Thursday, June 24, 2010

Accumulation of L-type Bovine Prions in Peripheral Nerve Tissues

Volume 16, Number 7–July 2010


Accumulation of L-type Bovine Prions in Peripheral Nerve Tissues

Yoshifumi Iwamaru, Morikazu Imamura, Yuichi Matsuura, Kentaro Masujin, Yoshihisa Shimizu, Yujing Shu, Megumi Kurachi, Kazuo Kasai, Yuichi Murayama, Shigeo Fukuda, Sadao Onoe, Ken'ichi Hagiwara, Yoshio Yamakawa, Tetsutaro Sata, Shirou Mohri, Hiroyuki Okada, and Takashi Yokoyama Author affiliations: National Institute of Animal Health, Tsukuba, Ibaraki, Japan (Y. Iwamaru, M. Imamura, Y. Matsuura, K. Masujin, Y. Shimizu, Y. Shu, M. Kurachi, K. Kasai, Y. Murayama, S. Mohri, H. Okada, T. Yokoyama); Hokkaido Animal Research Center, Hokkaido, Japan (S. Fukuda, S. Onoe); and National Institute of Infectious Diseases, Tokyo, Japan (K. Hagiwara, Y. Yamakawa, T. Sata)

Suggested citation for this article

Abstract We recently reported the intraspecies transmission of L-type atypical bovine spongiform encephalopathy (BSE). To clarify the peripheral pathogenesis of L-type BSE, we studied prion distribution in nerve and lymphoid tissues obtained from experimentally challenged cattle. As with classical BSE prions, L-type BSE prions accumulated in central and peripheral nerve tissues.

Bovine spongiform encephalopathy (BSE) is a fatal neurodegenerative disorder of cattle characterized by accumulation of a protease-resistant form of a normal cellular prion protein (PrPres) in the central nervous system. The scientific literature in general has assumed that BSE in cattle is caused by a uniform strain (classical BSE). However, different neuropathologic and molecular phenotypes of BSE (atypical BSEs) have recently been reported from various countries (1). Recent data from Western blot analyses of field cases of atypical BSEs are characterized by a higher (H-type BSE) or lower (L-type BSE) molecular mass of the unglycosylated form of PrPres than is classical BSE (2). The origins of atypical BSEs remain obscure; unlike classical BSE, atypical BSE has been detected mainly in aged cattle and suggested a as possible sporadic form of BSE (3).

Several lines of evidence demonstrate that classical BSE and a variant form of Creutzfeldt-Jacob disease are most likely caused by the same agent (4,5). Transmission of classical BSE to humans has been proposed to result from ingestion of contaminated food. Whether atypical BSEs are transmissible to humans remains uncertain; however, human susceptibility to L-type BSEs is suggested by recent experimental transmission in primates (6) and mice transgenic for human prion protein (PrP) (7) by using the most effective route of intracerebral inoculations of prions. The L-type BSE prion is much more virulent in primates and in humanized mice than is the classical BSE prion, which suggests the possibility of zoonotic risk associated with the L-type BSE prion. These findings emphasize the critical importance of understanding tissue distribution of L-type BSE prions in cattle because, among the current administrative measures for BSE controls, the specified risk materials removal policy plays a crucial role in consumer protection.

In Japan, atypical BSE was detected in an aged Japanese Black cow (BSE/JP24) (8). We recently reported the successful transmission of BSE/JP24 prions to cattle and showed that the characteristics of these prions closely resemble those of L-type BSE prions found in Italy (9). In this study, we report the peripheral distribution of L-type BSE prions in experimentally challenged cattle.

The Study


Conclusions We report accumulation of L-type atypical BSE prions in peripheral nerve tissues sampled from intracerebrally challenged cattle. Our study demonstrated that almost all of the peripheral nerve tissues tested became PrPres positive in a time-dependent manner, whereas no PrPres was detectable in lymphoid tissues, even in cattle with fatal atypical BSE. Our results suggest the possibility that, like classical BSE prions, L-type BSE prions propagated in the central nervous system and were spread centrifugally by nerve pathways (11,12). In Italy, L-type BSE prions have been characterized in detail by using cattle challenged intracerebrally. However, PrPres was not detected in their peripheral tissues, including the peripheral nerves (13). The reason for the discrepancy in PrPres detection is unclear. In view of the similarities between the L-type and BSE/JP24 prion characteristics (9), this discrepancy may result from differences in the methods used for PrPres detection.

