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TAHC accepting comments on two new rule proposals

Texas

The Texas Animal Health Commission (Commission) is currently accepting public comments on rule proposals that were authorized during the Jan. 24 Commission meeting.

The Commission proposed amendments to Chapter 51, entitled "Entry Requirements." The purpose of the proposed amendments are to make Texas' entry requirements more consistent with current national interstate movement standards.

The Commission is specifically amending Section 51.3 entitled "Exceptions," to clarify and modify interstate entry requirements for sheep, goats and swine. The first amendment will waive the prior requirement for an entry permit and certificate of veterinary inspection for swine consigned directly to slaughter, or consigned to a specifically approved livestock market from their farm of origin.

The proposed changes to Section 51.3 would also no longer require an entry permit for sheep and goats consigned from United States Department of Agriculture sanctioned "Consistent States," which means they have an active scrapie surveillance and control program.

The Commission is also removing a requirement related to Vesicular stomatitis in Section 51.7. Currently, when VS has been diagnosed in another state, the veterinarian issuing the CVI must write on it that any equine, bovine, porcine, caprine, ovine, or cervidae entering Texas from that state have not been exposed to the disease. The Commission has determined that other state's quarantine and movement restriction safeguards are adequate to ensure exposed animals are not moving, without requiring the written statement. The Texas requirement prohibiting entry of certain livestock from a premises or area under quarantine for vesicular stomatitis will be left in place to protect Texas livestock.

In Section 51.14, the Commission is removing the requirement that swine imported into Texas for feeding, breeding, or exhibition purposes must be accompanied by a certificate of veterinary inspection certifying that swine have not been exposed to hog cholera, which is now called Classical Swine Fever. CSF has been eradicated from the United States for several years so this requirement is no longer necessary.

The previous rule proposals have a comment period of 30 days. They may be commented on until 5 p.m. on March 18. A detailed description of the proposed rules can be found on the TAHC website under Rule Proposals www.tahc.state.tx.us/regs/proposals.html.

The Commission also proposed amendments to Chapter 35, entitled "Brucellosis." The Commission is proposing to remove the Brucellosis test requirement for change of ownership of adult sexually intact cattle, and to add a requirement that cattle be permanently and officially identified when there is a change of ownership.

On Aug. 1, 2011, the Commission ceased to enforce the requirement for a brucellosis test at change of ownership due to a lack of funds to supplement the cost of testing at livestock markets. The agency is therefore proposing to amend the rule to officially end the test requirement. Historically, cattle that were tested for brucellosis had permanent official identification (such as ear tags) applied at the same time. This practice was a significant asset to the agency's ability to successfully track or trace cattle as needed for all disease programs, not just brucellosis. The identification capability was also lost at the time that testing requirements ceased. The Commission is now proposing to require that all cattle that are parturient or post parturient or 18 months of age and older, except steers and spayed heifers, changing ownership within Texas shall be officially identified with Commission approved permanent identification.

This particular rule has a comment period of 60 days. It may be commented on until 5 p.m. on April 17. A detailed description of the proposed rule can be found on the TAHC website under Rule Proposals at www.tahc.state.tx.us/regs/proposals.html.

Comments on the TAHC's proposed regulations must be submitted in writing to Carol Pivonka, Texas Animal Health Commission, 2105 Kramer Lane, Austin, Texas 78758, by fax at 512-719-0721 or by email to comments@tahc.state.tx.us.

The Commission appreciates any comments on issues addressed in this document for consideration during drafting of the rule.

Founded in 1893, the Texas Animal Health Commission works to protect the health of all Texas livestock, including: cattle, swine, poultry, sheep, goats, equine animals, and exotic livestock.

