damned thing anyone can do about it. Don't even hint at it as it will be denied and laughed at.......... USDA is gonna do as little as possible until there is actually a human case in the USA of the nvcjd........ if you want to move this thing along and shake the earth.... then we gotta get the victims families to make sure whoever is doing the autopsy is credible, trustworthy, and a saint with the courage of Joan of Arc........ I am not kidding!!!! so, unless we get a human death from EXACTLY the same form with EXACTLY the same histopath lesions as seen in the UK nvcjd........ forget any action........ it is ALL gonna be sporadic!!! And, if there is a case....... there is gonna be every effort to link it to international travel, international food, etc. etc. etc. etc. etc. They will go so far as to find out if a sex partner had ever traveled to the UK/europe, etc. etc. .... It is gonna be a long, lonely, dangerous twisted journey to the truth. They have all the cards, all the money, and are willing to threaten and carry out those threats.... and this may be their biggest downfall...***
 
Thanks as always for your help. (Recently had a very startling revelation from a rather senior person in government here.......... knocked me out of my chair........ you must keep pushing. If I was a power person.... I would be demanding that there be at least a million bovine tested as soon as possible and agressively seeking this disease. The big players are coming out of the wood work as there is money to be made!!!
 
In short: "FIRE AT WILL"!!! for the very dumb.... who's "will"! "Will be the burden to bare if there is any coverup!"
 
again it was said years ago and it should be taken seriously.... BSE will NEVER be found in the US!
 
As for the BSE conference call... I think you did agreat service to freedom of information and making some people feign integrity... I find it scary to see that most of the "experts" are employed by the federal government or are supported on the "teat" of federal funds. A scary picture! I hope there is a confidential panel organized by the new government to really investigate this thing.
 
You need to watch your back........ but keep picking at them....... like a buzzard to the bone... you just may get to the truth!!! (You probably have more support than you know. Too many people are afraid to show you or let anyone else know. I have heard a few things myself... you ask the questions that everyone else is too afraid to ask.)
 
================================================
 
*** U.S.A. 50 STATE BSE MAD COW CONFERENCE CALL Jan. 9, 2001 SINGELTARY HACKS IN (DEEP THROAT ABOVE HELPED ME GET IN)
 
 
 
Mad cow disease: Could it be here?
 
Man's stubborn crusade attracts experts' notice By Carol Christian | August 5, 2001
 
 
yes, cjd is popping up in more and more places it seems. I think in 55 year and older, it's now 1 in 9,000.
 
see ;
 
lifetime risk of developing sporadic CJD is about 1 in 30,000, jumps to 1 in 9,000 in 50 years of age and above
 
IN REALITY, sporadic CJD is 1 in 9,000 in 50 years of age and above, and that's with a inadequate or what I call passive surveillance system. see below ;
 
Dr. William Shulaw, a veterinarian with The Ohio State University extension service, is involved in a nationwide program to eradicate scrapie, the form of BSE found in sheep.
 
Shulaw said the chances of a person getting sporadic Creutzfeldt- Jakob disease is about one in a million. But that's the total population, infants, children, adults and the elderly.
 
Chances increase to one in 9,000 when the group is restricted to those age 50 and older.
 
 
 
 
Monday, June 02, 2014
 
Confirmed Human BSE aka mad cow Variant CJD vCJD or nvCJD Case in Texas
 
fact is, BSE cases in Europe of the past years have dropped dramatically due to feed ban that was enforced, and extensive BSE testing, in large numbers. just the opposite has happened in the USA. it’s all been documented. there is ample evidence that there is as much of a chance (if not more), that this victim contracted human mad cow disease from sources right here in the USA. this PR push to alienate a USA source factor for human BSE in the USA is a PR stunt by the USDA inc., and not justified now, in my opinion. compare BSE testing figures in the EU compared to the USA, compare mad cow feed ban breaches, and you will see. hell, the 2004 enhanced BSE surveillance program was flawed so bad, the top Prion God at the NIH TSE prion expert Paul Brown, says he does not trust anything from the USDA since Texas covered up a mad cow for 7 months, on a 48 hour confirmation turn around. it’s all documented below in link. USDA inc shut down the mad cow testing after so many atypical BSE cases started showing up.
 
