Experimental Transmission of H-type Bovine Spongiform Encephalopathy to Bovinized Transgenic Mice

Experimental Transmission of H-type Bovine Spongiform Encephalopathy to Bovinized Transgenic Mice

Vet Pathol 0300985810382672, first published on October 4, 2010


Experimental Transmission of H-type Bovine Spongiform Encephalopathy to Bovinized Transgenic Mice

H. Okada okadahi@affrc.go.jp Prion Disease Research Center, National Institute of Animal Health, Tsukuba, K. Masujin Prion Disease Research Center, National Institute of Animal Health, Tsukuba, Y. Imamaru Prion Disease Research Center, National Institute of Animal Health, Tsukuba, M. Imamura Prion Disease Research Center, National Institute of Animal Health, Tsukuba, Y. Matsuura Prion Disease Research Center, National Institute of Animal Health, Tsukuba, S. Mohri Prion Disease Research Center, National Institute of Animal Health, Tsukuba, S. Czub Animal Disease Research Institute, Canadian Food Inspection Agency, T. Yokoyama Prion Disease Research Center, National Institute of Animal Health, Tsukuba,

Abstract

To characterize the biological and biochemical properties of H-type bovine spongiform encephalopathy (BSE), a transmission study with a Canadian H-type isolate was performed with bovinized transgenic mice (TgBoPrP), which were inoculated intracerebrally with brain homogenate from cattle with H-type BSE. All mice exhibited characteristic neurologic signs, and the subsequent passage showed a shortened incubation period. The distribution of disease-associated prion protein (PrPSc) was determined by immunohistochemistry, Western blot, and paraffin-embedded tissue (PET) blot. Biochemical properties and higher molecular weight of the glycoform pattern were well conserved within mice. Immunolabeled granular PrPSc, aggregates, and/or plaque-like deposits were mainly detected in the following brain locations: septal nuclei, subcallosal regions, hypothalamus, paraventricular nucleus of the thalamus, interstitial nucleus of the stria terminalis, and the reticular formation of the midbrain. Weak reactivity was detected by immunohistochemistry and PET blot in the cerebral cortex, most thalamic nuclei, the hippocampus, medulla oblongata, and cerebellum. These findings indicate that the H-type BSE prion has biological and biochemical properties distinct from those of C-type and L-type BSE in TgBoPrP mice, which suggests that TgBoPrP mice constitute a useful animal model to distinguish isolates from BSE-infected cattle.

© 2010 Sage Publications, Inc.



http://vet.sagepub.com/content/early/2010/10/02/0300985810382672.abstract




Greetings,


I have been most interested to see IF the h-BSE (h-BSE or g-h-BSEalabama???), but i have been most interested to see if in fact this atypical h-BSE is more virulent than c-BSE, as is the L-BSE (Italian strain) has been documented to be. We know from the studies of Kong et al that h-BSE will transmit to TG human mice;

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.

http://www.prion2009.com/sites/default/files/Prion2009_Book_of_Abstracts.pdf


HOWEVER, as to the virulance of it one way or the other compared to c-BSE and or L-BSE, i don't think no one has said yet or not? interesting this debate of the h-BSE TEXAS (2nd mad cow finally confirmed 7 months after the fact, and an act of Congress), compared to the g-h-BSEalabama strain documented in Alabama, that is identicle to the new human CJD in the USA that is killing the young and old, with clinical long duration, and different symptoms in some cases too, but not related to this ??? ALSO, this IBNC BSE, might this be the g-h-BSEalabama strain?


layperson


Terry S. Singeltary Sr.
P.O. Box 42
Bacliff, Texas USA 77518
flounder9@verizon.net



Given the large number of strains of scrapie and the possibility that BSE was one of them, it would be necessary to transmit every scrapie strain to cattle separately, to test the hypothesis properly. Such an experiment would be expensive. Secondly, as measures to control the epidemic took hold, the need for the experiment from the policy viewpoint was not considered so urgent. It was felt that the results would be mainly of academic interest.345 3.59 Nevertheless, from the first demonstration of transmissibility of BSE in 1988, the possibility of differences in the transmission properties of BSE and scrapie was clear. Scrapie was transmissible to hamsters, but by 1988 attempts to transmit BSE to hamsters had failed. Subsequent findings increased that possibility.

http://collections.europarchive.org/tna/20080102110838/http://www.bseinquiry.gov.uk/pdf/volume2/chapter3.pdf



Sunday, April 18, 2010

SCRAPIE AND ATYPICAL SCRAPIE TRANSMISSION STUDIES A REVIEW 2010

http://scrapie-usa.blogspot.com/2010/04/scrapie-and-atypical-scrapie.html



Sunday, October 3, 2010

Scrapie, Nor-98 atypical Scrapie, and BSE in sheep and goats North America, who's looking ?

http://nor-98.blogspot.com/2010/10/scrapie-nor-98-atypical-scrapie-and-bse.html



let's take a closer look at this new prionpathy or prionopathy, and then let's look at the g-h-BSEalabama mad cow.

This new prionopathy in humans? the genetic makeup is IDENTICAL to the g-h-BSEalabama mad cow, the only _documented_ mad cow in the world to date like this, ......wait, it get's better. this new prionpathy is killing young and old humans, with LONG DURATION from onset of symptoms to death, and the symptoms are very similar to nvCJD victims, OH, and the plaques are very similar in some cases too, bbbut, it's not related to the g-h-BSEalabama cow, WAIT NOW, it gets even better, the new human prionpathy that they claim is a genetic TSE, has no relation to any gene mutation in that family. daaa, ya think it could be related to that mad cow with the same genetic make-up ??? there were literally tons and tons of banned mad cow protein in Alabama in commerce, and none of it transmitted to cows, and the cows to humans there from ??? r i g h t $$$

ALABAMA MAD COW g-h-BSEalabama

In this study, we identified a novel mutation in the bovine prion protein gene (Prnp), called E211K, of a confirmed BSE positive cow from Alabama, United States of America. This mutation is identical to the E200K pathogenic mutation found in humans with a genetic form of CJD. This finding represents the first report of a confirmed case of BSE with a potential pathogenic mutation within the bovine Prnp gene. We hypothesize that the bovine Prnp E211K mutation most likely has caused BSE in "the approximately 10-year-old cow" carrying the E221K mutation.

http://www.plospathogens.org/article/info%3Adoi%2F10.1371%2Fjournal.ppat.1000156



http://www.plospathogens.org/article/fetchObjectAttachment.action?uri=info%3Adoi%2F10.1371%2Fjournal.ppat.1000156&representation=PDF



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)

http://prionpathy.blogspot.com/2010/08/bse-case-associated-with-prion-protein.html



Tuesday, August 03, 2010

Variably protease-sensitive prionopathy: A new sporadic disease of the prion protein

http://creutzfeldt-jakob-disease.blogspot.com/2010/08/variably-protease-sensitive-prionopathy.html



Monday, August 9, 2010

Variably protease-sensitive prionopathy: A new sporadic disease of the prion protein or just more PRIONBALONEY ?

http://prionunitusaupdate2008.blogspot.com/2010/08/variably-protease-sensitive-prionopathy.html



***+++***

Thursday, July 10, 2008

A Novel Human Disease with Abnormal Prion Protein Sensitive to Protease update July 10, 2008 Friday, June 20, 2008

http://cjdmadcowbaseoct2007.blogspot.com/2008/07/novel-human-disease-with-abnormal-prion.html



CJD TEXAS 38 YEAR OLD FEMALE WORKED SLAUGHTERING CATTLE EXPOSED TO BRAIN AND SPINAL CORD MATTER





Irma Linda Andablo CJD Victim, she died at 38 years old on February 6, 2010 in Mesquite Texas

Irma Linda Andablo CJD Victim, she died at 38 years old on February 6, 2010 in Mesquite Texas.She left 6 Kids and a Husband.The Purpose of this web is to give information in Spanish to the Hispanic community, and to all the community who want's information about this terrible disease.-

Physician Discharge Summary, Parkland Hospital, Dallas Texas

Admit Date: 12/29/2009 Discharge Date: 1/20/2010 Attending Provider: Greenberg, Benjamin Morris; General Neurology Team: General Neurology Team

Linda was a Hispanic female with no past medical history presents with 14 months of incresing/progressive altered mental status, generalized weakness, inability to walk, loss of appetite, inability to speak, tremor and bowel/blader incontinence.She was, in her usual state of health up until February, 2009, when her husbans notes that she began forgetting things like names and short term memories. He also noticed mild/vague personality changes such as increased aggression. In March, she was involved in a hit and run MVA,although she was not injured. The police tracked her down and ticketed her. At that time, her son deployed to Iraq with the Army and her husband assumed her mentation changes were due to stress over these two events. Also in March, she began to have weakness in her legs, making it difficult to walk. Over the next few months, her mentation and personality changes worsened, getting to a point where she could no longer recognized her children. She was eating less and less. She was losing more weight. In the last 2-3 months, she reached the point where she could not walk without an assist, then 1 month ago, she stopped talking, only making grunting/aggressive sounds when anyone came near her. She also became both bowel and bladder incontinent, having to wear diapers. Her '"tremor'" and body jerks worsened and her hands assumed a sort of permanent grip position, leading her family to put tennis balls in her hands to protect her fingers.

