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From: TSS ()
IF we all believe the BSe that the USDA is trying to put out now about atypical BSE in USA cattle just arising spontaneously, IF USA scrapie transmitted to USA cattle long ago in experiments in a lab in Mission Texas did not produce UK BSE, THERE are over 20 strains of scrapie, plus the atypical in sheep, and these strains are increasing in numbers. SCRAPIE, CWD, AND TSE IN CATTLE i.e. ANIMAL TSE RAMPANT IN USA FOR DECADES, and amplified via rendering and NO test tube TSE by either Prusiner or Soto, to date, have ever produced a TSE identical to the sporadic CJD. IN fact, IF you feed BSE tainted materials to cattle and primate, you have BSE and nvCJD. USA is in a very unique situation. there are more documented TSE in different species than any other country, IF, the spontaneous TSE was true, then this would be Prusiner and everyone else that is trying to cash in on this agent with How many more will become exposed and have to die $ Medical Sciences Cristina Casalone *, Gianluigi Zanusso , Pierluigi Acutis *, Sergio Ferrari , Lorenzo Capucci , Fabrizio Tagliavini ¶, Salvatore Monaco ||, and Maria Caramelli * Transmissible spongiform encephalopathies (TSEs), or prion diseases, are mammalian neurodegenerative disorders characterized by a posttranslational conversion and brain accumulation of an insoluble, protease-resistant isoform (PrPSc) of the host-encoded cellular prion protein (PrPC). Human and animal TSE agents exist as different phenotypes that can be biochemically differentiated on the basis of the molecular mass of the protease-resistant PrPSc fragments and the degree of glycosylation. Epidemiological, molecular, and transmission studies strongly suggest that the single strain of agent responsible for bovine spongiform encephalopathy (BSE) has infected humans, causing variant Creutzfeldt-Jakob disease. The unprecedented biological properties of the BSE agent, which circumvents the so-called "species barrier" between cattle and humans and adapts to different mammalian species, has raised considerable concern for human health. To date, it is unknown whether more than one strain might be responsible for cattle TSE or whether the BSE agent undergoes phenotypic variation after natural transmission. Here we provide evidence of a second cattle TSE. The disorder was pathologically characterized by the presence of PrP-immunopositive amyloid plaques, as opposed to the lack of amyloid deposition in typical BSE cases, and by a different pattern of regional distribution and topology of brain PrPSc accumulation. In addition, Western blot analysis showed a PrPSc type with predominance of the low molecular mass glycoform and a protease-resistant fragment of lower molecular mass than BSE-PrPSc. Strikingly, the molecular signature of this previously undescribed bovine PrPSc was similar to that encountered in a distinct subtype of sporadic Creutzfeldt-Jakob disease. C.C. and G.Z. contributed equally to this work. ||To whom correspondence should be addressed. E-mail: salvatore.monaco@mail.univr.it. 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. PMID: 6997404 snip... snip... WE interviewed officials at five laboratories that test for rabies. Those officials CONFIRMED THEY ARE NOT REQUIRED TO SUBMIT RABIES-NEGATIVE SAMPLES TO APHIS FOR BSE TESTING. A South Dakota laboratory official said they were not aware they could submit rabies-negative samples to APHIS for BSE testing. A laboratory official in another State said all rabies-negative cases were not submitted to APHIS because BSE was ''NOT ON THEIR RADAR SCREEN." Officials from New York, Wisconsin, TEXAS, and Iowa advised they would NOT submit samples from animals they consider too young. Four of the five States contacted defined this age as 24 months; Wisconsin defined it as 30 months. TEXAS officials also advised that they do not always have sufficient tissue remaining to submit a BSE sample. ... snip... Bovine Spongiform Encephalopathy (BSE) is a prion Compelling evidence indicates that BSE can be Chronic Wasting Disease (CWD) is a prion disease of elk p.s. please note the 47 PENDING CASES to Sept. 2005 p.s. please note the 2005 Prion D. total 120(8) p.s. please note sporadic CJD 2002(1) 1=3 TYPE UNKNOWN??? p.s. please note 2004 prion disease (6) 6=7 TYPE snip... http://www.bseinquiry.gov.uk/files/yb/1991/01/04004001.pdf Research Project: Study of Atypical Bse Location: Virus and Prion Diseases of Livestock Project Number: 3625-32000-073-07 Start Date: Sep 15, 2004 Objective: Approach: full text ; http://www.bseinquiry.gov.uk/files/mb/m11b/tab01.pdf http://www.bseinquiry.gov.uk/report/volume2/chaptea3.htm#820543 