yes, another foreigner comes to the USA, or another USA citizens does some traveling, and all of a sudden, it’s a foreign disease. evidently, these folks never eat anything in the USA, and contracts nvcjd. right. just like the last one in 2005. really? here are the facts of the TEXAS MAD COW, MAD COW FEED in Texas, CJD CLUSTER in Texas, CJD CASE IN 38 YEAR OLD WOMAN THAT APPARENTLY WORKED ON THE SLAUGHTER LINE FOR TYSON in Texas, AND OTHER STRANGE TSE PRION DISEASE IN VERY YOUNG VICTIMS HERE IN TEXAS with long duration of illness from onset of clinical symptoms to death, CALLED SPORADIC FFI (except it is not linked to any genetic make up of that family), another nvcjd victim back in 2005 in Texas, apparently another UK victim that had moved to Texas, and never ate anything. these are the facts as I have come to know them (official documents), since hvcjd took my mom in December of 1997. just made a promise to mom, never forget, and never let them forget, the rest of the story, the truth you don’t hear about. ...our fine federal friends and the USDA inc, has lied to all of us...
 
CONFIRMED HUMAN BSE AKA MAD COW DISEASE vCJD TEXAS USA
 
Monday, June 02, 2014 Confirmed Variant CJD Case in Texas
 
 
Wednesday, September 10, 2014
 
*** Creutzfeldt-Jakob disease (CJD) biannual update (August 2014), with updated guidance on decontamination of gastrointestinal endoscopy equipment ***
 
Research and analysis
 
 
Monday, July 28, 2014
 
Mitigating the Risk of Transmission and Environmental Contamination of Transmissible Spongiform Encephalopathies 2013 Annual Report
 
 
Thursday, October 25, 2012
 
Current limitations about the cleaning of luminal endoscopes and TSE prion risk factors there from
 
Article in Press
 
 
Saturday, January 16, 2010
 
Evidence For CJD TSE Transmission Via Endoscopes 1-24-3 re-Singeltary to Bramble et al
 
Evidence For CJD/TSE Transmission Via Endoscopes
 
From Terry S. Singletary, Sr flounder@wt.net 1-24-3
 
Terry S. Singeltary Sr., P.O. BOX 42, Bacliff, Texas 77518 USA
 
 
Professor Michael Farthing wrote:
 
Louise Send this to Bramble (author) for a comment before we post. Michael
 
snip...
 
Evidence For CJD/TSE Transmission Via Endoscopes
 
From Terry S. Singletary, Sr flounder@wt.net 1-24-3
 
I have researched human/animal TSEs now for over 5 years due to the death of my Mother from the Heidenhain Variant Creutzfeldt Jakob disease, one of six - known - variants of the infamous 'sporadic' CJD.
 
I did a little survey several years ago about CJD and ENDOSCOPY in 2001, and then went there again when another article was released recently. However, they seemed to only be concerned with the vCJD strain and risk from endoscopy equipment.
 
My concerns are if vCJD can be transmitted by blood, and there are now 6 variants of the infamous sporadic CJDs that they are documenting to date, how do they know that none of these 6 variants will not transmit the agent (prion) via blood?...especially since the sporadic CJDs are the only ones documented to date to transmit via the surgical arena and now that the CWD is spreading more and more, who knows about the cattle?
 
I would always read this study and it would bring me back to reality as to how serious/dangerous this agent is in the surgical/medical arena. You might want to read this short abstract from the late, great Dr. Gibbs twice, and let it really sink in. And please remember while reading some of these transmission studies, that most all, if not ALL these agents transmit freely to primates. Humans, of course, are primates.
 
Regarding claims that:
 
'Well, it has never been documented to transmit to humans."
 
There are two critical factors to think about:
 
A. CJD/TSEs in the USA are NOT reportable in most states and there is NO CJD/TSE questionnaire for most victims and their families, and the one they are now issuing asks absolutely nothing about route and source of the (prion) agent, only how the disease was diagnosed. Furthermore, the elderly are only very rarely autopsied, ie looking for Alzheimer's or 'FAST Alzheimer's' OR prion disease-related factors and phenomena, such as heart failure caused by disease.
 
B. It is unethical and against the law to do transmission studies of TSEs to humans, they are 100% FATAL.
 
I suggest you read these case studies about medical arena CJD transmission very carefully:
 
1: J Neurol Neurosurg Psychiatry 1994 Jun;57(6):757-8
 
Transmission of Creutzfeldt-Jakob disease to a chimpanzee by electrodes contaminated during neurosurgery.
 
Gibbs CJ Jr, Asher DM, Kobrine A, Amyx HL, Sulima MP, Gajdusek DC.
 