The husband says that they have lived in Nebraska for the past 21 years. They had seen a doctor there during the summer time who prescribed her Seroquel and Lexapro, Thinking these were sx of a mood disorder. However, the medications did not help and she continued to deteriorate clinically. 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. The husband says that he does not know any fellow workers with a similar illness. He also says that she did not have any preceeding illness or travel.


http://www.recordandoalinda.com/index.php?option=com_content&view=article&id=19:cjd-english-info&catid=9:cjd-ingles&Itemid=8




[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?"

SEE FULL TEXT ;


http://www.promedmail.org/pls/apex/f?p=2400:1001:568933508083034::NO::F2400_P1001_BACK_PAGE,F2400_P1001_PUB_MAIL_ID:1000,82101



.57 The experiment which might have determined whether BSE and scrapie were caused by the same agent (ie, the feeding of natural scrapie to cattle) was never undertaken in the UK. It was, however, performed in the USA in 1979, when it was shown that cattle inoculated with the scrapie agent endemic in the flock of Suffolk sheep at the United States Department of Agriculture in Mission, Texas, developed a TSE quite unlike BSE. 32 The findings of the initial transmission, though not of the clinical or neurohistological examination, were communicated in October 1988 to Dr Watson, Director of the CVL, following a visit by Dr Wrathall, one of the project leaders in the Pathology Department of the CVL, to the United States Department of Agriculture. 33 The results were not published at this point, since the attempted transmission to mice from the experimental cow brain had been inconclusive. The results of the clinical and histological differences between scrapie-affected sheep and cattle were published in 1995. Similar studies in which cattle were inoculated intracerebrally with scrapie inocula derived from a number of scrapie-affected sheep of different breeds and from different States, were carried out at the US National Animal Disease Centre. 34 The results, published in 1994, showed that this source of scrapie agent, though pathogenic for cattle, did not produce the same clinical signs of brain lesions characteristic of BSE.

32 Clark, W., Hourrigan, J. and Hadlow, W. (1995) Encephalopathy in Cattle Experimentally Infected with the Scrapie Agent, American Journal of Veterinary Research, 56, 606-12

33 YB88/10.00/1.1


http://web.archive.org/web/20040823105233/www.bseinquiry.gov.uk/files/yb/1988/10/00001001.pdf



Saturday, October 2, 2010

BSE surveillance front and centre: CFIA and USA

http://madcowtesting.blogspot.com/2010/10/bse-surveillance-front-and-centre-cfia.html



P.9.21

Molecular characterization of BSE in Canada

Jianmin Yang1, Sandor Dudas2, Catherine Graham2, Markus Czub3, Tim McAllister1, Stefanie Czub1 1Agriculture and Agri-Food Canada Research Centre, Canada; 2National and OIE BSE Reference Laboratory, Canada; 3University of Calgary, Canada

Background: Three BSE types (classical and two atypical) have been identified on the basis of molecular characteristics of the misfolded protein associated with the disease. To date, each of these three types have been detected in Canadian cattle.

Objectives: This study was conducted to further characterize the 16 Canadian BSE cases based on the biochemical properties of there associated PrPres. Methods: Immuno-reactivity, molecular weight, glycoform profiles and relative proteinase K sensitivity of the PrPres from each of the 16 confirmed Canadian BSE cases was determined using modified Western blot analysis.

Results: Fourteen of the 16 Canadian BSE cases were C type, 1 was H type and 1 was L type. The Canadian H and L-type BSE cases exhibited size shifts and changes in glycosylation similar to other atypical BSE cases. PK digestion under mild and stringent conditions revealed a reduced protease resistance of the atypical cases compared to the C-type cases. N terminal- specific antibodies bound to PrPres from H type but not from C or L type. The C-terminal-specific antibodies resulted in a shift in the glycoform profile and detected a fourth band in the Canadian H-type 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.

http://www.prion2009.com/sites/default/files/Prion2009_Book_of_Abstracts.pdf



Tuesday, September 14, 2010

Feed Safety and BSE/Ruminant Feed Ban Support Project (U18)

http://madcowfeed.blogspot.com/2010/09/feed-safety-and-bseruminant-feed-ban.html




THIS is why this question is so important to me, IS h-BSE more or less virulent than the c-BSE ?


Wednesday, July 28, 2010

re-Freedom of Information Act Project Number 3625-32000-086-05, Study of Atypical BSE UPDATE July 28, 2010


http://bse-atypical.blogspot.com/2010/07/re-freedom-of-information-act-project.html



Wednesday, August 11, 2010

REPORT ON THE INVESTIGATION OF THE SIXTEENTH CASE OF BOVINE SPONGIFORM ENCEPHALOPATHY (BSE) IN CANADA

http://bse-atypical.blogspot.com/2010/08/report-on-investigation-of-sixteenth.html



Thursday, August 19, 2010

REPORT ON THE INVESTIGATION OF THE SEVENTEENTH CASE OF BOVINE SPONGIFORM ENCEPHALOPATHY (BSE) IN CANADA

http://bseusa.blogspot.com/2010/08/report-on-investigation-of-seventeenth.html



Monday, August 30, 2010

Bovine Spongiform Encephalopathy (BSE) CANADA Import Policy for Bovine Animals and Their Products (TAHD-DSAT-IE-2005-9-2) Import Policy updates August

http://madcowtesting.blogspot.com/2010/08/bovine-spongiform-encephalopathy-bse.html



Thursday, August 19, 2010

SCRAPIE CANADA UPDATE Current as of 2010-07-31 The following table lists sheep flocks and/or goat herds confirmed to be infected with scrapie in Canada in 2010.

Current as of: 2010-07-31

http://nor-98.blogspot.com/2010/08/scrapie-canada-update-current-as-of.html



Atypical BSE in Cattle

BSE has been linked to the human disease variant Creutzfeldt Jakob Disease (vCJD). The known exposure pathways for humans contracting vCJD are through the consumption of beef and beef products contaminated by the BSE agent and through blood transfusions. However, recent scientific evidence suggests that the BSE agent may play a role in the development of other forms of human prion diseases as well. These studies suggest that classical type of BSE may cause type 2 sporadic CJD and that H-type atypical BSE is connected with a familial form of CJD.

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.

This study will contribute to a correct definition of specified risk material (SRM) in atypical BSE. The incumbent of this position will develop new and transfer existing, ultra-sensitive methods for the detection of atypical BSE in tissue of experimentally infected cattle.