We detected infectivity in the nerve tissue samples (including samples from the obex, sciatic nerve, adrenal gland, brachial nerve plexus, and vagus nerve) obtained 10, 12, and 16 mpi. On the basis of the incubation time of 223 ± 25 (mean ± SD) days in mice injected with a 1,000-fold dilution of the obex homogenate, infectious titers in peripheral nerve tissues appeared to be 1,000 × lower than those estimated in the obex during endpoint titration of infectivity.

Our results demonstrate that L-type atypical BSE prions can be distributed in the peripheral nerve tissues of intracerebrally challenged cattle. These findings are useful for understanding L-type BSE pathogenesis and accurately assessing the risks associated with this disease. Investigations of prion distribution in cattle that have been orally challenged with L-type BSE prions are critical.

full text ;

Importation of Whole Cuts of Boneless Beef from Japan [Docket No. 05-004-1] RIN 0579-AB93 TSS SUBMISSION

Subject: Importation of Whole Cuts of Boneless Beef from Japan [Docket No. 05-004-1] RIN 0579-AB93 TSS SUBMISSION

Date: August 24, 2005 at 2:47 pm PST

August 24, 2005

Importation of Whole Cuts of Boneless Beef from Japan [Docket No. 05-004-1] RIN 0579-AB93 TSS SUBMISSION

Greetings APHIS ET AL,

My name is Terry S. Singeltary Sr.

I would kindly like to comment on [Docket No. 05-004-1] RIN 0579-AB93 ;

PROPOSED RULES Exportation and importation of animals and animal products: Whole cuts of boneless beef from- Japan, 48494-48500 [05-16422]

[Federal Register: August 18, 2005 (Volume 70, Number 159)] [Proposed Rules] [Page 48494-48500] From the Federal Register Online via GPO Access [] [DOCID:fr18au05-7]

======================================================================== Proposed Rules Federal Register ________________________________________________________________________

This section of the FEDERAL REGISTER contains notices to the public of the proposed issuance of rules and regulations. The purpose of these notices is to give interested persons an opportunity to participate in the rule making prior to the adoption of the final rules.


[[Page 48494]]


Animal and Plant Health Inspection Service

9 CFR Part 94

[Docket No. 05-004-1] RIN 0579-AB93

Importation of Whole Cuts of Boneless Beef from Japan

AGENCY: Animal and Plant Health Inspection Service, USDA.

ACTION: Proposed rule.


SUMMARY: We are proposing to amend the regulations governing the importation of meat and other edible animal products by allowing, under certain conditions, the importation of whole cuts of boneless beef from Japan. We are proposing this action in response to a request from the Government of Japan and after conducting an analysis of the risk that indicates that such beef can be safely imported from Japan under the conditions described in this proposal.

DATES: We will consider all comments that we receive on or before September 19, 2005.

ADDRESSES: You may submit comments by any of the following methods: EDOCKET: Go to to submit or


BSE infectivity has never been demonstrated in the muscle tissue of cattle experimentally or naturally infected with BSE at any stage of the disease. Studies performed using TSEs other than BSE in non-bovine animals have detected prions in muscle tissue. However, the international scientific community largely considers that these studies cannot be directly extrapolated to BSE in cattle because of the significant interactions between the host species and the prion strain involved. Pathogenesis studies of naturally and experimentally infected cattle have not detected BSE infectivity in blood. However, transmission of BSE was demonstrated in sheep that received a transfusion of a large volume of blood drawn from other sheep that were experimentally infected with the BSE agent. The United Kingdom's Department for Environment, Food and Rural Affairs' Spongiform Encephalopathy Advisory Committee (SEAC) and the European Commission's Scientific Steering Committee (SSC), which are scientific advisory committees, evaluated the implication of this finding in relation to food safety.\5\ The SEAC concluded that the finding did not represent grounds for recommending any changes to the current control measures for BSE. The SSC determined that the research results do not support the hypothesis that bovine blood or muscle meat constitute a risk to human health.\6\