Laboratory of Central Nervous System Studies, National Institute of Neurological Disorders and Stroke, National Institutes of Health, Bethesda, MD 20892.
 
Stereotactic multicontact electrodes used to probe the cerebral cortex of a middle aged woman with progressive dementia were previously implicated in the accidental transmission of Creutzfeldt-Jakob disease (CJD) to two younger patients. The diagnoses of CJD have been confirmed for all three cases. More than two years after their last use in humans, after three cleanings and repeated sterilisation in ethanol and formaldehyde vapour, the electrodes were implanted in the cortex of a chimpanzee. Eighteen months later the animal became ill with CJD. This finding serves to re-emphasise the potential danger posed by reuse of instruments contaminated with the agents of spongiform encephalopathies, even after scrupulous attempts to clean them.
 
 
Tissue Infectivity and TSEs (brain = high / rectum = medium)
 
snip...see full text ;
 
Evidence For CJD TSE Transmission Via Endoscopes 1-24-3
 
re-Singeltary to Bramble et al
 
Evidence For CJD/TSE Transmission Via Endoscopes
 
From Terry S. Singletary, Sr flounder@wt.net 1-24-3
 
 
Monday, August 18, 2014
 
Barriers to Creutzfeldt-Jakob Disease Autopsies, California and the USA
 
 
Science and Technology Committee Oral evidence: Blood, tissue and organ screening, HC 990 Wednesday 5 March 2014 SPORADIC CJD
 
Science and Technology Committee
 
Oral evidence: Blood, tissue and organ screening, HC 990 Wednesday 5 March 2014
 
Ordered by the House of Commons to be published on 5 March 2014.
 
Written evidence from witnesses:
 
– Prometic BioSciences Ltd
 
– Terumo BCT
 
– Prionics AG
 
– DuPont Chemicals and Fluoroproducts
 
Watch the meeting
 
Members present: Andrew Miller (Chair); Jim Dowd; David Heath; Stephen Metcalfe; David Morris; Stephen Mosley; Pamela Nash; Sarah Newton; Graham Stringer; David Tredinnick
 
snip...
 
Professor Collinge: I think there is some misunderstanding there. The apparent incidence of sporadic CJD is increasing. There are about a hundred new cases a year at the moment, and that has gone up substantially from the start of surveillance. That is thought, principally, to reflect better diagnosis of the disease, although that may not be all the explanation. It is possible, and we can talk about that, that some of that may be BSE-related. However, I think that the specific confusion there is that people talk about sporadic CJD occurring at 1 per million. That is not your individual risk. Your risk is 1 per million every year. Actually, it is nearer 2 per million per year of the population will develop sporadic CJD, but your lifetime risk of developing sporadic CJD is about 1 in 30,000. So that has not really changed. When people talk about 1 per million, often they interpret that as thinking it is incredibly rare. They think they have a 1-in-a-million chance of developing this disease. You haven’t. You’ve got about a 1-in-30,000 chance of developing it.
 
snip...
 
Q112 Graham Stringer: Professor Collinge, the last time you were here you said that you believed that the risk of transmission of vCJD via surgical instruments was a significant problem. If that is the case, why have no cases been reported where vCJD has been transmitted in that particular way?
 
Professor Collinge: What I was trying to say is that there is clearly a risk there. We have not been able to quantify that risk. It is correct that we don’t know of any cases of secondary vCJD that can be attributed to surgical instruments at this point in time, but it can’t be excluded that some of the cases we have seen so far of vCJD have been related to that. It is a question of how you would tell. That really comes from epidemiological studies. Since a history of prior surgery—particularly a history of prior dental surgery—is so common, and many patients you see will have had prior surgery and certainly virtually all of them will have had dentistry, it is hard to make that link. The link has been made with sporadic CJD where there have been clearly documented instances of transmission of prions by surgical instruments, by neurosurgical instruments. There is also epidemiological evidence from several countries now that patients developing classical CJD are more likely to have had abdominal surgery beforehand, for example. There are sufficient numbers where an epidemiological link has been shown. You are right—we don’t have definite evidence that a surgically transmitted case of vCJD has occurred, but that is, perhaps, not surprising at this point in time.
 