Responsibilities include:

Driving research at the National and OIE BSE reference lab to ensure project milestones are met successfully. Contributing to the preparation of project progress reports. Directing technical staff working on the project. Communicating and discussing results, progress and future direction with project principle investigator(s). Communicating with collaborative project partners. Qualifications:

Successful completion of a PhD degree in an area focusing on or related to prion diseases. Extensive experience with molecular and/or morphologic techniques used in studying prion diseases and/or other protein misfolding disorders. Ability to think independently and contribute new ideas. Excellent written and oral communication skills. Ability to multitask, prioritize, and meet challenges in a timely manner. Proficiency with Microsoft Office, especially Word, PowerPoint and Excel. How to apply:

Please send your application and/or inquiry to: Dr. Stefanie Czub, DVM, Ph.D. Head, National and OIE BSE Reference Laboratory Canadian Food Inspection Agency Lethbridge Laboratory P.O. Box 640, Township Road 9-1 Lethbridge, AB, T1J 3Z4 Canada

phone: +1-403-382-5500 +1-403-382-5500 ext. 5549 email: stefanie.czub@inspection.gc.ca

Contact Info:

http://www.prionetcanada.ca/detail.aspx?menu=5&dt=293380&app=93&cat1=387&tp=20&lk=no&cat2



Wednesday, March 31, 2010

Atypical BSE in Cattle / position: Post Doctoral Fellow

http://bse-atypical.blogspot.com/2010/03/atypical-bse-in-cattle-position-post.html



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.


http://www.isid.org/14th_icid/


http://ww2.isid.org/Downloads/IMED2009_AbstrAuth.pdf


http://www.isid.org/publications/ICID_Archive.shtml



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.

page 114 ;


http://ww2.isid.org/Downloads/14th_ICID_ISE_Abstracts.pdf



Wednesday, February 24, 2010

Transmissible Spongiform encephalopathy (TSE) animal and human TSE in North America 14th

ICID International Scientific Exchange Brochure -


http://transmissiblespongiformencephalopathy.blogspot.com/2010/02/transmissible-spongiform-encephalopathy.html



TSE

http://transmissiblespongiformencephalopathy.blogspot.com/



Wednesday, July 28, 2010

Atypical prion proteins and IBNC in cattle DEFRA project code SE1796 FOIA Final report

http://bse-atypical.blogspot.com/2010/07/atypical-prion-proteins-and-ibnc-in.html



Saturday, February 28, 2009

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

SEAC 102/2

http://bse-atypical.blogspot.com/2009/02/new-results-on-idiopathic-brainstem.html



Monday, August 9, 2010

National Prion Disease Pathology Surveillance Center Cases Examined (July 31, 2010)

(please watch and listen to the video and the scientist speaking about atypical BSE and sporadic CJD and listen to Professor Aguzzi)

SEE where sporadic cjd in the USA went from 59 cases in 1997, to 216 cases in 2009. a steady increase since 1997. ...TSS

National Prion Disease Pathology Surveillance Center Cases Examined (July 31, 2010)

Year Total Referrals2 Prion Disease Sporadic Familial Iatrogenic vCJD

1997 114 68 59 9 0 0

to

2009 425 259 216 43 0 0

http://www.cjdsurveillance.com/pdf/case-table.pdf



see full text ;

http://prionunitusaupdate2008.blogspot.com/2010/08/national-prion-disease-pathology.html



Wednesday, August 18, 2010

Incidence of CJD Deaths Reported by CJD-SS in Canada as of July 31, 2010

http://creutzfeldt-jakob-disease.blogspot.com/2010/08/incidence-of-cjd-deaths-reported-by-cjd.html



Wednesday, September 08, 2010

Emerging Infectious Diseases: CJD, BSE, SCRAPIE, CWD, PRION, TSE Evaluation to Implementation for Transfusion and Transplantation September 2010

http://vcjdtransfusion.blogspot.com/2010/09/emerging-infectious-diseases-cjd-bse.html



Tuesday, September 14, 2010

Transmissible Spongiform Encephalopathies Advisory Committee; Notice of Meeting October 28 and 29, 2010 (COMMENT SUBMISSION)

http://tseac.blogspot.com/2010/09/transmissible-spongiform_14.html



Tuesday, September 28, 2010

Variant CJD: where has it gone, or has it?

Pract Neurol 2010; 10: 250–251

http://vcjdtransfusion.blogspot.com/2010/09/variant-cjd-where-has-it-gone-or-has-it.html



PRODUCT

1) Plasma Frozen within 24 hours (FP24). Recall # B-2448-10;

2) Red Blood Cells. Recall # B-2449-10;

3) Cryoprecipitated AHF. Recall # B-2450-10;

4) Plasma. Recall # B-2451-10

CODE

1) Units: W038509802210, W038509800965;

2) Units: W038509802210, W038509800965, W038508801111, W038508330725;

3) Unit: W03850830725;

4) Units: W038509801111, W038508330725

RECALLING FIRM/MANUFACTURER

Walter L. Shepeard Community Blood Center, Inc., Augusta, GA, by fax on July 9 and 21, 2010. Firm initiated recall is complete.

REASON

Blood products, collected from a donor considered to be at increased risk for variant Creutzfeldt-Jakob Disease (vCJD), were distributed.

VOLUME OF PRODUCT IN COMMERCE

9 units

DISTRIBUTION

Korea, SC, GA

___________________________________

PRODUCT

Recovered Plasma. Recall # B-2306-10

CODE

Unit: W137508110097

RECALLING FIRM/MANUFACTURER

Lane Memorial Blood Bank, Eugene, OR, by fax on June 10, 2010. Firm initiated recall is complete.

REASON

Blood product, collected from a donor considered to be at increased risk for variant Creutzfeldt-Jakob Disease (vCJD), was distributed.

VOLUME OF PRODUCT IN COMMERCE

1 unit

DISTRIBUTION

KY

___________________________________

PRODUCT

Red Blood Cells (Apheresis) Leukocytes Reduced. Recall # B-2348-10

CODE

Units: W041609075327D (part a and b), 3922801 (part a and b)

RECALLING FIRM/MANUFACTURER

Blood Systems Inc/dba United Blood Services, Meridian, MS, by telephone and fax on May 26, 2010 and May 28, 2010. Firm initiated recall is complete.

REASON

Blood products, collected from a donor considered to be at increased risk for variant Creutzfeldt-Jakob Disease (vCJD), were distributed.

VOLUME OF PRODUCT IN COMMERCE

4 units

DISTRIBUTION

MS

___________________________________

PRODUCT

1) Recovered Plasma. Recall # B-2363-10;

2) Cryoprecipitated AHF, Pooled. Recall # B-2364-10;

3) Red Blood Cells Leukocytes Reduced. Recall # B-2365-10

CODE

1) and 3) Units: 2613522, 2578779;

2) Unit: 2578779

RECALLING FIRM/MANUFACTURER

South Texas Blood and Tissue Center, San Antonio, TX, by fax and e-mail on May 5, 2010. Firm initiated recall is complete.

REASON

Blood products, collected from a donor considered to be at increased risk for variant Creutzfeldt-Jakob Disease (vCJD), were distributed.

VOLUME OF PRODUCT IN COMMERCE

5 units

DISTRIBUTION

TX

___________________________________

END OF ENFORCEMENT REPORT FOR OCTOBER 6, 2010

#

http://www.fda.gov/Safety/Recalls/EnforcementReports/ucm228605.htm



Friday, September 24, 2010

USA Blood products, collected from a donor who was at risk for vCJD, were distributed SEPTEMBER 2010

http://vcjdtransfusion.blogspot.com/2010/09/usa-blood-products-collected-from-donor.html




TSS

Accumulation of L-type Bovine Prions in Peripheral Nerve Tissues

Volume 16, Number 7–July 2010

Dispatch

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

snip...

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 ;


http://www.cdc.gov/eid/content/16/7/pdfs/1151.pdf



http://www.cdc.gov/eid/content/16/7/1151.htm





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 [wais.access.gpo.gov] [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]]

DEPARTMENT OF AGRICULTURE

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 http://www.epa.gov/feddocket to submit or

snip...

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\

snip...

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.

snip...

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.

snip...

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 http://www.oie.int). The guidelines on BSE are contained in

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

snip...end

http://a257.g.akamaitech.net/7/257/2422/01jan20051800/edocket.access.gpo.gov/2005/05-16422.htm

http://a257.g.akamaitech.net/7/257/2422/01jan20051800/edocket.access.gpo.gov/2005/pdf/05-16422.pdf



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 gan@affrc.go.jp

Abstract

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. ...

179

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

snip...