BSE Risk Factors for Whole Cuts of Boneless Beef

The most significant risk management strategy for ensuring the safety of whole cuts of boneless beef is the prevention of cross- contamination of the beef with SRMs during stunning and slaughter of the animal. Control measures that prevent contamination of such beef involve the establishment of procedures for the removal of SRMs, prohibitions on air-injection stunning and pithing, and splitting of carcasses. These potential pathways for contamination and the control measures that prevent contamination are described in detail in the risk analysis for this rulemaking. SRM Removal. Research has demonstrated that SRMs from infected cattle may contain BSE infectivity. Because infectivity has not been demonstrated in muscle tissue, the most important mitigation measure for whole cuts of boneless beef is the careful removal and segregation of SRMs. Removal of SRMs in a manner that avoids contamination of the beef with SRMs minimizes the risk of exposure to materials that have been demonstrated to contain the BSE agent in cattle.


Variant Creutzfeldt-Jakob disease (vCJD), a chronic and fatal neurodegenerative disease of humans, has been linked since 1996 through epidemiological, neuropathological, and experimental data to exposure to the BSE agent, most likely through consumption of cattle products contaminated with the agent before BSE control measures were in place. To date, approximately 170 probable and confirmed cases of vCJD have been identified worldwide. The majority of these cases have either been identified in the United Kingdom or were linked to exposure that occurred in the United Kingdom, and all cases have been linked to exposure in countries with native cases of BSE. Some studies estimate that more than 1 million cattle may have been infected with BSE throughout the epidemic in the United Kingdom. This number of infected cattle could have introduced a significant amount of infectivity into the human food supply. Yet, the low number of cases of vCJD identified to date indicates that there is a substantial species barrier that protects humans from widespread illness due to exposure to the BSE agent.


International Guidelines on BSE

International guidelines for trade in animal and animal products are developed by the World Organization for Animal Health (formerly known as the Office International des Epizooties (OIE)), which is recognized by the World Trade Organization (WTO) as the international organization responsible for the development of standards, guidelines, and recommendations with respect to animal health and zoonoses (diseases that are transmissible from animals to humans). The OIE guidelines for trade in terrestrial animals (mammals, birds, and bees) are detailed in the Terrestrial Animal Health Code (available on the internet at The guidelines on BSE are contained in

Chapter 2.3.13 of the Code and supplemented by Appendix 3.8.4 of the Code.


Greetings again APHIS ET AL,

THIS is not correct. IN fact, there are several factors i would like to kindly address.

Muscle tissue has recently been detected with PrPSc in the peripheral nerves (sciatic nerve, tibial nerve, vagus nerve) of the 11th BSE cow in Japan (Yoshifumi Iwamaru et al). also recently, Aguzzi et al Letter to the Editor Vet Pathol 42:107-108 (2005), Prusiner et al CDI test is another example of detection of the TSE agent in muscle in sCJD, Herbert Budka et al CJD and inclusion body myositis: Abundant Disease-Associated Prion Protein in Muscle, and older studies from Watson Meldrum et al Scrapie agent in muscle - Pattison I A (1990), references as follow ;

PrPSc distribution of a natural case of bovine spongiform encephalopathy

Yoshifumi Iwamaru, Yuka Okubo, Tamako Ikeda, Hiroko Hayashi, Mori- kazu Imamura, Takashi Yokoyama and Morikazu Shinagawa

Priori Disease Research Center, National Institute of Animal Health, 3-1-5 Kannondai, Tsukuba 305-0856 Japan


Bovine spongiform encephalopathy (BSE) is a disease of cattle that causes progressive neurodegeneration of the central nervous system. Infectivity of BSE agent is accompanied with an abnormal isoform of prion protein (PrPSc).

The specified risk materials (SRM) are tissues potentially carrying BSE infectivity. The following tissues are designated as SRM in Japan: the skull including the brain and eyes but excluding the glossa and the masse- ter muscle, the vertebral column excluding the vertebrae of the tail, spinal cord, distal illeum. For a risk management step, the use of SRM in both animal feed or human food has been prohibited. However, detailed PrPSc distribution remains obscure in BSE cattle and it has caused con- troversies about definitions of SRM. Therefore we have examined PrPSc distribution in a BSE cattle by Western blotting to reassess definitions of SRM.