What we do have is a great deal of scientific evidence that, first of all, the infective agent in vCJD is much more widely distributed in the body than it is in classical CJD, so there is more opportunity for it to contact surgical and medical instruments. Of course, as you heard from the HPA study, there are, potentially, quite a lot of people carrying the infection around. We also know, experimentally, that it is relatively easy to transmit prions by binding them to metal surfaces. In fact, it is something that we use experimentally now. It is a very efficient way of transmitting the disease. Indeed, the blood test that we talked about earlier exploits the affinity of prions on metal surfaces as a way of concentrating the infective agent prior to detection. There is plenty of scientific evidence that this would be an efficient route of transmission. There is plenty of evidence that there are many people in the UK incubating the disease and that the infection would be in tissues that come into contact with surgical instruments. But you are right: so far, there is not a documented example of that happening with vCJD.
 
 
Wednesday, December 11, 2013
 
*** Detection of Infectivity in Blood of Persons with Variant and Sporadic Creutzfeldt-Jakob Disease ***
 
 
Sunday, March 09, 2014
 
*** A Creutzfeldt-Jakob Disease (CJD) Lookback Study: Assessing the Risk of Blood Borne Transmission of Classic Forms of Creutzfeldt-Jakob Disease
 
FDA TSEAC CIRCUS AND TRAVELING ROAD SHOW FOR THE TSE PRION DISEASES
 
 
Thursday, January 2, 2014
 
*** CWD TSE Prion in cervids to hTGmice, Heidenhain Variant Creutzfeldt-Jacob Disease MM1 genotype, and iatrogenic CJD ??? ***
 
 
Thursday, February 20, 2014
 
*** Unnecessary precautions BSE MAD COW DISEASE Dr. William James FSIS VS Dr. Linda Detwiler 2014
 
 
Saturday, February 01, 2014
 
*** vCJD With Extremely Low Lymphoreticular Deposition of Prion Protein MAY NOT HAVE BEEN DETECTABLE
 
 
Monday, October 14, 2013
 
*** Researchers estimate one in 2,000 people in the UK carry variant CJD proteins ***
 
 
Wednesday, December 11, 2013
 
*** Detection of Infectivity in Blood of Persons with Variant and Sporadic Creutzfeldt-Jakob Disease ***
 
 
Thursday, March 6, 2014
 
*** TEXAS RECALL LIST MASSIVE FROM DEAD STOCK DOWNER CANCER COWS OFFAL from Class I Recall 002-2014 and 013-2014 Health Risk: High Jan 13, 2014 and Feb 8, 2014 shipped to Texas, Florida, and Illinois UPDATE FEBRUARY 14, 2014 ***
 
 
WHAT about the sporadic CJD TSE proteins ?
 
WE now know that some cases of sporadic CJD are linked to atypical BSE and atypical Scrapie, so why are not MORE concerned about the sporadic CJD, and all it’s sub-types $$$
 
Creutzfeldt-Jakob Disease CJD cases rising North America updated report August 2013
 
*** Creutzfeldt-Jakob Disease CJD cases rising North America with Canada seeing an extreme increase of 48% between 2008 and 2010 ***
 
 
Sunday, October 13, 2013
 
*** CJD TSE Prion Disease Cases in Texas by Year, 2003-2012
 
 
Friday, January 10, 2014
 
vpspr, sgss, sffi, TSE, an iatrogenic by-product of gss, ffi, familial type prion disease, what it ???
 
 
Monday, October 10, 2011
 
EFSA Journal 2011 The European Response to BSE: A Success Story
 
snip...
 
EFSA and the European Centre for Disease Prevention and Control (ECDC) recently delivered a scientific opinion on any possible epidemiological or molecular association between TSEs in animals and humans (EFSA Panel on Biological Hazards (BIOHAZ) and ECDC, 2011). This opinion confirmed Classical BSE prions as the only TSE agents demonstrated to be zoonotic so far *** but the possibility that a small proportion of human cases so far classified as "sporadic" CJD are of zoonotic origin could not be excluded. Moreover, transmission experiments to non-human primates suggest that some TSE agents in addition to Classical BSE prions in cattle (namely L-type Atypical BSE, Classical BSE in sheep, transmissible mink encephalopathy (TME) and chronic wasting disease (CWD) agents) might have zoonotic potential.
 
snip...
 
 
 
Thursday, August 12, 2010
 
Seven main threats for the future linked to prions First threat The TSE road map defining the evolution of European policy for protection against prion diseases is based on a certain numbers of hypotheses some of which may turn out to be erroneous. In particular, a form of BSE (called atypical Bovine Spongiform Encephalopathy), recently identified by systematic testing in aged cattle without clinical signs, may be the origin of classical BSE and thus potentially constitute a reservoir, which may be impossible to eradicate if a sporadic origin is confirmed.
 