"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; kitamoto@mail.tains.tohoku.ac.jp Symposium Secretariat Kyomi Sasaki TEL +81-22-717-8233 FAX +81-22-717-7656 e-mail: kvomi-sasaki@mail.tains.tohoku.ac.ip


================================


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


http://madcowfeed.blogspot.com/2008/07/importation-of-whole-cuts-of-boneless.html




*****URGENT NOTE HERE ABOUT OIE AND THEIR JUNK SCIENCE ABOUT ATYPICAL BSE*****


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.


http://www.prionetcanada.ca/detail.aspx?menu=5&dt=293380&app=93&cat1=387&tp=20&lk=no&cat2



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.


http://www.isid.org/14th_icid/


http://ww2.isid.org/Downloads/IMED2009_AbstrAuth.pdf


http://www.isid.org/publications/ICID_Archive.shtml



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.

see page 114 ;


http://ww2.isid.org/Downloads/14th_ICID_ISE_Abstracts.pdf




International Society for Infectious Diseases Web: http://www.isid.org/


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

P02.35

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.


http://www.neuroprion.com/pdf_docs/conferences/prion2007/abstract_book.pdf




Wednesday, March 31, 2010

Atypical BSE in Cattle


http://bse-atypical.blogspot.com/2010/03/atypical-bse-in-cattle-position-post.html




Sunday, February 14, 2010

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


http://bseusa.blogspot.com/2010/02/docket-no-fsis-2006-0011-fsis-harvard.html



http://transmissiblespongiformencephalopathy.blogspot.com/2010/02/transmissible-spongiform-encephalopathy.html




2009 UPDATE ON ALABAMA AND TEXAS MAD COWS 2005 and 2006


http://bse-atypical.blogspot.com/2006/08/bse-atypical-texas-and-alabama-update.html




WILL WE EVER KNOW WHAT STRAIN OF BSE THIS OTHER TEXAS MAD COW ?



FDA STATEMENT FOR IMMEDIATE RELEASE

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). ...


http://www.fda.gov/NewsEvents/Newsroom/PressAnnouncements/2004/ucm108292.htm



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.

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


http://www.usda.gov/oig/webdocs/sarc070619.pdf



snip... please see full text ;


http://bse-atypical.blogspot.com/2008/06/mad-cows-and-computer-models-us.html




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


BSE-NON-CONFIRMATION OF DISEASE

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.

snip...

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

CONCLUSION

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.

R BRADLEY

15 MAY 1990

90/5.15/3.2


http://collections.europarchive.org/tna/20090505194948/http://bseinquiry.gov.uk/files/yb/1990/05/15003001.pdf



http://collections.europarchive.org/tna/20090505194948/http://bseinquiry.gov.uk/files/yb/1990/05/15003001.pdf




Tuesday, November 17, 2009

SEAC NEW RESULTS ON IDIOPATHIC BRAINSTEM NEURONAL CHROMATOLYSIS (IBNC) FROM THE VETERINARY LABORATORIES AGENCY (VLA) SEAC 103/1

http://bse-atypical.blogspot.com/2009/11/seac-new-results-on-idiopathic.html




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

http://bse-atypical.blogspot.com/2009/02/new-results-on-idiopathic-brainstem.html



AND THE USDA ET AL KNEW IT TOO ;


""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."


THIS WAS DONE FOR A REASON!


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.

snip.............

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.

snip...


Completely Edited Version PRION ROUNDTABLE

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




Saturday, June 19, 2010

U.S. DENIED UPGRADED BSE STATUS FROM OIE

Greetings,

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.


http://www.efsa.europa.eu/EFSA/efsa_locale-1178620753812_1211902594180.htm



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

please see full text ;


http://www.efsa.europa.eu/en/scdocs/doc/3rax1.pdf



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 ;


http://usdameatexport.blogspot.com/2010/06/us-denied-upgraded-bse-status-from-oie.html




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:


http://www.ars.usda.gov/research/projects/projects.htm?ACCN_NO=408490&showpars=true&fy=2009 <<<


>>> 3. Studies on transmissibility and tissue distribution of atypical BSE isolates in cattle and other species. http://www.ushrl.saa.ars.usda.gov/research/projects/projects.htm?accn_no=408490 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. <<<




http://www.ushrl.saa.ars.usda.gov/research/projects/projects.htm?accn_no=408490




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. <<<




http://foiamadsheepmadrivervalley.blogspot.com/2010/02/final-report-of-testing-of-belgian.html



http://foiamadsheepmadrivervalley.blogspot.com/



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
flounder9@verizon.net


snip...


see full text ;


http://bse-atypical.blogspot.com/2010/06/publication-request-and-foia-request.html



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

snip...

[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?"

http://www.promedmail.org/pls/apex/f?p=2400:1001:568933508083034::NO::F2400_P1001_BACK_PAGE,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.


http://www.recordandoalinda.com/index.php?option=com_content&view=article&id=19:cjd-english-info&catid=9:cjd-ingles&Itemid=8




CJD TEXAS 38 YEAR OLD FEMALE WORKED SLAUGHTERING CATTLE EXPOSED TO BRAIN AND SPINAL CORD MATTER


http://cjdtexas.blogspot.com/2010/03/cjd-texas-38-year-old-female-worked.html




Monday, April 5, 2010

UPDATE - CJD TEXAS 38 YEAR OLD FEMALE WORKED SLAUGHTERING CATTLE EXPOSED TO BRAIN AND SPINAL CORD MATTER


http://prionunitusaupdate2008.blogspot.com/2010/04/update-cjd-texas-38-year-old-female.html




Tuesday, June 1, 2010

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


http://cjdtexas.blogspot.com/2010/06/usa-cases-of-dpcjd-rising-with-24-cases.html



Wednesday, June 16, 2010

Defining sporadic Creutzfeldt-Jakob disease strains and their transmission properties


http://creutzfeldt-jakob-disease.blogspot.com/2010/06/defining-sporadic-creutzfeldt-jakob.html




TRANSMISSIBLE SPONGIFORM ENCEPHALOPATHY UPDATE June 16, 2010


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

snip...

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.

snip...

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.


http://www.jci.org/articles/view/42051?key=456180f4a34aad821c6f#B87



see also ;

Monday, June 14, 2010

A molecular switch controls interspecies prion disease transmission in mice


http://chronic-wasting-disease.blogspot.com/2010/06/molecular-switch-controls-interspecies.html



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


http://chronic-wasting-disease.blogspot.com/2010/05/prion-strain-mutation-determined-by.html



http://chronic-wasting-disease.blogspot.com/



Saturday, June 5, 2010

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


http://nor-98.blogspot.com/2010/06/research-project-transmissible.html




Wednesday, June 16, 2010

Defining sporadic Creutzfeldt-Jakob disease strains and their transmission properties


http://creutzfeldt-jakob-disease.blogspot.com/2010/06/defining-sporadic-creutzfeldt-jakob.html




Wednesday, June 02, 2010

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


http://creutzfeldt-jakob-disease.blogspot.com/2010/06/cjd-annex-h-update-after-death.html





Sunday, August 09, 2009

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


http://creutzfeldt-jakob-disease.blogspot.com/2009/08/cjdstraight-talk-withjames.html



Tuesday, August 18, 2009

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


http://madcowusda.blogspot.com/2009/08/bse-untold-story-joe-gibbs-and.html



Terry S. Singeltary Sr.
P.O. Box 42
Bacliff, Texas USA 77518
flounder9@verizon.net

Atypical BSE in Cattle / position: Post Doctoral Fellow

position: Post Doctoral Fellow Atypical BSE in Cattle

Closing date: December 24, 2009

Anticipated start date: January/February 2010

Employer: Canadian and OIE Reference Laboratories for BSE CFIA Lethbridge Laboratory, Lethbridge/Alberta

The Canadian and OIE reference laboratories for BSE are extensively involved in prion diseases diagnosis and research. With a recent increase in research activities and funding, the laboratory is looking to fill two post doctoral fellow positions. Both positions will be located at the Canadian Food Inspection Agency (CFIA) Lethbridge Laboratory which offers biosaftey level 3 (BSL3) and BSL2 laboratory space and is well equipped for molecular and morphologic prion research. The facility also has a BSL3 large animal housing wing and a state of the art post mortem room certified for prion work. Successful candidates will have the opportunity to visit other laboratories to cooperate in various aspects of the projects and to be trained in new techniques and acquire new skills. With a recent increase in prion disease expertise and research in Alberta and Canada, these positions will offer significant exposure to cutting edge prion science via videoconferencing, meetings, workshops and conferences. These interactions will also provide a valuable opportunity to present research findings and discuss potential future work opportunities and collaborations with other Canadian and international research groups.