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Records 2 Reader Comments Terry S. Singeltary Sr. — 03/18/2012 03:03:54 part II Monday, December 1, 2008 When Atypical Scrapie cross species barriers Authors Andreoletti O., Herva M. H., Cassard H., Espinosa J. C., Lacroux C., Simon S., Padilla D., Benestad S. L., Lantier F., Schelcher F., Grassi J., Torres, J. M., UMR INRA ENVT 1225, Ecole Nationale Veterinaire de Toulouse.France; ICISA-INlA, Madrid, Spain; CEA, IBiTec-5, DSV, CEA/Saclay, Gif sur Yvette cedex, France; National Veterinary Institute, Postboks 750 Sentrum, 0106 Oslo, Norway, INRA IASP, Centre INRA de Tours, 3738O Nouzilly, France. Content Atypical scrapie is a TSE occurring in small ruminants and harbouring peculiar clinical, epidemiological and biochemical properties. Currently this form of disease is identified in a large number of countries. In this study we report the transmission of an atypical scrapie isolate through different species barriers as modeled by transgenic mice (Tg) expressing different species PRP sequence. The donor isolate was collected in 1995 in a French commercial sheep flock. inoculation into AHQ/AHQ sheep induced a disease which had all neuro-pathological and biochemical characteristics of atypical scrapie. Transmitted into Transgenic mice expressing either ovine or PrPc, the isolate retained all the described characteristics of atypical scrapie. Surprisingly the TSE agent characteristics were dramatically different v/hen passaged into Tg bovine mice. The recovered TSE agent had biological and biochemical characteristics similar to those of atypical BSE L in the same mouse model. Moreover, whereas no other TSE agent than BSE were shown to transmit into Tg porcine mice, atypical scrapie was able to develop into this model, albeit with low attack rate on first passage. Furthermore, after adaptation in the porcine mouse model this prion showed similar biological and biochemical characteristics than BSE adapted to this porcine mouse model. Altogether these data indicate. (i) the unsuspected potential abilities of atypical scrapie to cross species barriers (ii) the possible capacity of this agent to acquire new characteristics when crossing species barrier These findings raise some interrogation on the concept of TSE strain and on the origin of the diversity of the TSE agents and could have consequences on field TSE control measures. http://www.neuroprion.org/resources/pdf_docs/conferences/prion2008/abstract-book-prion2008.pdf Thursday, January 26, 2012 Facilitated Cross-Species Transmission of Prions in Extraneural Tissue Science 27 January 2012: Vol. 335 no. 6067 pp. 472-475 DOI: 10.1126/science.1215659 http://transmissiblespongiformencephalopathy.blogspot.com/2012/01/facilitated-cross-species-transmission.html Saturday, February 11, 2012 Prion cross-species transmission efficacy is tissue dependent http://transmissiblespongiformencephalopathy.blogspot.com/2012/02/prion-cross-species-transmission.html Thursday, January 26, 2012 The Risk of Prion Zoonoses Science 27 January 2012: Vol. 335 no. 6067 pp. 411-413 DOI: 10.1126/science.1218167 http://transmissiblespongiformencephalopathy.blogspot.com/2012/01/risk-of-prion-zoonoses.html 1: J Infect Dis 1980 Aug;142(2):205-8 Oral transmission of kuru, Creutzfeldt-Jakob disease, and scrapie to nonhuman primates. Gibbs CJ Jr, Amyx HL, Bacote A, Masters CL, Gajdusek DC. Kuru and Creutzfeldt-Jakob disease of humans and scrapie disease of sheep and goats were transmitted to squirrel monkeys (Saimiri sciureus) that were exposed to the infectious agents only by their nonforced consumption of known infectious tissues. The asymptomatic incubation period in the one monkey exposed to the virus of kuru was 36 months; that in the two monkeys exposed to the virus of Creutzfeldt-Jakob disease was 23 and 27 months, respectively; and that in the two monkeys exposed to the virus of scrapie was 25 and 32 months, respectively. Careful physical examination of the buccal cavities of all of the monkeys failed to reveal signs or oral lesions. One additional monkey similarly exposed to kuru has remained asymptomatic during the 39 months that it has been under observation. snip... The successful transmission of kuru, Creutzfeldt-Jakob disease, and scrapie by natural feeding to squirrel monkeys that we have reported provides further grounds for concern that scrapie-infected meat may occasionally give rise in humans to Creutzfeldt-Jakob disease. PMID: 6997404 http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_uids=6997404&dopt=Abstract 12/10/76 AGRICULTURAL RESEARCH COUNCIL REPORT OF THE ADVISORY COMMITTE ON SCRAPIE Office Note CHAIRMAN: PROFESSOR PETER WILDY snip... A The Present Position with respect to Scrapie A] The Problem Scrapie is a natural disease of sheep and goats. It is a slow and inexorably progressive degenerative disorder of the nervous system and it ia fatal. It is enzootic in the United Kingdom but not in all countries. The field problem has been reviewed by a MAFF working group (ARC 35/77). It is difficult to assess the incidence in Britain for a variety of reasons but the disease causes serious financial loss; it is estimated that it cost Swaledale breeders alone $l.7 M during the five years 1971-1975. A further inestimable loss arises from the closure of certain export markets, in particular those of the United States, to British sheep. It is clear that scrapie in sheep is important commercially and for that reason alone effective measures to control it should be devised as quickly as possible. Recently the question has again been brought up as to whether scrapie is transmissible to man. This has followed reports that the disease has been transmitted to primates. One particularly lurid speculation (Gajdusek 1977) conjectures that the agents of scrapie, kuru, Creutzfeldt-Jakob disease and transmissible encephalopathy of mink are varieties of a single "virus". The U.S. Department of Agriculture concluded that it could "no longer justify or permit scrapie-blood line and scrapie-exposed sheep and goats to be processed for human or animal food at slaughter or rendering plants" (ARC 84/77)" The problem is emphasised by the finding that some strains of scrapie produce lesions identical to the once which characterise the human dementias" Whether true or not. the hypothesis that these agents might be transmissible to man raises two considerations. First, the safety of laboratory personnel requires prompt attention. Second, action such as the "scorched meat" policy of USDA makes the solution of the acrapie problem urgent if the sheep industry is not to suffer grievously. snip... 76/10.12/4.6 http://web.archive.org/web/20010305223125/www.bseinquiry.gov.uk/files/yb/1976/10/12004001.pdf Nature. 1972 Mar 10;236(5341):73-4. Transmission of scrapie to the cynomolgus monkey (Macaca fascicularis). Gibbs CJ Jr, Gajdusek DC. Nature 236, 73 - 74 (10 March 1972); doi:10.1038/236073a0 Transmission of Scrapie to the Cynomolgus Monkey (Macaca fascicularis) C. J. GIBBS jun. & D. C. GAJDUSEK National Institute of Neurological Diseases and Stroke, National Institutes of Health, Bethesda, Maryland SCRAPIE has been transmitted to the cynomolgus, or crab-eating, monkey (Macaca fascicularis) with an incubation period of more than 5 yr from the time of intracerebral inoculation of scrapie-infected mouse brain. The animal developed a chronic central nervous system degeneration, with ataxia, tremor and myoclonus with associated severe scrapie-like pathology of intensive astroglial hypertrophy and proliferation, neuronal vacuolation and status spongiosus of grey matter. The strain of scrapie virus used was the eighth passage in Swiss mice (NIH) of a Compton strain of scrapie obtained as ninth intracerebral passage of the agent in goat brain, from Dr R. L. Chandler (ARC, Compton, Berkshire). http://www.nature.com/nature/journal/v236/n5341/abs/236073a0.html Nature. 