The 11th BSE case in Japan was detected in fallen stock surveillance. The carcass was stocked in the refrigerator. For the detection of PrPSc, 200 mg of tissue samples were homogenized. Following collagenase treatment, samples were digested with proteinase K. After digestion, PrPSc was precipitated by sodium phosphotungstate (PTA). The pellets were subjected to Western blotting using the standard procedure. Anti-prion protein monoclonal antibody (mAb) T2 conjugated horseradish peroxidase was used for the detection of PrPSc.

PrPSc was detected in brain, spinal cord, dorsal root ganglia, trigeminal ganglia, sublingual ganglion, retina. In addition, PrPSc was also detected in the peripheral nerves (sciatic nerve, tibial nerve, vagus nerve).

Our results suggest that the currently accepted definitions of SRM in BSE cattle may need to be reexamined. ...


T. Kitamoto (Ed.) PRIONS Food and Drug Safety ================

ALSO from the International Symposium of Prion Diseases held in Sendai, October 31, to November 2, 2004;

Bovine spongiform encephalopathy (BSE) in Japan


"Furthermore, current studies into transmission of cases of BSE that are atypical or that develop in young cattle are expected to amplify the BSE prion"

NO. Date conf. Farm Birth place and Date Age at diagnosis

8. 2003.10.6. Fukushima Tochigi 2001.10.13. 23

9. 2003.11.4. Hiroshima Hyogo 2002.1.13. 21

Test results

# 8b, 9c cows Elisa Positive, WB Positive, IHC negative, histopathology negative

b = atypical BSE case

c = case of BSE in a young animal

b,c, No PrPSc on IHC, and no spongiform change on histology

International Symposium of Prion Diseases held in Sendai, October 31, to November 2, 2004.

The hardback book title is 'PRIONS' Food and Drug Safety T. Kitamoto (Ed.)

Tetsuyuki Kitamoto Professor and Chairman Department of Prion Research Tohoku University School of Medicine 2-1 SeiryoAoba-ku, Sendai 980-8575, JAPAN TEL +81-22-717-8147 FAX +81-22-717-8148 e-mail; Symposium Secretariat Kyomi Sasaki TEL +81-22-717-8233 FAX +81-22-717-7656 e-mail:


snip...please see full text and other transmission studies here ;


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.

14th International Congress on Infectious Diseases H-type and L-type Atypical BSE January 2010 (special pre-congress edition)

18.173 page 189

Experimental Challenge of Cattle with H-type and L-type Atypical BSE

A. Buschmann1, U. Ziegler1, M. Keller1, R. Rogers2, B. Hills3, M.H. Groschup1. 1Friedrich-Loeffler-Institut, Greifswald-Insel Riems, Germany, 2Health Canada, Bureau of Microbial Hazards, Health Products & Food Branch, Ottawa, Canada, 3Health Canada, Transmissible Spongiform Encephalopathy Secretariat, Ottawa, Canada

Background: After the detection of two novel BSE forms designated H-type and L-type atypical BSE the question of the pathogenesis and the agent distribution of these two types in cattle was fully open. From initial studies of the brain pathology, it was already known that the anatomical distribution of L-type BSE differs from that of the classical type where the obex region in the brainstem always displays the highest PrPSc concentrations. In contrast in L-type BSE cases, the thalamus and frontal cortex regions showed the highest levels of the pathological prion protein, while the obex region was only weakly involved.

Methods:We performed intracranial inoculations of cattle (five and six per group) using 10%brainstemhomogenates of the two German H- and L-type atypical BSE isolates. The animals were inoculated under narcosis and then kept in a free-ranging stable under appropriate biosafety conditions.At least one animal per group was killed and sectioned in the preclinical stage and the remaining animals were kept until they developed clinical symptoms. The animals were examined for behavioural changes every four weeks throughout the experiment following a protocol that had been established during earlier BSE pathogenesis studies with classical BSE.

Results and Discussion: All animals of both groups developed clinical symptoms and had to be euthanized within 16 months. The clinical picture differed from that of classical BSE, as the earliest signs of illness were loss of body weight and depression. However, the animals later developed hind limb ataxia and hyperesthesia predominantly and the head. Analysis of brain samples from these animals confirmed the BSE infection and the atypical Western blot profile was maintained in all animals. Samples from these animals are now being examined in order to be able to describe the pathogenesis and agent distribution for these novel BSE types. Conclusions: A pilot study using a commercially avaialble BSE rapid test ELISA revealed an essential restriction of PrPSc to the central nervous system for both atypical BSE forms. A much more detailed analysis for PrPSc and infectivity is still ongoing.