Atypical BSE in Cattle

BSE has been linked to the human disease variant Creutzfeldt Jakob Disease (vCJD). The known exposure pathways for humans contracting vCJD are through the consumption of beef and beef products contaminated by the BSE agent and through blood transfusions. However, recent scientific evidence suggests that the BSE agent may play a role in the development of other forms of human prion diseases as well. These studies suggest that classical type of BSE may cause type 2 sporadic CJD and that H-type atypical BSE is connected with a familial form of CJD.

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.

This study will contribute to a correct definition of specified risk material (SRM) in atypical BSE. The incumbent of this position will develop new and transfer existing, ultra-sensitive methods for the detection of atypical BSE in tissue of experimentally infected cattle.

Responsibilities include:

Driving research at the National and OIE BSE reference lab to ensure project milestones are met successfully. Contributing to the preparation of project progress reports. Directing technical staff working on the project. Communicating and discussing results, progress and future direction with project principle investigator(s). Communicating with collaborative project partners. Qualifications:

Successful completion of a PhD degree in an area focusing on or related to prion diseases. Extensive experience with molecular and/or morphologic techniques used in studying prion diseases and/or other protein misfolding disorders. Ability to think independently and contribute new ideas. Excellent written and oral communication skills. Ability to multitask, prioritize, and meet challenges in a timely manner. Proficiency with Microsoft Office, especially Word, PowerPoint and Excel. How to apply:

Please send your application and/or inquiry to: Dr. Stefanie Czub, DVM, Ph.D. Head, National and OIE BSE Reference Laboratory Canadian Food Inspection Agency Lethbridge Laboratory P.O. Box 640, Township Road 9-1 Lethbridge, AB, T1J 3Z4 Canada

phone: +1-403-382-5500 +1-403-382-5500 ext. 5549 email: stefanie.czub@inspection.gc.ca

Contact Info:


http://www.prionetcanada.ca/detail.aspx?menu=5&dt=293380&app=93&cat1=387&tp=20&lk=no&cat2



The scoop on changes to how CFIA reports BSE

The last case of BSE certainly caused a flap in the industry, even though it’s not unexpected to find additional cases of Bovine Spongiform Encephalopathy as Canada’s effective control measures eliminate the disease from the national herd. What was different this time around was the way the Feb. 25 event was announced, as it was the first case to fall under a new reporting policy announced by the CFIA last August.

The CFIA revised how it reports online for disease detections in farmed animals to provide a more comprehensive view of Canada’s animal health status. The intent was to standardize the agency’s approach to BSE with other federally reportable diseases, said George Shaw, vice-president of public affairs, CFIA.

There are about 30 reportable diseases and the CFIA doesn’t issue a release on any of those in real time unless there is a significant human or animal health risk, so the agency wanted to put BSE in the same category as the other ones, he said.

The new reporting process still sees key trading partners, governments and some industry stakeholders notified in real time of confirmed cases of BSE and moves the public notification to a new, animal health disease report updated monthly on the agency website.

Although an information bulletin explaining why the agency was moving in this direction and what changes it would entail was posted on the CFIA website last August, it wasn’t until Feb. 25 that the new process was put to the test.

It’s a given that there will be hiccups when implementing new systems and this new policy was no exception. While trading partners, including the appropriate authorities in Korea, were notified right away, industry notification on Feb. 25 seemingly wasn’t as broad as it had been under the former process. The result was that many in the industry were left out of the loop while those unaware of the reporting changes assumed the CFIA would post a notification on their website as per usual. R-Calf, meanwhile, issued a release flinging accusations that Canada was hiding BSE cases.

Looking back on the Feb. 25 experience, the CFIA said that some fine-tuning needs to happen around what information is shared and when. Back in 2003, the process involved the agency issuing a release, which later evolved into a short notice that would go up on its website, a document shared with industry and trading partners to give them a heads-up.

The agency didn’t do that on Feb. 25 because the public piece was moved to the new monthly report that captures all animal health diseases in one spot.

Going forward, the agency will continue to fine tune its new reporting policy. When future cases of BSE are confirmed the CFIA will still issue a short summary with all the pertinent details of the case in real time to key trading partners and governments, and intends to alert a broader base of industry stakeholders than reached on Feb. 25. The factual paragraph is intended to ensure governments, trading partners and industry stakeholders have the same information.

If industry gets a call from media or stakeholders in the interim, they will be equipped with the information to deal with it.

Notification to the OIE is done on a six-month reporting cycle. Shaw said Canada has reached a point of credibility under the controlled-risk scenario that its not obliged to do immediate reporting on incidents of BSE.

One of the recommendations to come out of the National Beef Value Chain Roundtable in Calgary March 16-17 was that the Government of Canada announce all future cases of BSE in a timely fashion with appropriate information.


http://www.cattle.ca/action-news/03-29-10-email.html



Damn shame Canada is now going to take pages from the books of the USDA. reminds me of that mad cow testing bungle in Texas, the 48 hour BSE testing turn around that took 7+ months and finally an act of Congress OIG and the Honorable Fong to finally get that cow confirmed. all the while another suspect specimen was sitting on a shelf cooling off to for some 4 months for some kind of BSe reasons, all the while the USDA and the OIE were putting the final touches on the BSE MRR policy to override the BSE GBR risk assessments and policy there from ;

TRADE JUNK SCIENCE TO FOLLOW ;

In 2009, the World Animal Health Organization (OIE) recognized Nor98-like scrapie as a separate disease from classical scrapie because of differences in laboratory findings, transmissibility, and distribution. This determination means that Nor98-like scrapie is not a reportable disease to OIE, and should be of no trade concern.

How has APHIS’ policy changed regarding Nor98-like scrapie?

APHIS will no longer require the depopulation or movement restriction of Nor98-like scrapie exposed sheep and goats. APHIS will propose changes to the Code of Federal Regulations (CFR) in 2010 to allow the APHIS Administrator to eliminate or reduce post exposure requirements for certain scrapie types (or certain/specific types of scrapie)-- such as Nor98-like scrapie -- that are determined to pose minimal risk of lateral transmission under natural conditions.

SNIP...

The National Animal Health Laboratory Network and NAIS are just two ways we’re using science and technology to protect animal health. On a global scale, we believe in international accepted science-based standards for animal health. We think it is appropriate to establish worldwide trade based on these standards. We’ve made a commitment to work toward that goal, and we expect our trading partners to do the same. We took a major step forward in May, when the OIE formally classified the United States as a controlled risk country for BSE. I’ll continue to press our trading partners to align their own import regulations with OIE guidelines, and give us full market access for our live cattle, beef and beef products, regardless of age.

SNIP...

Dr. DeHaven has often represented the United States in delicate and often difficult trade negotiations. As the former U.S. Chief Veterinary Officer and U.S. delegate to the OIE, he routinely used his diplomatic skills as he facilitated agreements that are science-based. He was instrumental in building consensus that led to the current OIE BSE chapter

SNIP...


http://www.usaha.org/meetings/2007/2007_USAHA_Proceedings.pdf



SEE FULL TEXT ;

Sent: Sunday, March 28, 2010 10:16 PM

Subject: Nor-98 atypical Scrapie, atypical BSE, spontaneous TSE, trade policy, sound science ?


http://nor-98.blogspot.com/2010/03/nor-98-atypical-scrapie-atypical-bse.html



OIE Procedure for Validation and Certification of Diagnostic Assays

OIE Procedure for Validation and Certification of Diagnostic Assays

Abstract sheet

Name of the diagnostic kit: TeSeE™ WESTERN BLOT

Manufacturer: Bio-Rad

OIE Approval number: 20090105

Date of Registration: May 2009

Disease: Prion associated diseases (Scrapie, Bovine Spongiform Encephalopathy, Chronical Waste Disease)

Pathogen Agent: Abnormal prion protein PrPRes

Type of Assay: The TeSeE™ WESTERN BLOT kit is based on the Western blotting technique and allows detection of abnormal prion protein PrPRes after protease digestion.