1972 Mar 10;236(5341):73-4. Transmission of scrapie to the cynomolgus monkey (Macaca fascicularis). Gibbs CJ Jr, Gajdusek DC. Nature 236, 73 - 74 (10 March 1972); doi:10.1038/236073a0 Transmission of Scrapie to the Cynomolgus Monkey (Macaca fascicularis) C. J. GIBBS jun. & D. C. GAJDUSEK National Institute of Neurological Diseases and Stroke, National Institutes of Health, Bethesda, Maryland SCRAPIE has been transmitted to the cynomolgus, or crab-eating, monkey (Macaca fascicularis) with an incubation period of more than 5 yr from the time of intracerebral inoculation of scrapie-infected mouse brain. The animal developed a chronic central nervous system degeneration, with ataxia, tremor and myoclonus with associated severe scrapie-like pathology of intensive astroglial hypertrophy and proliferation, neuronal vacuolation and status spongiosus of grey matter. The strain of scrapie virus used was the eighth passage in Swiss mice (NIH) of a Compton strain of scrapie obtained as ninth intracerebral passage of the agent in goat brain, from Dr R. L. Chandler (ARC, Compton, Berkshire). http://www.nature.com/nature/journal/v236/n5341/abs/236073a0.html Wednesday, February 16, 2011 IN CONFIDENCE SCRAPIE TRANSMISSION TO CHIMPANZEES IN CONFIDENCE http://scrapie-usa.blogspot.com/2011/02/in-confidence-scrapie-transmission-to.html why do we not want to do TSE transmission studies on chimpanzees $ snip... 5. A positive result from a chimpanzee challenged severly would likely create alarm in some circles even if the result could not be interpreted for man. I have a view that all these agents could be transmitted provided a large enough dose by appropriate routes was given and the animals kept long enough. Until the mechanisms of the species barrier are more clearly understood it might be best to retain that hypothesis. snip... R. BRADLEY http://collections.europarchive.org/tna/20080102222950/http://www.bseinquiry.gov.uk/files/yb/1990/09/23001001.pdf Friday, February 11, 2011 Atypical/Nor98 Scrapie Infectivity in Sheep Peripheral Tissues http://nor-98.blogspot.com/2011/02/atypicalnor98-scrapie-infectivity-in.html Monday, April 25, 2011 Experimental Oral Transmission of Atypical Scrapie to Sheep Volume 17, Number 5-May 2011 http://nor-98.blogspot.com/2011/04/experimental-oral-transmission-of.html 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 Thursday, November 18, 2010 Increased susceptibility of human-PrP transgenic mice to bovine spongiform encephalopathy following passage in sheep http://bse-atypical.blogspot.com/2010/11/increased-susceptibility-of-human-prp.html Wednesday, January 19, 2011 EFSA and ECDC review scientific evidence on possible links between TSEs in animals and humans Webnachricht 19 Januar 2011 http://transmissiblespongiformencephalopathy.blogspot.com/2011/01/efsa-and-ecdc-review-scientific.html Monday, June 27, 2011 Comparison of Sheep Nor98 with Human Variably Protease-Sensitive Prionopathy and Gerstmann-Sträussler-Scheinker Disease http://prionopathy.blogspot.com/2011/06/comparison-of-sheep-nor98-with-human.html Sunday, March 11, 2012 APHIS Proposes New Bovine Spongiform Encephalopathy Import Regulations in Line with International Animal Health Standards Proposal Aims to Ensure Health of the U.S. Beef Herd, Assist in Negotiations http://transmissiblespongiformencephalopathy.blogspot.com/2012/03/aphis-proposes-new-bovine-spongiform.html Friday, March 09, 2012 Experimental H-type and L-type bovine spongiform encephalopathy in cattle: observation of two clinical syndromes and diagnostic challenges Research article http://bse-atypical.blogspot.com/2012/03/experimental-h-type-and-l-type-bovine.html see CWD map Minnesota, compare wild to captive. WHICH CAME FIRST, THE CART OR THE HORSE ??? Minnesota CAPTIVE CWD CONFIRMED 2002 FREE RANGING CWD CONFIRMED 2011 http://wwwnc.cdc.gov/eid/article/18/3/11-0685-f1.htm Colorado Captive CWD discovered 1967 Free ranging CWD discovered 1981 PLEASE STUDY THIS MAP ! SEE CWD MAP, RELATE TO DATES OF GAME FARM INFECTION, TO DATE OF INFECTION RATE IN WILD, SURROUNDING SAID INFECTED GAME FARMS. ...TSS http://wwwnc.cdc.gov/eid/article/18/3/11-0685-f1.htm *** Chronic Wasting Disease CWD CDC REPORT MARCH 2012 *** Saturday, February 18, 2012 Occurrence, Transmission, and Zoonotic Potential of Chronic Wasting Disease CDC Volume 18, Number 3—March 2012 SNIP... Long-term effects of CWD on cervid populations and ecosystems remain unclear as the disease continues to spread and prevalence increases. In captive herds, CWD might persist at high levels and lead to complete herd destruction in the absence of human culling. Epidemiologic modeling suggests the disease could have severe effects on free-ranging deer populations, depending on hunting policies and environmental persistence (8,9). CWD has been associated with large decreases in free-ranging mule deer populations in an area of high CWD prevalence (Boulder, Colorado, USA) (5). SNIP... Reasons for Caution There are several reasons for caution with respect to zoonotic and interspecies CWD transmission. First, there is strong evidence that distinct CWD strains exist (36). Prion strains are distinguished by varied incubation periods, clinical symptoms, PrPSc conformations, and CNS PrPSc depositions (3,32). Strains have been identified in other natural prion diseases, including scrapie, BSE, and CJD (3). Intraspecies and interspecies transmission of prions from CWD-positive deer and elk isolates resulted in identification of >2 strains of CWD in rodent models (36), indicating that CWD strains likely exist in cervids. However, nothing is currently known about natural distribution and prevalence of CWD strains. Currently, host range and pathogenicity vary with prion strain (28,37). Therefore, zoonotic potential of CWD may also vary with CWD strain. In addition, diversity in host (cervid) and target (e.g., human) genotypes further complicates definitive findings of zoonotic and interspecies transmission potentials of CWD. Intraspecies and interspecies passage of the CWD agent may also increase the risk for zoonotic CWD transmission. The CWD prion agent is undergoing serial passage naturally as the disease continues to emerge. In vitro and in vivo intraspecies transmission of the CWD agent yields PrPSc with an increased capacity to convert human PrPc to PrPSc (30). Interspecies prion transmission can alter CWD host range (38) and yield multiple novel prion strains (3,28). The potential for interspecies CWD transmission (by cohabitating mammals) will only increase as the disease spreads and CWD prions continue to be shed into the environment. This environmental passage itself may alter CWD prions or exert selective pressures on CWD strain mixtures by interactions with soil, which are known to vary with prion strain (25), or exposure to environmental or gut degradation. Given that prion disease in humans can be difficult to diagnose and the asymptomatic incubation period can last decades, continued research, epidemiologic surveillance, and caution in handling risky material remain prudent as CWD continues to spread and the opportunity for interspecies transmission increases. Otherwise, similar to what occurred in the United Kingdom after detection of variant CJD and its subsequent link to BSE, years of prevention could be lost if zoonotic transmission of CWD is subsequently identified, SNIP...SEE FULL TEXT ; *** Chronic Wasting Disease CWD CDC REPORT MARCH 2012 *** Saturday, February 18, 2012 Occurrence, Transmission, and Zoonotic Potential of Chronic Wasting Disease CDC Volume 18, Number 3—March 2012 http://wwwnc.cdc.gov/eid/article/18/3/11-0685_article.htm