The findings of the initial transmission, though not of the clinical or The results were not published at this point, since the attempted 3.58 There are several possible reasons why the experiment was not performed Chair: Dr. Jim Logan, Cheyenne, WY Vice Chair: Dr. Joe D. Ross, Sonora, TX Dr. Deborah L. Brennan, MS; Dr. Beth Carlson, ND; Dr. John R. Clifford, DC; Dr. Thomas F. Conner, OH; Dr. Walter E. Cook, WY; Dr. Wayne E. Cunningham, CO; Dr. Jerry W. Diemer, TX; Dr. Anita J. Edmondson, CA; Dr. Dee Ellis, TX; Dr. Lisa A. Ferguson, MD; Dr. Keith R. Forbes, NY; Dr. R. David Glauer, OH; Dr. James R. Grady, CO; Dr. William L. Hartmann, MN; Dr. Carolyn Inch, CAN; Dr. Susan J. Keller, ND; Dr. Allen M. Knowles, TN; Dr. Thomas F. Linfield, MT; Dr. Michael R. Marshall, UT; Dr. Cheryl A. Miller, In; Dr. Brian V. Noland, CO; Dr. Charles Palmer, CA; Dr. Kristine R. Petrini, MN; Mr. Stan Potratz, IA; Mr. Paul E. Rodgers, CO; Dr. Joan D. Rowe, CA; Dr. Pamela L. Smith, IA; Dr. Diane L. Sutton, MD; Dr. Lynn Anne Tesar, SD; Dr. Delwin D. Wilmot, NE; Dr. Nora E. Wineland, CO; Dr. Cindy B. Wolf, MN. The Committee met on November 9, 2005, from 8:00am until 11:55am, Hershey Lodge and Convention Center, Hershey, Pennsylvania. The meeting was called to order by Dr. Jim Logan, chair, with vice chairman Dr. Joe D. Ross attending. There were 74 people in attendance. The Scrapie Program Update was provided by Dr. Diane Sutton, National Scrapie Program Coordinator, United States Department of Agriculture (USDA), Animal and Plant Health Inspection Services (APHIS), Veterinary Services (VS). The complete text of the Status Report is included in these Proceedings. Dr. Patricia Meinhardt, USDA-APHIS-VS-National Veterinary Services Laboratory (NVSL) gave the Update on Genotyping Labs and Discrepancies in Results. NVSL conducts investigations into discrepancies on genotype testing results associated with the Scrapie Eradication Program. It is the policy of the Program to conduct a second genotype test at a second laboratory on certain individual animals. Occasionally, there are discrepancies in those results. The NVSL conducts follow-up on these situations through additional testing on additional samples from the field and archive samples from the testing laboratories. For the period of time from January 1, 2005, until October 15, 2005, there were 23 instances of discrepancies in results from 35 flocks. Of those 23 instances, 14 were caused by laboratory error (paperwork or sample mix-up), 3 results from field error, 5 were not completely resolved, and 1 originated from the use of a non-approved laboratory for the first test. As a result of inconsistencies, one laboratory’s certification was revoked by APHIS-VS. snip... Infected and Source Flocks As of September 30, 2005, there were 105 scrapie infected and source flocks. There were a total of 165** new infected and source flocks reported for FY 2005. The total infected and source flocks that have been released in FY 2005 was 128. The ratio of infected and source flocks cleaned up or placed on clean up plans vs. new infected and source flocks discovered in FY 2005 was 1.03 : 1*. In addition 622 scrapie cases were confirmed and reported by the National Veterinary Services Laboratories (NVSL) in FY 2005, of which 130 were RSSS cases. Fifteen cases of scrapie in goats have been reported since 1990. The last goat case was reported in May 2005. Approximately 5,626 animals were indemnified comprised of 49% non-registered sheep, 45% registered sheep, 1.4% non-registered goats and 4.6% registered goats. Regulatory Scrapie Slaughter Surveillance (RSSS) RSSS was designed to utilize the findings of the Center for Epidemiology and Animal Health (CEAH) Scrapie: Ovine Slaughter Surveillance (SOSS) study. The results of SOSS can be found at http://www.aphis.usda.gov/vs/ceah/cahm/Sheep/sheep.htm . RSSS started April 1, 2003. It is a targeted slaughter surveillance program which is designed to identify infected flocks for clean-up. During FY 2005 collections increased by 32% overall and by 90% for black and mottled faced sheep improving overall program effectiveness and efficiency as demonstrated by the 26% decrease in percent positive black faced sheep compared to FY 2004. Samples have been collected from 62,864 sheep since April 1, 2003, of which results have been reported for 59,105 of which 209 were confirmed positive. During FY 2005, 33,137 samples were collected from 81 plants. There have been 130 NVSL confirmed positive cases (30 collected in FY 2004 and confirmed in FY 2005 and 100 collected and confirmed in FY 2005) in FY 2005. Face colors of these positives were 114 black, 14 mottled, 1 white and 1 unknown. The