Purpose of Assay: Certified by the OIE in May 2009 as fit for the post mortem detection of Transmissible Spongiform Encephalopathies (TSEs) in cattle (Bovine Spongiform Encephalopathy, BSE), in ovine and caprine (BSE and scrapie), and in cervids (Chronic Wasting Disease, CWD) and as validated fit for the following purposes:

1. To confirm TSE suspected positive samples detected at the screening laboratories in countries with active/passive surveillance programmes. Any sample with a negative result according to the TeSeE™ WESTERN BLOT assay interpretation criteria, following a positive rapid test result, should be tested with one of the other OIE certified confirmatory methods, Immunohistochemistry (IHC) or Scrapie-associated fibrils (SAF)-Immunoblot;

2. To confirm the prevalence of infection with one of the TSE associated diseases (BSE, scrapie, CWD) in the context of an epidemiological survey in a low prevalence country;

3. To estimate prevalence of infection to facilitate risk analysis (e.g. surveys, implementation of disease control measures) and to assist the demonstration of the efficiency of eradication policies.

Species and Specimen: Bovine (obex), Cervids (obex and lymph nodes) and ovine (obex)

snip...

see full text ;


http://www.oie.int/vcda/eng/Registre/Abstract%20sheet_OIE%20Register_TeSeEWB_v1.pdf


Completely Edited Version

PRION ROUNDTABLE

2003

Dr. Linda Detwiler

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.

snip.............

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.

snip...

FULL TEXT;

Completely Edited Version PRION ROUNDTABLE

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

=============================


http://madcowtesting.blogspot.com/



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.

snip...

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



http://www.usda.gov/oig/webdocs/sarc070619.pdf




CJD TEXAS 38 YEAR OLD FEMALE WORKED SLAUGHTERING CATTLE EXPOSED TO BRAIN AND SPINAL CORD MATTER


http://cjdtexas.blogspot.com/2010/03/cjd-texas-38-year-old-female-worked.html


http://transmissiblespongiformencephalopathy.blogspot.com/




>>> 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. <<<




http://www.euroweeklynews.com/news_spanish_press.html




http://74.125.47.132/search?q=cache:MfWnPKrMDEwJ:www.euroweeklynews.com/news_spanish_press.html+Principe+de+Asturias+in+Alcala+de+Henares+cjd&cd=5&hl=en&ct=clnk&gl=us



Topic: Emerging Infectious Diseases Preferred type of presentation:


International Scientific Exchange


This abstract has been ACCEPTED. #0670:


Transmissible Spongiform encephalopathy (TSE) animal and human TSE in North America update October 2009


Authors: T. Singeltary; Bacliff, TX/US


Title: Transmissible Spongiform encephalopathy (TSE) animal and human TSE in North America update October 2009


Body: 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.


Keywords: Transmissible Spongiform Encephalopathy Creutzfeldt Jakob Disease Prion


see page 114 ;


http://ww2.isid.org/Downloads/14th_ICID_ISE_Abstracts.pdf


http://www.isid.org/14th_icid/


http://www.isid.org/publications/ICID_Archive.shtml


http://ww2.isid.org/Downloads/IMED2009_AbstrAuth.pdf



Sent: Friday, January 29, 2010 3:23 PM

Subject: 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.


http://www.isid.org/14th_icid/


http://ww2.isid.org/Downloads/IMED2009_AbstrAuth.pdf


http://www.isid.org/publications/ICID_Archive.shtml



Monday, October 19, 2009



Atypical BSE, BSE, and other human and animal TSE in North America Update October 19, 2009


snip...



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




I look forward to further transmission studies, and a true ENHANCED BSE/atypical BSE surveillance program put forth testing all cattle for human and animal consumption for 5 years. a surveillance program that uses the most sensitive TSE testing, and has the personnel that knows how to use them, and can be trusted. I look forward to a stringent mad cow feed ban being put forth, and then strictly enforced. we need a forced, not voluntary feed ban, an enhanced feed ban at that, especially excluding blood. we need some sort of animal traceability. no more excuses about privacy. if somebody is putting out a product that is killing folks and or has the potential to kill you, then everybody needs to know who they are, and where that product came from. same with hospitals, i think medical incidents in all states should be recorded, and made public, when it comes to something like a potential accidental transmission exposure event. so if someone is out there looking at a place to go have surgery done, if you have several hospitals having these type 'accidental exposure events', than you can go some place else. it only makes sense. somewhere along the road, the consumer lost control, and just had to take whatever they were given, and then charged these astronomical prices. some where along the line the consumer just lost interest, especially on a long incubating disease such as mad cow disease i.e. Transmissible Spongiform Encephalopathy. like i said before, there is much more to the mad cow story than bovines and eating a hamburger, we must start focusing on all TSE in all species. ...TSS



SEE SOME REFERENCES HERE ;



http://bse-atypical.blogspot.com/2009/10/atypical-bse-bse-and-other-human-and.html



Archive Number 20100405.1091 Published Date 05-APR-2010

Subject PRO/AH/EDR> Prion disease update 1010 (04)

snip...

[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?"



http://www.promedmail.org/pls/apex/f?p=2400:1001:568933508083034::NO::F2400_P1001_BACK_PAGE,F2400_P1001_PUB_MAIL_ID:1000,82101


Sent: Friday, April 16, 2010 11:38 AM

Subject: PRO-MED ATYPICAL SCRAPIE


Background ----------- "Retrospective studies have identified cases predating the initial identification of this form of scrapie, and epidemiological studies have indicated that it does not conform to the behaviour of an infectious disease, giving rise to the hypothesis that it represents spontaneous disease. However, atypical scrapie isolates have been shown to be infectious experimentally, through intracerebral inoculation in transgenic mice and sheep. [Many of the neurological diseases can be transmitted by intracerebral inoculation, which causes this moderator to approach intracerebral studies as a tool for study, but not necessarily as a direct indication of transmissibility of natural diseases. - Mod.TG]

"The 1st successful challenge of a sheep with 'field' atypical scrapie from an homologous donor sheep was reported in 2007.

"Results -------- "This study demonstrates that atypical scrapie has distinct clinical, pathological, and biochemical characteristics which are maintained on transmission and sub-passage, and which are distinct from other strains of transmissible spongiform encephalopathies in the same host genotype.

"Conclusions ------------ Atypical scrapie is consistently transmissible within AHQ homozygous sheep, and the disease phenotype is preserved on sub-passage."

Lastly, this moderator wishes to thank Terry Singletary for some of his behind the scenes work of providing citations and references for this posting. - Mod.TG]

The HealthMap/ProMED-mail interactive map of Australia is available at . - Sr.Tech.Ed.MJ]


http://www.promedmail.org/pls/otn/f?p=2400:1001:962575216785367::NO::F2400_P1001_BACK_PAGE,F2400_P1001_PUB_MAIL_ID:1000,81729



Sunday, April 18, 2010

SCRAPIE AND ATYPICAL SCRAPIE TRANSMISSION STUDIES A REVIEW 2010


http://scrapie-usa.blogspot.com/2010/04/scrapie-and-atypical-scrapie.html





Tuesday, August 12, 2008

Biosafety in Microbiological and Biomedical Laboratories Fifth Edition 2007 (occupational exposure to prion diseases)


http://creutzfeldt-jakob-disease.blogspot.com/2008/08/biosafety-in-microbiological-and.html



Subject: COMMONWEALTH OF AUSTRALIA Hansard

Import restrictions on beef FRIDAY, 5 FEBRUARY 2010

AUSTRALIA COMMONWEALTH OF AUSTRALIA Proof Committee Hansard

SENATE RURAL AND REGIONAL AFFAIRS AND TRANSPORT REFERENCES COMMITTEE

Reference: Import restrictions on beef FRIDAY, 5 FEBRUARY 2010 CANBERRA

CONDITIONS OF DISTRIBUTION This is an uncorrected proof of evidence taken before the committee. It is made available under the condition that it is recognised as such.


BY AUTHORITY OF THE SENATE [PROOF COPY] TO EXPEDITE DELIVERY, THIS TRANSCRIPT HAS NOT BEEN SUBEDITED


SNIP...