see much more here ; http://chronic-wasting-disease.blogspot.com/2012/02/occurrence-transmission-and-zoonotic.html Sunday, January 22, 2012 Chronic Wasting Disease CWD cervids interspecies transmission http://chronic-wasting-disease.blogspot.com/2012/01/chronic-wasting-disease-cwd-cervids.html Thursday, January 26, 2012 The Risk of Prion Zoonoses Science 27 January 2012: Vol. 335 no. 6067 pp. 411-413 DOI: 10.1126/science.1218167 http://transmissiblespongiformencephalopathy.blogspot.com/2012/01/risk-of-prion-zoonoses.html Thursday, January 26, 2012 Facilitated Cross-Species Transmission of Prions in Extraneural Tissue Science 27 January 2012: Vol. 335 no. 6067 pp. 472-475 DOI: 10.1126/science.1215659 http://transmissiblespongiformencephalopathy.blogspot.com/2012/01/facilitated-cross-species-transmission.html CJD9/10022 October 1994 Mr R.N. Elmhirst Chairman British Deer Farmers Association Holly Lodge Spencers Lane BerksWell Coventry CV7 7BZ Dear Mr Elmhirst, CREUTZFELDT-JAKOB DISEASE (CJD) SURVEILLANCE UNIT REPORT Thank you for your recent letter concerning the publication of the third annual report from the CJD Surveillance Unit. I am sorry that you are dissatisfied with the way in which this report was published. The Surveillance Unit is a completely independant outside body and the Department of Health is committed to publishing their reports as soon as they become available. In the circumstances it is not the practice to circulate the report for comment since the findings of the report would not be amended. In future we can ensure that the British Deer Farmers Association receives a copy of the report in advance of publication. The Chief Medical Officer has undertaken to keep the public fully informed of the results of any research in respect of CJD. This report was entirely the work of the unit and was produced completely independantly of the the Department. The statistical results reqarding the consumption of venison was put into perspective in the body of the report and was not mentioned at all in the press release. Media attention regarding this report was low key but gave a realistic presentation of the statistical findings of the Unit. This approach to publication was successful in that consumption of venison was highlighted only once by the media ie. in the News at one television proqramme. I believe that a further statement about the report, or indeed statistical links between CJD and consumption of venison, would increase, and quite possibly give damaging credence, to the whole issue. From the low key media reports of which I am aware it seems unlikely that venison consumption will suffer adversely, if at all. http://web.archive.org/web/20030511010117/http://www.bseinquiry.gov.uk/files/yb/1994/10/00003001.pdf THIRD CJD REPORT UK 1994 snip... Consumption of venison and veal was much less widespread among both cases and controls. For both of these meats, there was evidence of a trend with increasing frequency of consumption being associated with increasing risk of CJD. These associations were largely unchanged when attention was restricted to pairs with data obtained from relatives. ... http://www.cjd.ed.ac.uk/Archive%20reports/report3.pdf Saturday, March 10, 2012 Enhanced Surveillance Strategies for Detecting and Monitoring Chronic Wasting Disease in Free-Ranging Cervids Open-File Report 2012–1036 National Wildlife Health Center Open-File Report 2012–1036 http://chronic-wasting-disease.blogspot.com/2012/03/enhanced-surveillance-strategies-for.html a few things to consider please. one, CWD has already been transmitted to many cattle in the lab (86% in one study). the oral route would have a much longer incubation period, but we already know that CWD will transmit back to cervids via the oral route, very efficiently. the threat of spreading CWD via close contact, like at feeding grounds is great. every bit of science to date shows this. so to congregate deer together by unnatural means is not smart in my opinion. another fear has come to pass as well, another strain of CWD, yes a second strain. and just recently science has shown that a natural case of BSE has been transmitted to a GOAT. These findings demonstrate that when CWD is directly inoculated into the brain of cattle, 86% of inoculated cattle develop clinical signs of the disease. http://www.ars.usda.gov/research/publications/publications.htm?seq_no_115=194089 Thursday, February 09, 2012 50 GAME FARMS IN USA INFECTED WITH CHRONIC WASTING DISEASE http://chronic-wasting-disease.blogspot.com/2012/02/50-game-farms-to-date-in-usa-infected.html and when these game farms claim they are testing, and everything is o.k., think again... Saturday, February 04, 2012 Wisconsin 16 age limit on testing dead deer Game Farm CWD Testing Protocol Needs To Be Revised http://chronic-wasting-disease.blogspot.com/2012/02/wisconsin-16-age-limit-on-testing-dead.html National Wildlife Health Center Open-File Report 2012–1036 U.S. Enhanced Surveillance Strategies for Detecting and Monitoring Chronic Wasting Disease in Free-Ranging Cervids snip... In addition to locations of known CWD-positive individuals, other spatial risk factors related to CWD exposure should be considered. For example, the risk of free-ranging animals being exposed to CWD is likely greater in areas where captive cervid facilities have or had CWD-positive animals. Current evidence indicates that CWD infection rates are much higher in captive facilities than in wild populations (Keane and others, 2008), and perhaps this is driven by environmental contamination (Miller and others, 2006). This higher rate of infection in captive animals can increase the risk of disease exposure to surrounding wild populations. Furthermore, movement of infectious animals, carcasses, or other materials across the landscape, naturally or with human assistance, likely increases the risk to uninfected populations. The frequent movement of farmed elk (Cervus elaphus) and deer between production facilities, the concentration of infected animals on some facilities, and the possibility of their escape into the wild increases the risk of spreading CWD to uninfected populations of free-ranging animals. Because the infectious prions may persist in the environment for long periods, the introduction of either captive or free-ranging uninfected animals into a contaminated environment could increase their risk of infection. For example, locations from which sheep have been removed may remain contaminated with scrapie agent for more than 15 years (Georgsson and others, 2006). Saturday, March 10, 2012 Enhanced Surveillance Strategies for Detecting and Monitoring Chronic Wasting Disease in Free-Ranging Cervids Open-File Report 2012–1036 National Wildlife Health Center Open-File Report 2012–1036 http://chronic-wasting-disease.blogspot.com/2012/03/enhanced-surveillance-strategies-for.html UPDATED CORRESPONDENCE FROM AUTHORS OF THIS STUDY I.E. COLBY, PRUSINER ET AL, ABOUT MY CONCERNS OF THE DISCREPANCY BETWEEN THEIR FIGURES AND MY FIGURES OF THE STUDIES ON CWD TRANSMISSION TO CATTLE ; ----- Original Message ----- From: David Colby To: flounder9@verizon.net Cc: stanley@XXXXXXXX Sent: Tuesday, March 01, 2011 8:25 AM Subject: Re: FW: re-Prions David W. Colby1,* and Stanley B. Prusiner1,2 + Author Affiliations Dear Terry Singeltary, Thank you for your correspondence regarding the review article Stanley Prusiner and I recently wrote for Cold Spring Harbor Perspectives. Dr. Prusiner asked that I reply to your message due to his busy schedule. We agree that the transmission of CWD prions to beef livestock would be a troubling development and assessing that risk is important. In our article, we cite a peer-reviewed publication reporting confirmed cases of laboratory transmission based on stringent