***Also, a link is suspected between atypical BSE and some apparently sporadic cases of Creutzfeldt-Jakob disease in humans. These atypical BSE cases constitute an unforeseen first threat that could sharply modify the European approach to prion diseases.
 
Second threat
 
snip...
 
 
*** The potential impact of prion diseases on human health was greatly magnified by the recognition that interspecies transfer of BSE to humans by beef ingestion resulted in vCJD. While changes in animal feed constituents and slaughter practices appear to have curtailed vCJD, there is concern that CWD of free-ranging deer and elk in the U.S. might also cross the species barrier. Thus, consuming venison could be a source of human prion disease. Whether BSE and CWD represent interspecies scrapie transfer or are newly arisen prion diseases is unknown. Therefore, the possibility of transmission of prion disease through other food animals cannot be ruled out. There is evidence that vCJD can be transmitted through blood transfusion. There is likely a pool of unknown size of asymptomatic individuals infected with vCJD, and there may be asymptomatic individuals infected with the CWD equivalent. These circumstances represent a potential threat to blood, blood products, and plasma supplies.
 
 
Monday, March 10, 2014
 
Docket No. 00-108-10 Chronic Wasting Disease Herd Certification Program and Interstate Movement of Farmed or Captive Deer, Elk, and Moose; Program Standards
 
Singeltary Submission
 
 
BSE prions propagate as either variant CJD-like or sporadic CJD-like prion strains in transgenic mice expressing human prion protein
 
Variant Creutzfeldt–Jakob disease (vCJD) has been recognized to date only in individuals homozygous for methionine at PRNP codon 129. Here we show that transgenic mice expressing human PrP methionine 129, inoculated with either bovine spongiform encephalopathy (BSE) or variant CJD prions, may develop the neuropathological and molecular phenotype of vCJD, consistent with these diseases being caused by the same prion strain. Surprisingly, however, BSE transmission to these transgenic mice, in addition to producing a vCJD-like phenotype, can also result in a distinct molecular phenotype that is indistinguishable from that of sporadic CJD with PrPSc type 2. These data suggest that more than one BSE-derived prion strain might infect humans; it is therefore possible that some patients with a phenotype consistent with sporadic CJD may have a disease arising from BSE exposure.
 
 
Wednesday, December 4, 2013
 
Bovine Spongiform Encephalopathy; Importation of Bovines and Bovine Products; Final Rule Federal Register / Vol. 78 , No. 233 / Wednesday, December 4, 2013
 
TO ALL IMPORTING COUNTRIES THAT IMPORTS FROM THE USA, BE WARNED, NEW MAD COW BSE REGULATIONS USDA, AND OIE, not worth the paper the regulations were wrote on, kind of like the mad cow feed ban of August 1997, nothing but ink on paper $$$
 
full text ;
 
 
Monday, December 02, 2013
 
*** A parliamentary inquiry has been launched today into the safety of blood, tissue and organ screening following fears that vCJD – the human form of ‘mad cow’ disease – may be being spread by medical procedures
 
 
U.S.A. 50 STATE BSE MAD COW CONFERENCE CALL Jan. 9, 2001
 
 
 
Aug. 5, 2001, 12:25AM
 
Mad cow disease: Could it be here?
 
Man's stubborn crusade attracts experts' notice
 
By CAROL CHRISTIAN Copyright 2001 Houston Chronicle
 
Like Paul Revere with e-mail, Terry Singeltary Sr. is on a mission to sound an alarm: Beware of mad cow disease.
 
As is true of many crusaders, however, his pleas often fall on deaf ears. Health officials here and abroad insist that bovine spongiform encephalopathy -- popularly known as mad cow disease, a fatal brain disorder that can make cows shake uncontrollably -- has been kept out of this country through surveillance of the cattle industry.
 
But since his mother's death in December 1997, the Galveston County man has been obsessed with possible connections between her deadly brain disorder, sporadic Creutzfeldt-Jakob Disease, and mad cow disease.
 
And after much persistence on his part, people are taking notice of this former machinist and high school dropout who jokes that he has a Ph.D. -- a Pool Hall Degree.
 
"They called me Chicken Little for four years," he said. "Now they're calling back, asking for more information."
 