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


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.


12 years independent research of available data


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.


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.

see page 114 ;

International Society for Infectious Diseases Web:

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 ;


''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



Molecular Features of the Protease-resistant Prion Protein (PrPres) in H-type BSE

Biacabe, A-G1; Jacobs, JG2; Gavier-Widén, D3; Vulin, J1; Langeveld, JPM2; Baron, TGM1 1AFSSA, France; 2CIDC-Lelystad, Netherlands; 3SVA, Sweden

Western blot analyses of PrPres accumulating in the brain of BSE-infected cattle have demonstrated 3 different molecular phenotypes regarding to the apparent molecular masses and glycoform ratios of PrPres bands. We initially described isolates (H-type BSE) essentially characterized by higher PrPres molecular mass and decreased levels of the diglycosylated PrPres band, in contrast to the classical type of BSE. This type is also distinct from another BSE phenotype named L-type BSE, or also BASE (for Bovine Amyloid Spongiform Encephalopathy), mainly characterized by a low representation of the diglycosylated PrPres band as well as a lower PrPres molecular mass. Retrospective molecular studies in France of all available BSE cases older than 8 years old and of part of the other cases identified since the beginning of the exhaustive surveillance of the disease in 20001 allowed to identify 7 H-type BSE cases, among 594 BSE cases that could be classified as classical, L- or H-type BSE. By Western blot analysis of H-type PrPres, we described a remarkable specific feature with antibodies raised against the C-terminal region of PrP that demonstrated the existence of a more C-terminal cleaved form of PrPres (named PrPres#2 ), in addition to the usual PrPres form (PrPres #1). In the unglycosylated form, PrPres #2 migrates at about 14 kDa, compared to 20 kDa for PrPres #1. The proportion of the PrPres#2 in cattle seems to by higher compared to the PrPres#1. Furthermore another PK–resistant fragment at about 7 kDa was detected by some more N-terminal antibodies and presumed to be the result of cleavages of both N- and C-terminal parts of PrP. These singular features were maintained after transmission of the disease to C57Bl/6 mice. The identification of these two additional PrPres fragments (PrPres #2 and 7kDa band) reminds features reported respectively in sporadic Creutzfeldt-Jakob disease and in Gerstmann-Sträussler-Scheinker (GSS) syndrome in humans.

Wednesday, March 31, 2010

Atypical BSE in Cattle

Sunday, February 14, 2010

[Docket No. FSIS-2006-0011] FSIS Harvard Risk Assessment of Bovine Spongiform Encephalopathy (BSE)




May 4, 2004 Media Inquiries: 301-827-6242 Consumer Inquiries: 888-INFO-FDA

Statement on Texas Cow With Central Nervous System Symptoms

On Friday, April 30th, 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, which is responsible for the safety of animal feed, immediately began an investigation. On Friday and throughout the weekend, FDA investigators inspected the slaughterhouse, the rendering facility, the farm where the animal came from, and the processor that initially received the cow from the slaughterhouse.

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.

Cattle with central nervous system symptoms are of particular interest because cattle with bovine spongiform encephalopathy or BSE, also known as "mad cow disease," can exhibit such symptoms. In this case, there is no way now to test for BSE. But even if the cow had BSE, FDA's animal feed rule would prohibit the feeding of its rendered protein to other ruminant animals (e.g., cows, goats, sheep, bison). ...

OR, what about this ;

Owner and Corporation Plead Guilty to Defrauding Bovine Spongiform Encephalopathy (BSE) Surveillance Program

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.


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),


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


snip... please see full text ;

THEY KNEW 2 DECADES AGO the damn BSE mad cow testing were not finding cases ;


3. A question posed by Mr Whaley (para 2) is that classical lesions of BSE may not occur in all cases. Supposing we had a strain variant that produced it's lesions in the cerebrum these would not be detected by our current method. I think this would be unlikely but not impossible - another reason why at least a proportion of complete brains (or blocks) should be retained during the epidemic so if the problem Mr Whaley indicates escalates, it can be investigated.


5. IF you had the information what benefit would there be ? what would you do with it ?


I do not recommend any action. The situation should be accepted. I do not think the VIS can do more at present. The situation should be kept under review particularly if there is an escalation in numbers in this category.