Friday, 5 February 2010 Senate RRA&T 1 RURAL AND REGIONAL AFFAIRS AND TRANSPORT


Committee met at 9.01 am


CHAIR (Senator Nash)—I declare open this public hearing of the Rural and Regional Affairs and Transport References Committee. The committee is hearing evidence on the committee’s inquiry into the impact and consequences of the government’s decision to relax import restrictions on beef. Before the committee starts taking evidence I remind all witnesses that, in giving evidence to the committee, they are protected by parliamentary privilege. It is unlawful for anyone to threaten or disadvantage a witness on account of evidence given to a committee and such action may be treated by the Senate as a contempt. It is also a contempt to give false or misleading evidence to a committee. The committee prefers all evidence to be given in public but, under the Senate’s resolutions, witnesses have the right to request to be heard in private session. It is important that witnesses give the committee notice if they intend to ask to give evidence in camera. If a witness objects to answering a question, the witness should state the ground upon which the objection is taken and the committee will determine whether it will insist on an answer, having regard to the ground which is claimed. If the committee determines to insist on an answer, a witness may request that the answer be given in camera. Such a request may, of course, also been made at any other time. On behalf of the committee, I thank all those who have made submissions and sent representatives here today for their cooperation in this inquiry.



RRA&T 2 Senate Friday, 5 February 2010


RURAL AND REGIONAL AFFAIRS AND TRANSPORT


[9.03 am]


BELLINGER, Mr Brad, Chairman, Australian Beef Association


CARTER, Mr John Edward, Director, Australian Beef Association


CHAIR—Welcome. Would you like to make an opening statement?


Mr Bellinger—Thank you. The ABA stands by its submission, which we made on 14 December last year, that the decision made by the government to allow the importation of beef from BSE affected countries is politically based, not science based. During this hearing we will bring forward compelling new evidence to back up this statement. When I returned to my property after the December hearing I received a note from an American citizen. I will read a small excerpt from the mail he sent me in order to reinforce the dangers of allowing the importation of beef from BSE affected countries. I have done a number of press releases on this topic, and this fellow has obviously picked my details up from the internet. His name is Terry Singeltary and he is from Bacliff, Texas. He states, and rightfully so:


You should be worried. Please let me explain. I’ve kept up with the mad cow saga for 12 years today, on December 14th 1997, some four months post voluntary and partial mad cow feed ban in the USA, I lost my mother to the Heinemann variant Creutzfeldt-Jakob disease (CJD). I know this is just another phenotype of the infamous sporadic CJDs. Here in the USA, when USA sheep scrapie was transmitted to USA bovine, the agent was not UK BSE—it was a different strain. So why then would human TSE from USA cattle look like UK CJD from UK BSE? It would not. So this accentuates that the science is inconclusive still on this devastating disease. He goes on to state:


The OIE— the International Organisation of Epizootics, the arm of the WTO— is a failed global agent that in my opinion is bought off via bogus regulations for global trade and industry reps. I have done this all these years for nothing but the truth. I am a consumer, I eat meat, but I do not have to sit idly by and see the ignorance and greed of it all while countless numbers of humans and animals are being exposed to the TSE agents. All the USA is interested in is trade, nothing else matters.


Even Dr Stanley Prusiner, who incidentally won the Nobel Health Prize in 1997 for his work on the prion—he invented the word ‘prion’, or it came from him—states:


The BSC policy was set up for one purpose only, trade—the illegal trading of all strains of TSE globally throughout North America, which is home to CBSC, IBSC and HBSC, many scrapie strains and two strains of CJD to date. (please note typo error, those should have read cBSE, lBSE, and hBSE...tss)


I would also like, while I have the opportunity, to explain the beef-off-the-shelves myth. At the first Senate hearing on 14 December, it was explained that the reason why they allowed BSC beef into Australia was the beef-off-the-shelves policy, whereby if we found a case of BSC in Australia they would have to recall all—


Friday, 5 February 2010 Senate RRA&T 3 RURAL AND REGIONAL AFFAIRS AND TRANSPORT


Senator HEFFERNAN—Which of course is total BS.


Mr Bellinger—Correct. This is written in the FSANZ document—Food Standards Australia New Zealand. Why isn’t this same policy in New Zealand? It is not—it is only in Australia. We are the only country in the world to have this idiotic policy. So we again call for the tabling of the WTO obligations paperwork. We do not believe that exists.


Mr Carter—We have an additional concern about human health. We are not scientists, but on 18 December, four days after the last hearing here, the BBC reported a new wave of deaths due to variant CJD linked to eating BSE infected beef could be underway. This is based on the work of Professor John Collinge of the National Prion Clinic, who reported that a 2009 death in Scotland was from a different genetic pool to that of the 166 deaths already reported in the UK. Those are all thought to share one gene, but Professor Collinge and his colleagues estimate that up to 350 people in this new group, represented by the person who died in Scotland, could get CJD. He thinks that CJD has moved into a new phase, and the incubation period is a long one. We tender the Australian Red Cross donor policy sheet, which bears out what Senator Back brought up last time, questioning the Chief Medical Officer, and we say that blood from people who were in the UK between 1980 and 1996 is not acceptable. That is the current ruling. We believe this now should be extended to anyone who has visited the UK, and this new evidence should ensure that Australia revisits the science of CJD.


CHAIR—Thank you, Mr Carter. Before we kick off, can I just remind colleagues that we are short of time today, so I ask that we do not traverse ground the we have previously covered and make sure that we stick to new information that is required. Mr Bellinger, when you started you referred to your view that this decision to allow the importation was politically based. I know you are going to go into this in the course of the next 20 minutes or so, but could you just give us a quick outline of what your definition of politically based is and why you think the decision was politically based?


Mr Bellinger—On the lowering of BSE standards: if you go back to 2006, for example, there were five categories for describing countries that had BSE and Australia was in the category for BSE free. Suddenly, by the time the United States got their third instance of BSE, through the influence of Robert Zoellick—who was the trade minister that signed the BSE corresponding side letter in 2004 and was George Bush’s appointment to the WTO—they suddenly changed the five categories to three categories and, instead of being BSE free, Australia became BSE negligible risk. At the time I put out a press release alerting the media to the dangers of this happening, and we are coming to the stage here when suddenly our government is saying, ‘Now let’s allow the importation of beef from BSE affected countries.’ I believe that the WTO has been influenced by large multinational meat processors and retailers to change and allow the trading of BSE beef throughout the world.


CHAIR—Thanks, Mr Bellinger.


Mr Carter—Of course, the side letter that Minister Vaile signed was at the request of Mr Zoellick, who is now in the position that Mr Bellinger has explained.


Senator HEFFERNAN—I just want to put the committee on notice that, if we do not get through what we have got to get through today, I suggest we have another hearing, because this


RRA&T 4 Senate Friday, 5 February 2010 RURAL AND REGIONAL AFFAIRS AND TRANSPORT


is the greatest ambush of Australia’s farmers of all time by a government. The evidence given at the last meeting was deadset lies. The proposition that this whole change of government policy was led by the industry is a deadset lie. While Simon Crean might want to change his mind because of the WTO and his lack of knowledge, the Australian beef industry, as you know, is under great challenge, not only from the currency but also from the undermining of our markets. This is a disgrace.


SNIP...


PLEASE SEE FULL TEXT ;


*************


Tuesday, March 16, 2010

COMMONWEALTH OF AUSTRALIA Hansard
Import restrictions on beef FRIDAY, 5 FEBRUARY 2010
AUSTRALIA COMMONWEALTH OF AUSTRALIA
Proof Committee Hansard


snip...see full text 110 pages ;


http://www.aph.gov.au/hansard/senate/commttee/S12742.pdf


for those interested, please see much more here ;


http://docket-aphis-2006-0041.blogspot.com/2010/03/commonwealth-of-australia-hansard.html


BSE MRR TSS, R-CALF ON CANADA VS USA


Bill Rancher Joined: 10 Feb 2005 Posts: 1418 Location: GWN Posted: Fri Jan 05, 2007 9:49 am Post subject:


Texan wrote: Hey Terry, I'd like to get a little further clarification on something if/when you have time. I'm not sure if I'm reading you correctly....


flounder wrote: This is what sank my battleship in regards to testifying for r-calf. they actually appoached me about it, but i told them i would be glad to testify, but i was not stopping at the Canadian border, my testimony was to come south as well if given the opportunity. and that ended that, but i did supply them with a load of data, for whatever that was worth. I highlighted the parts that confuse me. This almost makes it seem as if R-CALF was asking you to testify for them, but changed their mind when they found out that you were going to tell the WHOLE truth, instead of just the truth as regards Canadian imports. I thought that R-CALF was only interested in the WHOLE truth - not just the selected parts of the truth that fit their protectionist agenda? After reading your post, it makes a person wonder. Maybe I read it wrong... Am I reading this correctly, Terry? That can't be right, can it? Thanks.


snip...