criteria. The less stringent criteria for transmission described in the abstract you refer to lead to the discrepancy between your numbers and ours and thus the interpretation of the transmission rate. We stand by our assessment of the literature--namely that the transmission rate of CWD to bovines appears relatively low, but we recognize that even a low transmission rate could have important implications for public health and we thank you for bringing attention to this matter. Warm Regards, David Colby -- David Colby, PhDAssistant ProfessorDepartment of Chemical EngineeringUniversity of Delaware ====================END...TSS============== SNIP...SEE FULL TEXT ; http://betaamyloidcjd.blogspot.com/2011/01/enlarging-spectrum-of-prion-like.html UPDATED DATA ON 2ND CWD STRAIN Wednesday, September 08, 2010 CWD PRION CONGRESS SEPTEMBER 8-11 2010 http://chronic-wasting-disease.blogspot.com/2010/09/cwd-prion-2010.html end layperson Terry S. Singeltary Sr. P.O. Box 42 Bacliff, Texas USA 77518 flounder9@verizon.net This particular rule has a comment period of 60 days. It may be commented on until 5 p.m. on April 17. A detailed description of the proposed rule can be found on the TAHC website under Rule Proposals at www.tahc.state.tx.us/regs/proposals.html. Comments on the TAHC's proposed regulations must be submitted in writing to Carol Pivonka, Texas Animal Health Commission, 2105 Kramer Lane, Austin, Texas 78758, by fax at 512-719-0721 or by email to comments@tahc.state.tx.us. The Commission appreciates any comments on issues addressed in this document for consideration during drafting of the rule. Founded in 1893, the Texas Animal Health Commission works to protect the health of all Texas livestock, including: cattle, swine, poultry, sheep, goats, equine animals, and exotic livestock. END...TSS Reader Comments Terry S. Singeltary Sr. — 03/18/2012 03:03:55 EXPLANATION OF PROPOSED RULE Chapter 51, Entry Requirements - Proposed Changes Regarding Sheep, Goats, Swine, & Cattle COMMENT SUBMISSION The Texas Animal Health Commission (Commission) proposes amendments to Chapter 51 entitled "Entry Requirements." The purpose of these amendments is to make the entry requirements more consistent with the current national interstate movement standards. The Commission is amending §51.3 entitled “Exceptions” to clarify and modify requirements for sheep, goats and swine. The first amendment is providing an exception for swine consigned from out-of-state directly to slaughter or from an out-of-state premise of origin to a Texas livestock market specifically approved under the Code of Federal Regulations, Title 9, §71.20. This amendment is expanding the current exception for entry permits to include an exception to both entry permits and health certificates. The Commission is also amending the entry permit exception for sheep and goats consigned from out-of-state to clarify it is for those originating from “Consistent States” which have an active scrapie surveillance and control program. SNIP...END http://www.tahc.state.tx.us/regs/proposed/2012Feb_Ch51EntryReqs.pdf >>> The Commission is also amending the entry permit exception for sheep and goats consigned from out-of-state to clarify it is for those originating from “Consistent States” which have an active scrapie surveillance and control program. >>> (56) Sheep and goats consigned from out-of-state and originating from Consistent States (having an active scrapie surveillance and control program); Greetings TAHC et al, I kindly wish to make the following submission to this comment. I urge you NOT to add scrapie or any TSE prion disease to the list of acceptable disease i.e. as being exempt, simply because of the active scrapie, active BSE, or active CWD surveillance and control program. >>> The Commission is also amending the entry permit exception for sheep and goats consigned from out-of-state to clarify it is for those originating from “Consistent States” which have an active scrapie surveillance and control program. the active scrapie surveillance and control program, WILL NOT identify and eradicate your SUB-CLINICAL scrapie cases. thus, you risk bringing additional scrapie cases into Texas, thus risk your sheep, goat, deer, elk, and cattle industry, let alone any human risk factors there from. please see ; “INDICATING THAT SUBCLINICAL SCRAPIE INFECTION IS LIKELY TO BE A COMMON OCCURRENCE” The Oral Secretion of Infectious Scrapie Prions Occurs in Preclinical Sheep with a Range of PRNP Genotypes Kevin C. Gougha, Claire A. Bakerb, Helen C. Reesb, Linda A. Terryc, John Spiropoulosc, Leigh Thornec and Ben C. Maddisonb + Author Affiliations aSchool of Veterinary Medicine and Science, The University of Nottingham, Sutton Bonington Campus, Sutton Bonington, Leicestershire, United Kingdom bADAS UK, School of Veterinary Medicine and Science, The University of Nottingham, Sutton Bonington Campus, Sutton Bonington, Leicestershire, United Kingdom cAnimal Health and Veterinary Laboratories Agency, Addlestone, Surrey, United Kingdom ABSTRACT Preclinical sheep with the highly scrapie-susceptible VRQ/VRQ PRNP genotype secrete prions from the oral cavity. In order to further understand the significance of orally available prions, buccal swabs were taken from sheep with a range of PRNP genotypes and analyzed by serial protein misfolding cyclic amplification (sPMCA). Prions were detected in buccal swabs from scrapie-exposed sheep of genotypes linked to high (VRQ/VRQ and ARQ/VRQ) and low (ARR/VRQ and AHQ/VRQ) lymphoreticular system involvement in scrapie pathogenesis. For both groups, the level of prion detection was significantly higher than that for scrapie-resistant ARR/ARR sheep which were kept in the same farm environment and acted as sentinel controls for prions derived from the environment which might contaminate the oral cavity. In addition, sheep with no exposure to the scrapie agent did not contain any measurable prions within the oral cavity. Furthermore, prions were detected in sheep over a wide age range representing various stages of preclinical disease. These data demonstrate that orally available scrapie prions may be a common feature in sheep incubating scrapie, regardless of the PRNP genotype and any associated high-level accumulation of PrPSc within lymphoreticular tissues. PrPSc was present in buccal swabs from a large proportion of sheep with PRNP genotypes associated with relatively low disease penetrance, indicating that subclinical scrapie infection is likely to be a common occurrence. The significance of positive sPMCA reactions was confirmed by the transmission of infectivity in buccal swab extracts to Tg338 mice, illustrating the likely importance of orally available prions in the horizontal transmission of scrapie. FOOTNOTES Received 1 July 2011. Accepted 11 October 2011. Address correspondence to Ben Charles Maddison, ben.maddison@adas.co.uk. Published ahead of print 19 October 2011 Copyright © 2012, American Society for Microbiology. All Rights Reserved. http://jvi.asm.org/content/86/1/566.abstract In Confidence - Perceptions of unconventional slow virus diseases of animals in the USA - APRIL-MAY 1989 - G A H Wells http://web.archive.org/web/20060307063531/http://www.bseinquiry.gov.uk/files/mb/m11b/tab01.pdf *** Spraker suggested an interesting explanation for the occurrence of CWD. The deer pens at the Foot Hills Campus were built some 30-40 years ago by a Dr. Bob Davis. At or abut that time, allegedly, some scrapie work was conducted at this site. When deer were introduced to the pens they occupied ground that had previously been occupied by sheep. (PLEASE NOTE SOME OF THESE OLD UK GOVERNMENT FILE URLS ARE SLOW TO OPEN, AND SOMETIMES YOU MAY HAVE TO CLICK ON MULTIPLE TIMES, PLEASE BE PATIENT, ANY PROBLEMS PLEASE WRITE ME