15 MAY 1990


Tuesday, November 17, 2009


NEW RESULTS ON IDIOPATHIC BRAINSTEM NEURONAL CHROMATOLYSIS "All of the 15 cattle tested showed that the brains had abnormally accumulated PrP" 2009


""These 9,200 cases were different because brain tissue samples were preserved with formalin, which makes them suitable for only one type of test--immunohistochemistry, or IHC."


THE IHC test has been proven to be the LEAST LIKELY to detect BSE/TSE in the bovine, and these were probably from the most high risk cattle pool, the ones the USDA et al, SHOULD have been testing. ...TSS

USDA 2003

We have to be careful that we don't get so set in the way we do things that we forget to look for different emerging variations of disease. We've gotten away from collecting the whole brain in our systems. We're using the brain stem and we're looking in only one area. In Norway, they were doing a project and looking at cases of Scrapie, and they found this where they did not find lesions or PRP in the area of the obex. They found it in the cerebellum and the cerebrum. It's a good lesson for us. Ames had to go back and change the procedure for looking at Scrapie samples. In the USDA, we had routinely looked at all the sections of the brain, and then we got away from it. They've recently gone back. Dr. Keller: Tissues are routinely tested, based on which tissue provides an 'official' test result as recognized by APHIS.

Dr. Detwiler: That's on the slaughter. But on the clinical cases, aren't they still asking for the brain? But even on the slaughter, they're looking only at the brainstem. We may be missing certain things if we confine ourselves to one area.


Dr. Detwiler: It seems a good idea, but I'm not aware of it. Another important thing to get across to the public is that the negatives do not guarantee absence of infectivity. The animal could be early in the disease and the incubation period. Even sample collection is so important. If you're not collecting the right area of the brain in sheep, or if collecting lymphoreticular tissue, and you don't get a good biopsy, you could miss the area with the PRP in it and come up with a negative test. There's a new, unusual form of Scrapie that's been detected in Norway. We have to be careful that we don't get so set in the way we do things that we forget to look for different emerging variations of disease. We've gotten away from collecting the whole brain in our systems. We're using the brain stem and we're looking in only one area. In Norway, they were doing a project and looking at cases of Scrapie, and they found this where they did not find lesions or PRP in the area of the obex. They found it in the cerebellum and the cerebrum. It's a good lesson for us. Ames had to go back and change the procedure for looking at Scrapie samples. In the USDA, we had routinely looked at all the sections of the brain, and then we got away from it. They've recently gone back.

Dr. Keller: Tissues are routinely tested, based on which tissue provides an 'official' test result as recognized by APHIS .

Dr. Detwiler: That's on the slaughter. But on the clinical cases, aren't they still asking for the brain? But even on the slaughter, they're looking only at the brainstem. We may be missing certain things if we confine ourselves to one area.


Completely Edited Version PRION ROUNDTABLE

Accomplished this day, Wednesday, December 11, 2003, Denver, Colorado

Saturday, June 19, 2010



IF the truth were known (and it's not like I have not been trying), the USA, Canada, and Mexico (there are other Countries too), should all be listed in this new TSE prion trader friendly atmosphere as ''undetermined risk''. Because USDA et al have absolutely no idea. The ideology of only the UK BSE theory and there from only imported MBM and feed, to ignore the fact that the continuous rendering technology was developed and the USDA got the UK to use it first, some five years before the USA started using the same technology, and then the fact of all the different TSE in different species here in North America, and different strains there from, to continue to believe in only the imported factor of feed and animals, and not take seriously the _home grown_ factor, from tainted _home grown TSE tainted feed_, from the same type rendering technology, is like sticking your head in a hole in the ground and hoping for the best. kind of like what BP did in the Gulf of Mexico. But for the OIE to continue to go by this decades old science on BSE, and continue to ignore the risk factors from other strains of BSE, and other TSE in other species, when scientist from around the globe continue to wave flags of concern, to continue this ignorance is dangerous for human and animal health. But typical for the OIE and the USDA in reference to the Transmissible Spongiform Encephalopathy disease. Both the USDA and the OIE have ignored these documented risk factors for years, even decades, simply for trading purposes. The USDA et al until 2003 when the first documented case of c-BSE was documented in Washington State, the USDA had nothing to do with countries that had BSE. Until that cow old Luther capped in Washington, then the shoe was on the other foot. The USDA and the OIE after that literally changed the rules and regulations on BSE that had been in place for almost a decade trying to eradicate it all around the globe before it mutated, by doing away with the BSE GBR risk assessments and ignoring them, and implementing the infamous force fed USDA BSE MRR policy (all this is explained below in the source reference). But two mad cows sat on ice while all this political science was taking place for 7 months. One finally confirmed thanks to the OIG and the Honorable Phyllis Fong, and the other could not be confirmed due to the fact in had been improperly stored for 4 MONTHS, before testing. Kind of like the other stumbling and staggering mad cow in Texas that got away, went straight to be rendered for pet food, without NO TSE prion test at all. I could go on, about the healthy brains, from healthy cows, cows they knew did not have BSE, submitted for the infamous 2004 Enhanced BSE surveillance and testing program, or the other 9,200 brains they only used IHC testing, the least likely to find BSE. Sadly, once they did start documenting BSE back to back, they shut it down, said that was enough, let's cancel this right here in it's tracks, and we have heard nothing since, like the USA has now become immune to any TSE in any bovine. ;