I was wondering exactly the same thing Texan.

_________________


Canadian Beef....A cut above the rest!


my answer to big muddy from canada ;


http://ranchers.net/forum/viewtopic.php?t=15704&postdays=0&postorder=asc&start=12


http://ranchers.net/forum/viewtopic.php?t=15704&postdays=0&postorder=asc&start=24


http://ranchers.net/forum/viewtopic.php?t=15704&postdays=0&postorder=asc&start=36


http://ranchers.net/forum/viewtopic.php?t=15704&postdays=0&postorder=asc&start=48


full text submission ;


http://www.regulations.gov/search/Regs/contentStreamer?objectId=09000064801f3412&disposition=attachment&contentType=crtext



Monday, November 30, 2009


USDA AND OIE COLLABORATE TO EXCLUDE ATYPICAL SCRAPIE NOR-98 ANIMAL HEALTH CODE


http://nor-98.blogspot.com/2009/11/usda-and-oie-collaborate-to-exclude.html



“The U.S. has lower sanitary and phyto-sanitary standards (SPS) for imports than many other countries, especially those concerning bovine spongiform encephalopathy (BSE). These low standards have made the U.S. a dumping ground for beef from the countries that have experienced BSE problems. Food Safety and SPS issues continue to be problematic for our industry, as some countries comply with OIE standards, while others ignore them either for cultural reasons, or too often use them as trade barriers. The USITC October 7, 2008 release reported, ‘U.S. beef processors and beef cattle ranchers lose billions of dollars in export opportunities each year because of animal health and food safety measures in other countries that are inconsistent with international standards and vary by country.



http://www.cattlenetwork.com/USCA-Testifies--Before-USITC/2010-03-03/Article_Latest_News.aspx?oid=996238&fid=CN-LATEST_NEWS_



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

Subject: RE: Questions on your testing plans.

Date: Mon, 9 Apr 2007 13:01:30 -0500 From: Joe B. Meng To: CC: References:

XXXXXXXX

As of our most recent conversations, it is still our intent to test every animal.

The testing would be uniform for all programs.

Our certification for all Creekstone brands (Premium, Natural and International) currently requires an "A" maturity animal. These are considered to be 30 months and younger as determined by detention. It has been our experience that this system errs on the side of caution as we have had numerous age-verified animals well under 30 months to be excluded based on detention. All countries to which we export, except Japan, require under 30 months and have approved the detention process.

We are asking that USDA and FSIS supervise all testing at our expense and any inconclusive would be sent to Ames, IA for confirmatory testing. Once they have a questionable sample, the same procedures will be followed that are currently approved. In the past, that has included announcing the inconclusive as well as the final result. As you know, the two native born cases were determined to be atypical, but I don't believe that was announced at the same time as the positive results were announced, so I'm not certain on USDA policy for announcing the strain.

We have had conversations with both BioRad and IDEXX, both of which are approved by USDA. Most of the equipment in the lab is from BioRad, but some of that would need to be replaced with updated equipment. I have met with representatives of both companies and they tell me we could have the new equipment in place and personnel training updated inside of two weeks. Both companies would keep people on site for the early stages of testing. The lab has already been approved so we could be ready fairly quickly unless USDA finds a way to complicate the process. Additionally, both BioRad and IDEXX have looked at the facility and tell us it is superior to the currently approved facilities.

Joe Bill

________________________________

TSS

Friday, August 29, 2008

CREEKSTONE VS USDA COURT OF APPEALS, BUSH SAYS, NO WAY, NO HOW


http://madcowtesting.blogspot.com/2008/08/creekstone-vs-usda-court-of-appeals.html


LIKE i said before, the OIE not only sold their soul to the devil over the BSE MRR, they sold yours too ;

Wednesday, February 10, 2010

NAIS MAD COW TRACEABILITY DUMPED BY USDA APHIS 2010


http://naiscoolyes.blogspot.com/2010/02/nais-mad-cow-traceability-dumped-by.html


Docket APHIS-2006-0041 Docket Title Bovine Spongiform Encephalopathy; Minimal-Risk Regions; Importation of Live Bovines and Products Derived from Bovines Commodities Docket Type Rulemaking Document APHIS-2006-0041-0001 Document Title Bovine Spongiform Encephalopathy; Minimal-Risk Regions; Importation of Live Bovines and Products Derived From Bovines Public Submission APHIS-2006-0041-0028.1 Public Submission Title Attachment to Singletary comment

January 28, 2007

Greetings APHIS,

I would kindly like to submit the following to ;

BSE; MRR; IMPORTATION OF LIVE BOVINES AND PRODUCTS DERIVED FROM BOVINES [Docket No. APHIS-2006-0041] RIN 0579-AC01


http://www.regulations.gov/fdmspublic/ContentViewer?objectId=09000064801f8152&disposition=attachment&contentType=msw8



THE OIE has now shown they are nothing more than a National Trading Brokerage for all strains of animal TSE. 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.

...snip

IT'S as obvious as day and night, either Larry, Curley, and Mo have been at the helm of the USDA/APHIS/FSIS/FDA/CDC/NIH et al for many many years, or the incompetence of these agencies are so inept, either through ignorance and or just too overweight with industry reps., they then should be all done away with and a single agency brought forth, and if not, how will you correct this ongoing problem ?

Thank you, I am sincerely disgusted,

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

...snip

full text ;

Sunday, February 14, 2010

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

http://bseusa.blogspot.com/2010/02/docket-no-fsis-2006-0011-fsis-harvard.html

PLEASE SEE FULL TEXT 98 PAGES HERE ;


http://www.fsis.usda.gov/OPPDE/Comments/2006-0011/2006-0011-1.pdf



The most recent assessments (and reassessments) were published in June 2005 (Table I; 18), and included the categorisation of Canada, the USA, and Mexico as GBR III. Although only Canada and the USA have reported cases, the historically open system of trade in North America suggests that it is likely that BSE is present also in Mexico.


http://www.oie.int/boutique/extrait/06heim937950.pdf



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,


http://www.oie.int/eng/Session2007/RF2006.pdf



Tuesday, July 14, 2009

U.S. Emergency Bovine Spongiform Encephalopathy Response Plan Summary and BSE Red Book Date: February 14, 2000 at 8:56 am PST

WHERE did we go wrong $$$


http://madcowtesting.blogspot.com/2009/07/us-emergency-bovine-spongiform.html



SEE FULL TEXT OF ALL THIS HERE ;

2009 UPDATE ON ALABAMA AND TEXAS MAD COWS 2005 and 2006


http://bse-atypical.blogspot.com/2006/08/bse-atypical-texas-and-alabama-update.html



BSE MRR BOUGHT AND PAID FOR BY YOUR LOCAL CATTLE DEALER I.E. USDA ET AL !


Japan had better hold tight to the '20 months or younger' rule. Australia better hold tight period, and Korea and Taiwan, they had better keep a keen eye as well, In 2010 highly suspect banned mad cow protein is still in commerce, and very young humans are dying from CJD, of long duration, with psychotic symptoms. WHY has this case been kept secret ?


>>> 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, March 29, 2010 Irma Linda Andablo CJD Victim, she died at 38 years old on February 6, 2010 in Mesquite Texas <<<



http://cjdtexas.blogspot.com/2010/03/cjd-texas-38-year-old-female-worked.html




http://transmissiblespongiformencephalopathy.blogspot.com/




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