For the past year he has been U.S. co-coordinator of an international monitoring group called CJD Watch. He regularly gets e-mail from scientists and journalists around the world.
 
Debora MacKenzie, a reporter for the British magazine New Scientist, described Singeltary, 47, as a "dogged unearther and tabulator of government documents." Singeltary monitors "every word written about CJD/BSE," said Anita Manning of USA Today, also by e-mail.
 
"He's passionate, opinionated and not always tactful, although I like him because he's such a character and he is so transparent," Manning said. "He is what he appears to be."
 
Science and environment writer Jonathan Leake of the Sunday Times in London said Singeltary has helped him track down families of people with CJD along with academic research papers.
 
"I strongly suspect he is right in thinking the USA has had BSE cases," Leake said by e-mail.
 
"The American government is making the same mistake as the British in putting the short-term commercial interests of its farmers before health considerations," he added.
 
"It should start formal and widespread testing of cattle plus compulsory autopsies for all human CJD victims at the state's expense. If there is BSE, then leaving it to spread will kill people -- and that would eventually destroy the industry, too."
 
Texas Department of Health epidemiologist Julie Rawlings said Singeltary's careful monitoring of the disease had proven useful.
 
"Terry has been helpful in providing contact information regarding suspect CJD cases so that the Health Department can initiate case investigations and learn more about CJD in Texas," she said.
 
Noting that the department cannot release records on individual patients, she added, "I think we learn more from him than he does from us."
 
Mad cow disease surfaced in England in 1986 and quickly became an epidemic. It since has been reported in 15 European countries, most recently Greece on July 2, and the Czech Republic on June 14. Two German-born cows tested positive for BSE in November.
 
Singeltary said he became convinced that BSE is here as he watched his mother, Barbara Poulter of Crystal Beach, dying of sporadic Creutzfeldt-Jakob Disease. The rare, fatal brain disease is sometimes accompanied by severe jerking.
 
"She would jerk so bad at times, it would take three of us to hold her down," Singeltary said. "They can call it whatever they want, but I know what I saw, and what she went through. `Sporadic' simply means they don't know."
 
Poulter, a retired telephone-company field worker, had a form of sporadic CJD -- Haidenhain variant -- that is even less common than the typical sporadic case. One of its first symptoms is loss of vision.
 
She started seeing brown spots in September 1997 and was virtually blind within two weeks. By the eighth week of the illness Poulter was bedridden, and in the 10th week she died. Before that she had been in good health.
 
In many countries and most U.S. states, physicians are not required to report CJD cases to health officials. Texas made the disease reportable in 1998. Through 2000, there were 17 probable or confirmed cases, according to the Texas Department of Health.
 
In mid-June, a case of sporadic CJD was confirmed through brain biopsy at Christus Spohn Hospital Shoreline in Corpus Christi, said Jane Bakos, hospital vice president. The patient has since died, the hospital reported.
 
CJD and mad cow disease leave their victims' brains full of holes like a sponge.
 
Although not contagious, the illnesses are thought to be transmissible through prions, or nearly indestructible abnormal proteins.
 
Because the prion protein is not killed by standard sterilization, sporadic CJD can be spread by contaminated surgical instruments.
 
In March 1996, the British government announced the discovery of a new variant of CJD, most likely explained by exposure to bovine spongiform encephalopathy.
 
Through June, 101 cases of new-variant CJD have been reported in the United Kingdom, three in France and one in Ireland. In contrast to sporadic CJD, the new variant usually affects younger patients and lasts longer.
 
No cases of new-variant CJD or BSE have been reported in the United States. No relationship has been shown between sporadic CJD and mad cow disease.
 
There is no indication that new-variant CJD can be spread through blood transfusions, but a U.S. Food and Drug Administration advisory committee voted in June to broaden the categories for excluding potential donors. The recommendations have not yet been approved by the FDA.
 
The American Red Cross has announced that on Sept. 17 it will begin rejecting potential blood donors who, since 1980, have spent at least three months in the United Kingdom or at least six months in any European country or combination of countries. Those who have received a blood transfusion in Britain since 1980 also will be rejected.
 
The primary collector of local blood donations is the Gulf Coast Regional Blood Center, which will follow the FDA's guidelines, said Bill Teague, president and chief executive officer.
 
Singeltary said it's naive to think that U.S. prevention efforts have kept mad cow and new-variant CJD out of the United States.
 
"They haven't found it," he said, "because they haven't looked."
 