When the OIE and the USDA et al collaborated to make legal the trading of Transmissible Spongiform Encephalopathy, when they did away with the BSE GBR risk assessments, where the USA, Canada, and Mexico were categorized as BSE GBR III. please see ;

EFSA concludes that the current GBR level of USA is III, i.e. it is likely but not confirmed that domestic cattle are (clinically or pre-clinically) infected with the BSE-agent. As long as there are no significant changes in rendering or feeding, the stability remains extremely/very unstable. Thus, the probability of cattle to be (pre-clinically or clinically) infected with the BSE-agent persistently increases.

Annex to the EFSA Scientific Report (2004) 3, 1-17 on the Assessment of the Geographical BSE Risk of USA

please see full text ;

YET, in 2010, tons and tons of banned mad cow protein are still in commerce here in the USA, scientific studies are being misconstrued and manipulated by ARS USDA, which are still going by TSE science that is decades old, while refusing to acknowledge new scientific studies, and FOIA requests are still being held up by the USDA et al on these urgent matters (see source related materials below). CJD of unknown phenotype, in victims that are getting younger, with longer clinical course from first onset of symptoms to death are occurring, in fact, sporadic CJD is still rising, where the TSEs in the different species are mutating here in the USA, and we still have this same dog and pony show by the OIE and USDA et al. IF you go back and look at the Countries that went by these OIE BSE guidelines, most all came down with BSE. I have said it before, I was say it again now, 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. ...TSS

see full text and reasons why here ;

Saturday, June 12, 2010

PUBLICATION REQUEST AND FOIA REQUEST Project Number: 3625-32000-086-05 Study of Atypical Bse

Sent: Friday, June 18, 2010 11:48 AM Subject: Re: [BSE-L] Freedom of Information Act Project Number 3625-32000-086-05, Study of Atypical BSE

Greetings Ms Williams, ARS, USDA, ET AL,

Thank you for your kind reply and correction of the information in your data base on the Project Number 3625-32000-086-05, Study of Atypical BSE, and whether it is necessary to change SRM removal due to any different tissue infectivity distribution. Your request was logged in and assigned FOIA No. 10-93.

Ms Williams ARS USDA et al stated ;

>>> In searching for records responsive to your request, we discovered that our Agricultural Research Information System (ARIS) database contained incorrect information. The ARIS database incorrectly linked the same progress report to Project Numbers 3625-32000-086-05S and 3625-32000-086-04S, which resulted in inaccurate information being reported for the Study of Atypical BSE. This discrepancy was reported to the managing office and has been resolved. To view the progress report, click or copy and paste the URL into your browser window: <<<

>>> 3. Studies on transmissibility and tissue distribution of atypical BSE isolates in cattle and other species. my FOIA, two questions still have not been answered. There have been 5 years gone by now Start Date: Sep 15, 2004 End Date: Sep 14, 2009 <<<

>>> 1a.Objectives (from AD-416) The objective of this cooperative research project with Dr. Maria Caramelli from the Italian BSE Reference Laboratory in Turin, Italy, is to conduct comparative studies with the U.S. bovine spongiform encephalopathy (BSE) isolate and the atypical BSE isolates identified in Italy. The studies will cover the following areas: 1. Evaluation of present diagnostics tools used in the U.S. for the detection of atypical BSE cases. 2. Molecular comparison of the U.S. BSE isolate and other typical BSE isolates with atypical BSE cases. 3. Studies on transmissibility and tissue distribution of atypical BSE isolates in cattle and other species. <<<