PRIVATELY, AND I WILL TRY AND FIX OR SEND YOU OLD PDF FILE...TSS) http://collections.europarchive.org/tna/20080102193705/http://www.bseinquiry.gov.uk/files/mb/m11b/tab01.pdf ONE HUNDRED AND FOURTEENTH ANNUAL MEETING of the United States Animal Health Association White-tailed Deer are Susceptible to Scrapie by Natural Route of Infection Jodi D. Smith, Justin J. Greenlee, and Robert A. Kunkle; Virus and Prion Research Unit, National Animal Disease Center, USDA-ARS Interspecies transmission studies afford the opportunity to better understand the potential host range and origins of prion diseases. Previous experiments demonstrated that white-tailed deer are susceptible to sheepderived scrapie by intracranial inoculation. The purpose of this study was to determine susceptibility of white-tailed deer to scrapie after a natural route of exposure. Deer (n=5) were inoculated by concurrent oral (30 ml) and intranasal (1 ml) instillation of a 10% (wt/vol) brain homogenate derived from a sheep clinically affected with scrapie. Non-inoculated deer were maintained as negative controls. All deer were observed daily for clinical signs. Deer were euthanized and necropsied when neurologic disease was evident, and tissues were examined for abnormal prion protein (PrPSc) by immunohistochemistry (IHC) and western blot (WB). One animal was euthanized 15 months post-inoculation (MPI) due to an injury. At that time, examination of obex and lymphoid tissues by IHC was positive, but WB of obex and colliculus were negative. Remaining deer developed clinical signs of wasting and mental depression and were necropsied from 28 to 33 MPI. Tissues from these deer were positive for scrapie by IHC and WB. Tissues with PrPSc immunoreactivity included brain, tonsil, retropharyngeal and mesenteric lymph nodes, hemal node, Peyer’s patches, and spleen. This work demonstrates for the first time that white-tailed deer are susceptible to sheep scrapie by potential natural routes of inoculation. In-depth analysis of tissues will be done to determine similarities between scrapie in deer after intracranial and oral/intranasal inoculation and chronic wasting disease resulting from similar routes of inoculation. http://www.usaha.org/Portals/6/Proceedings/USAHAProceedings-2010-114th.pdf PPo3-22: Detection of Environmentally Associated PrPSc on a Farm with Endemic Scrapie Ben C. Maddison,1 Claire A. Baker,1 Helen C. Rees,1 Linda A. Terry,2 Leigh Thorne,2 Susan J. Belworthy2 and Kevin C. Gough3 1ADAS-UK LTD; Department of Biology; University of Leicester; Leicester, UK; 2Veterinary Laboratories Agency; Surry, KT UK; 3Department of Veterinary Medicine and Science; University of Nottingham; Sutton Bonington, Loughborough UK Key words: scrapie, evironmental persistence, sPMCA Ovine scrapie shows considerable horizontal transmission, yet the routes of transmission and specifically the role of fomites in transmission remain poorly defined. Here we present biochemical data demonstrating that on a scrapie-affected sheep farm, scrapie prion contamination is widespread. It was anticipated at the outset that if prions contaminate the environment that they would be there at extremely low levels, as such the most sensitive method available for the detection of PrPSc, serial Protein Misfolding Cyclic Amplification (sPMCA), was used in this study. We investigated the distribution of environmental scrapie prions by applying ovine sPMCA to samples taken from a range of surfaces that were accessible to animals and could be collected by use of a wetted foam swab. Prion was amplified by sPMCA from a number of these environmental swab samples including those taken from metal, plastic and wooden surfaces, both in the indoor and outdoor environment. At the time of sampling there had been no sheep contact with these areas for at least 20 days prior to sampling indicating that prions persist for at least this duration in the environment. These data implicate inanimate objects as environmental reservoirs of prion infectivity which are likely to contribute to disease transmission. http://www.prion2010.org/bilder/prion_2010_program_latest_w_posters_4_.pdf?139&PHPSESSID=a30a38202cfec579000b77af81be3099 Wednesday, September 08, 2010 CWD PRION CONGRESS SEPTEMBER 8-11 2010 http://chronic-wasting-disease.blogspot.com/2010/09/cwd-prion-2010.html P35 ADAPTATION OF CHRONIC WASTING DISEASE (CWD) INTO HAMSTERS, EVIDENCE OF A WISCONSIN STRAIN OF CWD Chad Johnson1, Judd Aiken2,3,4 and Debbie McKenzie4,5 1 Department of Comparative Biosciences, University of Wisconsin, Madison WI, USA 53706 2 Department of Agriculture, Food and Nutritional Sciences, 3 Alberta Veterinary Research Institute, 4.Center for Prions and Protein Folding Diseases, 5 Department of Biological Sciences, University of Alberta, Edmonton AB, Canada T6G 2P5 The identification and characterization of prion strains is increasingly important for the diagnosis and biological definition of these infectious pathogens. Although well-established in scrapie and, more recently, in BSE, comparatively little is known about the possibility of prion strains in chronic wasting disease (CWD), a disease affecting free ranging and captive cervids, primarily in North America. We have identified prion protein variants in the white-tailed deer population and demonstrated that Prnp genotype affects the susceptibility/disease progression of white-tailed deer to CWD agent. The existence of cervid prion protein variants raises the likelihood of distinct CWD strains. Small rodent models are a useful means of identifying prion strains. We intracerebrally inoculated hamsters with brain homogenates and phosphotungstate concentrated preparations from CWD positive hunter-harvested (Wisconsin CWD endemic area) and experimentally infected deer of known Prnp genotypes. These transmission studies resulted in clinical presentation in primary passage of concentrated CWD prions. Subclinical infection was established with the other primary passages based on the detection of PrPCWD in the brains of hamsters and the successful disease transmission upon second passage. Second and third passage data, when compared to transmission studies using different CWD inocula (Raymond et al., 2007) indicate that the CWD agent present in the Wisconsin white-tailed deer population is different than the strain(s) present in elk, mule-deer and white-tailed deer from the western United States endemic region http://www.istitutoveneto.it/prion_09/Abstracts_09.pdf Thursday, February 17, 2011 Environmental Sources of Scrapie Prions http://scrapie-usa.blogspot.com/2011/02/environmental-sources-of-scrapie-prions.html Increased Atypical Scrapie Detections Press reports indicate that increased surveillance is catching what otherwise would have been unreported findings of atypical scrapie in sheep. In 2009, five new cases have been reported in Quebec, Ontario, Alberta, and Saskatchewan. With the exception of Quebec, all cases have been diagnosed as being the atypical form found in older animals. Canada encourages producers to join its voluntary surveillance program in order to gain scrapie-free status. The World Animal Health will not classify Canada as scrapie-free until no new cases are reported for seven years. The Canadian Sheep Federation is calling on the government to fund a wider surveillance program in order to establish the level of prevalence prior to setting an eradication date. Besides long-term testing, industry is calling for a compensation program for farmers who report unusual deaths in their flocks. http://gain.fas.usda.gov/Recent%20GAIN%20Publications/This%20Week%20in%20Canadian%20Agriculture%20%20%20%20%20Issue%2028_Ottawa_Canada_11-6-2009.pdf J Vet Diagn Invest 21:454-463 (2009) Nor98 scrapie identified in the United States Christie