For one thing, he said, too few cows are tested for the disease. In the first six months of this year, the European Union tested more than 3.2 million cows, David Byrne of the European Commission said in a speech last month.
 
By contrast, it took the U.S. Department of Agriculture nearly 10 years to analyze about 13,000 cow brains, according to the department's Web site.
 
With more than 68 million cattle slaughtered since 1990 in the United States, according to the USDA, checking about 13,000 falls far short, Singeltary said.
 
Though not a scholar, Singeltary has collected voluminous material on mad cow and CJD. Disabled from a neck injury, Singeltary never used a computer until 1998.
 
He now spends hours each day on the Internet while his wife, Bonnie Singeltary, runs a flower shop in their home in Bacliff, in north Galveston County.
 
His challenge to the CJD/BSE establishment is courageous and refreshing, said Dr. Lynette Dumble, former visiting professor of surgery at University of Texas Medical School at Houston and a former senior research fellow in the history and philosophy of science at the University of Melbourne in Australia.
 
"I certainly have no problem with Terry's ideas on BSE/CJD," said Dumble, who coordinates the Global Sisterhood Network, a computer service that posts media reports on developments affecting women. "His research skills are excellent, and he is abreast of each and every development in the field."
 
Among Singeltary's worries now, he said, are widespread violations of an August 1997 ban on feeding animal products to U.S. cattle. The FDA reported in January that hundreds of feed manufacturers were not complying with regulations designed to keep BSE out of this country.
 
(That same month, a Purina Mills feedlot near San Antonio told the FDA that a "very low level" of cow parts had been found in cattle feed. The company voluntarily removed 1,222 animals who had been fed the prohibited materials.)
 
He obtained copies of FDA letters to various feed mills that had been found in violation of the regulations and immediately sent them by e-mail to hundreds of people around the world.
 
Singeltary might not be so zealous in getting the word out if he weren't convinced that someone is covering up the truth.
 
"They used to say BSE would never transmit to humans," he said, "and it has. They lied about the feed ban being in place.
 
"I've lost faith in the whole process. I've discovered too many things."
 
 
layperson
 
just made a promise, never forget, never let them forget...
 
MOM DOD 12/14/97 confirmed hvCJD Heidenhain Variant Creutzfeldt Jakob Disease Case Report
 
snip...
 
Heidenhain Variant Creutzfeldt Jakob Disease autopsy case report 'MOM' DIVISION OF NEUROPATHOLOGY University of Texas Medical Branch 114 McCullough Bldg. Galveston, Texas 77555-0785 FAX COVER SHEET DATE: 4-23-98 TO: Mr. Terry Singeltary @ ------- FROM: Gerald Campbell FAX: (409) 772-5315 PHONE: (409) 772-2881 Number of Pages (including cover sheet): Message: *CONFIDENTIALITY NOTICE* This document accompanying this transmission contains confidential information belonging to the sender that is legally privileged. This information is intended only for the use of the individual or entry names above. If you are not the intended recipient, you are hereby notified that any disclosure, copying distribution, or the taking of any action in reliances on the contents of this telefaxed information is strictly prohibited. If you received this telefax in error, please notify us by telephone immediately to arrange for return of the original documents.
 
--------------------------
 
Patient Account: 90000014-518 Med. Rec. No.: (0160)118511Q
 
Patient Name: POULTER, BARBARA
 
Age: 63
 
YRS DOB: 10/17/34
 
Sex: F
 
Admitting Race: C
 
Attending Dr.: Date / Time Admitted : 12/14/97 1228
 
Copies to: UTMB University of Texas Medical Branch Galveston, Texas 77555-0543 (409) 772-1238 Fax (409) 772-5683 Pathology Report
 
FINAL AUTOPSY DIAGNOSIS
 
Autopsy' Office (409)772-2858 Autopsy NO.: AU-97-00435 AUTOPSY INFORMATION:
 
Occupation: Unknown Birthplace: Unknown Residence: Crystal Beach Date/Time of Death: 12/14/97 13:30 Date/Time of Autopsy: 12/15/97 15:00 Pathologist/Resident: Pencil/Fernandez Service: Private Restriction:
 
Brain only FINAL AUTOPSY DIAGNOSIS I. Brain: Creutzfeldt-Jakob disease, Heidenhain variant. snip...see full text ;
 
 
2001-2002ish
 
greetings TSE PRION WORLD,
 
i am reminded of a few things deep throat told me years ago;
 
*** The most frightening thing I have read all day is the report of Gambetti's finding of a new strain of sporadic cjd in young people.........
 