Ms Williams et al at ARS USDA,

please tell me via FOIA or not, what the results of the tissue distribution and transmissibility of atypical BSE isolates and comparisons were as stated ; 3. Studies on transmissibility and tissue distribution of atypical BSE isolates in cattle and other species. The study plainly stated that during the 5 years study in question, that Studies on transmissibility and tissue distribution of atypical BSE isolates in cattle and other species would be done ;

>>>Transmission studies are already underway using brain homogenates from atypical BSE cases into mice, cattle and sheep. It will be critical to see whether the atypical BSE isolates behave similarly to typical BSE isolates in terms of transmissibility and disease pathogenesis. If transmission occurs, tissue distribution comparisons will be made between cattle infected with the atypical BSE isolate and the U.S. BSE isolate. Differences in tissue distribution could require new regulations regarding specific risk material (SRM) removal. <<<

Thank You,

Kindly still waiting FOIA request answers Project Number 3625-32000-086-05, Study of Atypical BSE,

Terry S. Singeltary Sr.
P.O. Box 42
Bacliff, Texas USA 77518


see full text ;

Archive Number 20100405.1091 Published Date 05-APR-2010 Subject PRO/AH/EDR> Prion disease update 1010 (04)


[Terry S. Singeltary Sr. has added the following comment:

"According to the World Health Organisation, the future public health threat of vCJD in the UK and Europe and potentially the rest of the world is of concern and currently unquantifiable. However, the possibility of a significant and geographically diverse vCJD epidemic occurring over the next few decades cannot be dismissed


The key word here is diverse. What does diverse mean? If USA scrapie transmitted to USA bovine does not produce pathology as the UK c-BSE, then why would CJD from there look like UK vCJD?",F2400_P1001_PUB_MAIL_ID:1000,82101

> 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.


Monday, April 5, 2010


Tuesday, June 1, 2010

USA cases of dpCJD rising with 24 cases so far in 2010

Wednesday, June 16, 2010

Defining sporadic Creutzfeldt-Jakob disease strains and their transmission properties


J Clin Invest. doi:10.1172/JCI42051. Copyright © 2010, The American Society for Clinical Investigation.

Research Article

A molecular switch controls interspecies prion disease transmission in mice

Christina J. Sigurdson1,2,3, K. Peter R. Nilsson3, Simone Hornemann4, Giuseppe Manco3, Natalia Fernández-Borges5, Petra Schwarz3, Joaquín Castilla5,6, Kurt Wüthrich4,7 and Adriano Aguzzi3


These observations suggest striking differences in the ß-sheet alignment of PrPSc aggregates between prion-infected 170S and 170N animals and may provide a plausible starting point for clarifying the structural basis of prion species barriers that are highly relevant to public health, including the potential transmissibility of bovine and cervid prions to humans.


As a possible exception to these observations, cattle may be susceptible to CWD from white-tailed deer (86). The latter finding suggests that specific prion strains can overrule the codon 170 homology requirement.

see also ;

Monday, June 14, 2010

A molecular switch controls interspecies prion disease transmission in mice

Friday, May 14, 2010

Prion Strain Mutation Determined by Prion Protein Conformational Compatibility and Primary Structure

Published Online May 13, 2010 Science DOI: 10.1126/science.1187107 Science Express Index

Saturday, June 5, 2010

Research Project: Transmissible Spongiform Encephalopathies: Identification of atypical scrapie in Canadian sheep

Wednesday, June 16, 2010

Defining sporadic Creutzfeldt-Jakob disease strains and their transmission properties

Wednesday, June 02, 2010

CJD Annex H UPDATE AFTER DEATH PRECAUTIONS Published: 2 June 2003 Updated: May 2010

Sunday, August 09, 2009

CJD...Straight talk with...James Ironside...and...Terry Singeltary... 2009

Tuesday, August 18, 2009

BSE-The Untold Story - joe gibbs and singeltary 1999 - 2009

Terry S. Singeltary Sr.
P.O. Box 42
Bacliff, Texas USA 77518

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