M. Loiacono,' Bruce V. Thomsen, S. Mark Hall, Matti Kiupe!, Diane Sutton, Katherine O'Rourke, Bradd Barr, Lucy Anthenill, Deiwyn Keane Abstract. A distinct strain of scrapic identified in sheep of Norway in 1998 has since been identified in numerous countries throughout Europe. The disease is known as Nor98 or Not-98-like scrapic. among other names. Distinctions between classic scrapie and Nor98 scrapie are made based on histopathologv and immunodiagnostic results. There are also differences in the epidemiology, typical signalment, and likelihood of clinical signs being observed. In addition, sheep that have genotypes associated with resistance to classic scrapie are not spared from Nor98 disease. The various differences between classic and Nor98 scrapie have been consistently reported in the vast majority of cases described across Europe. The current study describes in detail the patholo gic changes and diagnostic results of the first 6 cases of' Nor98 scrapic disease diagnosed in sheep of the United States. Key words: Hisiopathology: Nor98: PrP imniunolabeling; scrapie: sheep. snip... Results Case I The first case identified as consistent with Nor98 scrapie had nonclassic PrP distribution in brain tissue, no PrPSC in lymph tissue, and nonclassic migration of protein bands on a Western blot test. The animal was an aged, mottled-faced ewe that was traced back to a commercial flock in Wyoming. ... Case 2 The second case was a clinically normal 8-year-old Suffolk ewe that had been in a quarantined flock for 5 years at a USDA facility in Iowa. Case 3 A 16-year-old, white-faced, cross-bred wether was born to a black-faced ewe. He lived his entire life as a pet on a farm in California. Case 4 The fourth case of Nor98 scrapie was identified in an approximately 8-year-old Dorset ewe that was born into a flock of approximately 20 ewes in Indiana. Case 5 The fifth case was a clinically normal, approximately 3-year-old, white-faced, cross-bred ewe from an approximately 400 head commercial flock in Minnesota. Case 6 The sixth case of Nor98 scrapie was identified in a 4-year-old, white-faced ewe that was purchased and added to a commercial flock in Pennsylvania snip... see full text ; http://ddr.nal.usda.gov/bitstream/10113/33943/1/IND44241920.pdf Monday, October 10, 2011 EFSA Journal 2011 The European Response to BSE: A Success Story snip... EFSA and the European Centre for Disease Prevention and Control (ECDC) recently delivered a scientific opinion on any possible epidemiological or molecular association between TSEs in animals and humans (EFSA Panel on Biological Hazards (BIOHAZ) and ECDC, 2011). This opinion confirmed Classical BSE prions as the only TSE agents demonstrated to be zoonotic so far but the possibility that a small proportion of human cases so far classified as "sporadic" CJD are of zoonotic origin could not be excluded. Moreover, transmission experiments to non-human primates suggest that some TSE agents in addition to Classical BSE prions in cattle (namely L-type Atypical BSE, Classical BSE in sheep, transmissible mink encephalopathy (TME) and chronic wasting disease (CWD) agents) might have zoonotic potential. snip... http://www.efsa.europa.eu/en/efsajournal/pub/e991.htm?emt=1 http://www.efsa.europa.eu/en/efsajournal/doc/e991.pdf see follow-up here about North America BSE Mad Cow TSE prion risk factors, and the ever emerging strains of Transmissible Spongiform Encephalopathy in many species here in the USA, including humans ; http://transmissiblespongiformencephalopathy.blogspot.com/2011/10/efsa-journal-2011-european-response-to.html 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 Friday, February 11, 2011 Atypical/Nor98 Scrapie Infectivity in Sheep Peripheral Tissues http://nor-98.blogspot.com/2011/02/atypicalnor98-scrapie-infectivity-in.html Sunday, December 12, 2010 EFSA reviews BSE/TSE infectivity in small ruminant tissues News Story 2 December 2010 http://transmissiblespongiformencephalopathy.blogspot.com/2010/12/efsa-reviews-bsetse-infectivity-in.html Wednesday, January 18, 2012 Selection of Distinct Strain Phenotypes in Mice Infected by Ovine Natural Scrapie Isolates Similar to CH1641 Experimental Scrapie Journal of Neuropathology & Experimental Neurology: February 2012 - Volume 71 - Issue 2 - p 140–147 http://transmissiblespongiformencephalopathy.blogspot.com/2012/01/selection-of-distinct-strain-phenotypes.html Thursday, July 14, 2011 Histopathological Studies of "CH1641-Like" Scrapie Sources Versus Classical Scrapie and BSE Transmitted to Ovine Transgenic Mice (TgOvPrP4) http://transmissiblespongiformencephalopathy.blogspot.com/2011/07/histopathological-studies-of-ch1641.html Wednesday, January 18, 2012 BSE IN GOATS CAN BE MISTAKEN FOR SCRAPIE February 1, 2012 http://transmissiblespongiformencephalopathy.blogspot.com/2012/01/bse-in-goats-can-be-mistaken-for.html Thursday, December 23, 2010 Molecular Typing of Protease-Resistant Prion Protein in Transmissible Spongiform Encephalopathies of Small Ruminants, France, 2002-2009 Volume 17, Number 1 January 2011 http://transmissiblespongiformencephalopathy.blogspot.com/2010/12/molecular-typing-of-protease-resistant.html Thursday, November 18, 2010 Increased susceptibility of human-PrP transgenic mice to bovine spongiform encephalopathy following passage in sheep http://bse-atypical.blogspot.com/2010/11/increased-susceptibility-of-human-prp.html hmmm, no I ponder why some of these sporadic CJD cases, are now being linked to a genetic TSE, that has NO link to the family ? hmmm, could it be these atypical TSE in animals, that are linked to the human TSE in the USA, and also, these animals have been fed back and forth to each other ? hmmm, why no link there $$$ P03.141 Aspects of the Cerebellar Neuropathology in Nor98 Gavier-Widén, D1; Benestad, SL2; Ottander, L1; Westergren, E1 1National Veterinary Insitute, Sweden; 2National Veterinary Institute, Norway Nor98 is a prion disease of old sheep and goats. This atypical form of scrapie was first described in Norway in 1998. Several features of Nor98 were shown to be different from classical scrapie including the distribution of disease associated prion protein (PrPd) accumulation in the brain. The cerebellum is generally the most affected brain area in Nor98. The study here presented aimed at adding information on the neuropathology in the cerebellum of Nor98 naturally affected sheep of various genotypes in Sweden and Norway. A panel of histochemical and immunohistochemical (IHC) stainings such as IHC for PrPd, synaptophysin, glial fibrillary acidic protein, amyloid, and cell markers for phagocytic cells were conducted. The type of histological lesions and tissue reactions were evaluated. The types of PrPd deposition were characterized. The cerebellar cortex was regularly affected, even though there was a variation in the severity of the lesions from case to case. Neuropil vacuolation was more marked in the molecular layer, but affected also the granular cell layer. There was a loss of granule cells. Punctate deposition of PrPd was characteristic. It was morphologically and in distribution identical with that of synaptophysin, suggesting that PrPd accumulates in the synaptic structures. PrPd was also observed in the granule cell layer and in the white matter. The pathology features of Nor98 in the cerebellum of the affected sheep showed similarities with those of sporadic Creutzfeldt-Jakob disease in humans. ***The pathology features of Nor98 in the cerebellum of the affected sheep showed similarities with those of sporadic Creutzfeldt-Jakob disease in humans. http://www.prion2007.com/pdf/Prion%20Book%20of%20Abstracts.pdf PR-26 NOR98 SHOWS MOLECULAR FEATURES REMINISCENT OF GSS R. Nonno1, E. Esposito1, G. Vaccari1, E. Bandino2, M. Conte1, B. Chiappini1, S. Marcon1, M. Di