Dear God, what in the name of all that is holy is that!!! If the US has different strains of scrapie..... why???? than the UK... then would the same mechanisms that make different strains of scrapie here make different strains of BSE... if the patterns are different in sheep and mice for scrapie..... could not the BSE be different in the cattle, in the mink, in the humans....... I really think the slides or tissues and everything from these young people with the new strain of sporadic cjd should be put up to be analyzed by many, many experts in cjd........ bse..... scrapie
 
Scrape the damn slide and put it into mice..... wait..... chop up the mouse brain and and spinal cord........ put into some more mice..... dammit amplify the thing and start the damned research..... This is NOT rocket science... we need to use what we know and get off our butts and move.... the whining about how long everything takes..... well it takes a whole lot longer if you whine for a year and then start the research!!!
 
Not sure where I read this but it was a recent press release or something like that: I thought I would fall out of my chair when I read about how there was no worry about infectivity from a histopath slide or tissues because they are preserved in formic acid, or formalin or formaldehyde..... for God's sake........ Ask any pathologist in the UK what the brain tissues in the formalin looks like after a year....... it is a big fat sponge... the agent continues to eat the brain ...... you can't make slides anymore because the agent has never stopped........ and the old slides that are stained with Hemolysin and Eosin...... they get holier and holier and degenerate and continue... what you looked at 6 months ago is not there........ Gambetti better be photographing every damned thing he is looking at.....
 
***Okay, you need to know. You don't need to pass it on as nothing will come of it and there is not a damned thing anyone can do about it. Don't even hint at it as it will be denied and laughed at.......... USDA is gonna do as little as possible until there is actually a human case in the USA of the nvcjd........ if you want to move this thing along and shake the earth.... then we gotta get the victims families to make sure whoever is doing the autopsy is credible, trustworthy, and a saint with the courage of Joan of Arc........ I am not kidding!!!! so, unless we get a human death from EXACTLY the same form with EXACTLY the same histopath lesions as seen in the UK nvcjd........ forget any action........ it is ALL gonna be sporadic!!! And, if there is a case....... there is gonna be every effort to link it to international travel, international food, etc. etc. etc. etc. etc. They will go so far as to find out if a sex partner had ever traveled to the UK/europe, etc. etc. .... It is gonna be a long, lonely, dangerous twisted journey to the truth. They have all the cards, all the money, and are willing to threaten and carry out those threats.... and this may be their biggest downfall...***
 
Thanks as always for your help. (Recently had a very startling revelation from a rather senior person in government here.......... knocked me out of my chair........ you must keep pushing. If I was a power person.... I would be demanding that there be at least a million bovine tested as soon as possible and agressively seeking this disease. The big players are coming out of the wood work as there is money to be made!!!
 
In short: "FIRE AT WILL"!!! for the very dumb.... who's "will"! "Will be the burden to bare if there is any coverup!"
 
again it was said years ago and it should be taken seriously.... BSE will NEVER be found in the US!
 
As for the BSE conference call... I think you did agreat service to freedom of information and making some people feign integrity... I find it scary to see that most of the "experts" are employed by the federal government or are supported on the "teat" of federal funds. A scary picture! I hope there is a confidential panel organized by the new government to really investigate this thing.
 
You need to watch your back........ but keep picking at them....... like a buzzard to the bone... you just may get to the truth!!! (You probably have more support than you know. Too many people are afraid to show you or let anyone else know. I have heard a few things myself... you ask the questions that everyone else is too afraid to ask.)
 
 
 
Thursday, April 24, 2014
 
Brazil investigates possible BSE mad cow case
 
 
 
Monday, May 5, 2014
 
Brazil BSE Mad Cow disease confirmed OIE 02/05/2014
 
 
 
Thursday, September 26, 2013
 
Brazil evaluate the implementation of health rules on animal by-products and derived products SRM BST TSE PRION aka MAD COW DISEASE
 
 
 
Friday, December 07, 2012
 
ATYPICAL BSE BRAZIL 2010 FINALLY CONFIRMED OIE 2012
 
 
 
Wednesday, December 19, 2012
 
Scientific Report of the European Food Safety Authority on the Assessment of the Geographical BSE Risk (GBR) of Brazil
 
 
 
 
 
Terry S. Singeltary Sr. xxx Bacliff, Texas USA 77518 flounder9@verizon.net