Bari1, S.L. Benestad3, U. Agrimi1 1 Istituto Superiore di Sanità, Department of Food Safety and Veterinary Public Health, Rome, Italy (romolo.nonno@iss.it); 2 Istituto Zooprofilattico della Sardegna, Sassari, Italy; 3 National Veterinary Institute, Department of Pathology, Oslo, Norway Molecular variants of PrPSc are being increasingly investigated in sheep scrapie and are generally referred to as "atypical" scrapie, as opposed to "classical scrapie". Among the atypical group, Nor98 seems to be the best identified. We studied the molecular properties of Italian and Norwegian Nor98 samples by WB analysis of brain homogenates, either untreated, digested with different concentrations of proteinase K, or subjected to enzymatic deglycosylation. The identity of PrP fragments was inferred by means of antibodies spanning the full PrP sequence. We found that undigested brain homogenates contain a Nor98-specific PrP fragment migrating at 11 kDa (PrP11), truncated at both the C-terminus and the N-terminus, and not N-glycosylated. After mild PK digestion, Nor98 displayed full-length PrP (FL-PrP) and N-glycosylated C-terminal fragments (CTF), along with increased levels of PrP11. Proteinase K digestion curves (0,006-6,4 mg/ml) showed that FL-PrP and CTF are mainly digested above 0,01 mg/ml, while PrP11 is not entirely digested even at the highest concentrations, similarly to PrP27-30 associated with classical scrapie. Above 0,2 mg/ml PK, most Nor98 samples showed only PrP11 and a fragment of 17 kDa with the same properties of PrP11, that was tentatively identified as a dimer of PrP11. Detergent solubility studies showed that PrP11 is insoluble in 2% sodium laurylsorcosine and is mainly produced from detergentsoluble, full-length PrPSc. Furthermore, among Italian scrapie isolates, we found that a sample with molecular and pathological properties consistent with Nor98 showed plaque-like deposits of PrPSc in the thalamus when the brain was analysed by PrPSc immunohistochemistry. Taken together, our results show that the distinctive pathological feature of Nor98 is a PrP fragment spanning amino acids ~ 90-155. This fragment is produced by successive N-terminal and C-terminal cleavages from a full-length and largely detergent-soluble PrPSc, is produced in vivo and is extremely resistant to PK digestion. *** Intriguingly, these conclusions suggest that some pathological features of Nor98 are reminiscent of Gerstmann-Sträussler-Scheinker disease. 119 http://www.neuroprion.com/pdf_docs/conferences/prion2006/abstract_book.pdf A newly identified type of scrapie agent can naturally infect sheep with resistant PrP genotypes Annick Le Dur*,?, Vincent Béringue*,?, Olivier Andréoletti?, Fabienne Reine*, Thanh Lan Laï*, Thierry Baron§, Bjørn Bratberg¶, Jean-Luc Vilotte?, Pierre Sarradin**, Sylvie L. Benestad¶, and Hubert Laude*,? +Author Affiliations *Virologie Immunologie Moléculaires and ?Génétique Biochimique et Cytogénétique, Institut National de la Recherche Agronomique, 78350 Jouy-en-Josas, France; ?Unité Mixte de Recherche, Institut National de la Recherche Agronomique-Ecole Nationale Vétérinaire de Toulouse, Interactions Hôte Agent Pathogène, 31066 Toulouse, France; §Agence Française de Sécurité Sanitaire des Aliments, Unité Agents Transmissibles Non Conventionnels, 69364 Lyon, France; **Pathologie Infectieuse et Immunologie, Institut National de la Recherche Agronomique, 37380 Nouzilly, France; and ¶Department of Pathology, National Veterinary Institute, 0033 Oslo, Norway ***Edited by Stanley B. Prusiner, University of California, San Francisco, CA (received for review March 21, 2005) Abstract Scrapie in small ruminants belongs to transmissible spongiform encephalopathies (TSEs), or prion diseases, a family of fatal neurodegenerative disorders that affect humans and animals and can transmit within and between species by ingestion or inoculation. Conversion of the host-encoded prion protein (PrP), normal cellular PrP (PrPc), into a misfolded form, abnormal PrP (PrPSc), plays a key role in TSE transmission and pathogenesis. The intensified surveillance of scrapie in the European Union, together with the improvement of PrPSc detection techniques, has led to the discovery of a growing number of so-called atypical scrapie cases. These include clinical Nor98 cases first identified in Norwegian sheep on the basis of unusual pathological and PrPSc molecular features and "cases" that produced discordant responses in the rapid tests currently applied to the large-scale random screening of slaughtered or fallen animals. Worryingly, a substantial proportion of such cases involved sheep with PrP genotypes known until now to confer natural resistance to conventional scrapie. Here we report that both Nor98 and discordant cases, including three sheep homozygous for the resistant PrPARR allele (A136R154R171), efficiently transmitted the disease to transgenic mice expressing ovine PrP, and that they shared unique biological and biochemical features upon propagation in mice. *** These observations support the view that a truly infectious TSE agent, unrecognized until recently, infects sheep and goat flocks and may have important implications in terms of scrapie control and public health. http://www.pnas.org/content/102/44/16031.abstract Monday, December 1, 2008 When Atypical Scrapie cross species barriers Authors Andreoletti O., Herva M. H., Cassard H., Espinosa J. C., Lacroux C., Simon S., Padilla D., Benestad S. L., Lantier F., Schelcher F., Grassi J., Torres, J. M., UMR INRA ENVT 1225, Ecole Nationale Veterinaire de Toulouse.France; ICISA-INlA, Madrid, Spain; CEA, IBiTec-5, DSV, CEA/Saclay, Gif sur Yvette cedex, France; National Veterinary Institute, Postboks 750 Sentrum, 0106 Oslo, Norway, INRA IASP, Centre INRA de Tours, 3738O Nouzilly, France. Content Atypical scrapie is a TSE occurring in small ruminants and harbouring peculiar clinical, epidemiological and biochemical properties. Currently this form of disease is identified in a large number of countries. In this study we report the transmission of an atypical scrapie isolate through different species barriers as modeled by transgenic mice (Tg) expressing different species PRP sequence. The donor isolate was collected in 1995 in a French commercial sheep flock. inoculation into AHQ/AHQ sheep induced a disease which had all neuro-pathological and biochemical characteristics of atypical scrapie. Transmitted into Transgenic mice expressing either ovine or PrPc, the isolate retained all the described characteristics of atypical scrapie. Surprisingly the TSE agent characteristics were dramatically different v/hen passaged into Tg bovine mice. The recovered TSE agent had biological and biochemical characteristics similar to those of atypical BSE L in the same mouse model. Moreover, whereas no other TSE agent than BSE were shown to transmit into Tg porcine mice, atypical scrapie was able to develop into this model, albeit with low attack rate on first passage. Furthermore, after adaptation in the porcine mouse model this prion showed similar biological and biochemical characteristics than BSE adapted to this porcine mouse model. Altogether these data indicate. (i) the unsuspected potential abilities of atypical scrapie to cross species barriers (ii) the possible capacity of this agent to acquire new characteristics when crossing species barrier These findings raise some interrogation on the concept of TSE strain and on the origin of the diversity of the TSE agents and could have consequences on field TS Add Your Comment To post a comment on this story, enter your screen name and email address then click "Add Comment." Your email address will not be displayed. 110 Recommend | 2 Comments

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