percent positive by face color is shown in the chart below. Scrapie Testing In FY 2005, 35,845 animals have been tested for scrapie: 30,192 RSSS; 4,742 regulatory field cases; 772 regulatory third eyelid biopsies; 10 third eyelid validations; and 129 necropsy validations (chart 9). Animal ID As of October 04, 2005, 103,580 sheep and goat premises have been assigned identification numbers in the Scrapie National Generic Database. Official eartags have been issued to 73,807 of these premises. *This number based on an adjusted 12 month interval to accommodate the 60 day period for setting up flock plans. Date: April 30, 2006 at 4:49 pm PST Proc. Natl. Acad. Sci. USA, 10.1073/pnas.0502296102 A newly identified type of scrapie agent can naturally infect sheep with resistant PrP genotypes ( sheep prion | transgenic mice ) 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 * 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. Author contributions: H.L. designed research; A.L.D., V.B., O.A., F.R., T.L.L., J.-L.V., and H.L. performed research; T.B., B.B., P.S., and S.L.B. contributed new reagents/analytic tools; V.B., O.A., and H.L. analyzed data; and H.L. wrote the paper. A.L.D. and V.B. contributed equally to this work. To whom correspondence should be addressed. Hubert Laude, E-mail: laude@jouy.inra.fr www.pnas.org/cgi/doi/10.1073/pnas.0502296102 snip... A The Present Position with respect to Scrapie Scrapie is a natural disease of sheep and goats. It is a slow The field problem has been reviewed by a MAFF working group It is clear that scrapie in sheep is important commercially and Recently the question has again been brought up as to whether Whether true or not. the hypothesis that these agents might be snip... 76/10.12/4.6 http://www.bseinquiry.gov.uk/files/yb/1976/10/12004001.pdf Most doctors believe that sCJD is caused by a prion protein deforming by Now scientists in France have stumbled across new evidence that adds weight The complete article is 889 words long. full text; http://www.newscientist.com/article.ns?id=mg16922840.300 Edited by D. Carleton Gajdusek, Centre National de la Recherche There is substantial scientific evidence to support the notion that bovine http://www.aphis.usda.gov/vs/nahps/cwd/ USDA CWD MAP (slow to update) http://www.aphis.usda.gov/vs/nahps/cwd/cwd-distribution.html WYOMING GAME AND FISH DEPARTMENT CHRONIC WASTING DISEASE MANAGEMENT PLAN February 17, 2006 1Department of Microbiology, Immunology and Molecular Genetics, 2Sanders *These authors contributed equally to this work. †Present address: Department of Infectology, Scripps Research Institute, ‡Present address: Institute of Neuropathology, University of Zurich, §To whom correspondence should be addressed: E-mail: gtell2@uky.edu Prions are transmissible proteinaceous agents of mammals that cause fatal To test whether skeletal muscle of diseased cervids contained prion Our results show that skeletal muscle as well as CNS tissue of deer with CWD While the risk of exposure to CWD infectivity following consumption of References and Notes 1. P. J. Bosque et al., Proc. Natl. Acad. Sci. U.S.A. 99, 3812 (2002). 2. S. R. Browning et al., J. Virol. 78, 13345 (2004). 3. A. Buschmann, M. H. Groschup, J. Infect. Dis. 192, 934 (2005). 4. O. Andreoletti et al., Nat. Med. 10, 591 (2004). 5. T. R. Spraker et al., Vet. Pathol. 39, 110 (2002). 6. A. N. Hamir, J. M. Miller, R. C. Cutlip, Vet. Pathol. 41, 78 (2004). 7. S. B. Prusiner et al., Biochemistry 21, 4883 (1980). 8. M. Prinz et al., Am. J. Pathol. 162, 1103 (2003). 9. This work was supported by grants from the U.S. Public Health Service Supporting Online Material www.sciencemag.org/ Materials and Methods Fig. S1 21 November 2005; accepted 13 January 2006 Published online 26 January 2006; Table 1. Incubation times following inoculation of Tg(CerPrP)1536 mice with Inocula Incubation time, mean d ± SEM (n/n0)* Skeletal muscle Brain CWD-affected deer H92 360 ± 2 d (6/6) 283 ± 7 d (6/6) 33968 367 ± 9 d (8/8) 278 ± 11 d (6/6) 5941 427 ± 18 d (7/7) D10 483 ± 8 d (8/8) 231 ± 17 d (7/7) D08 492 ± 4 d (7/7) Averages 426 d 264 d Non-diseased deer FPS 6.98 >523 d (0/6) FPS 9.98 >454 d (0/7) >454 d (0/6) None >490 d (0/6) PBS >589 d (0/5) *The number of mice developing prion disease divided by the original number http://www.sciencemag.org/ Prions in Skeletal Muscles of Deer with Chronic Wasting Disease Rachel C. Angers, Shawn R. Browning, Tanya S. Seward, Christina J. Michael W. Miller, Edward A. Hoover, Glenn C. Telling§ §To whom correspondence should be addressed: E-mail: gtell2@uky.edu Published 26 January 2006 on Science Express DOI: 10.1126/science.1122864 This PDF file includes: Materials and Methods Fig. S1 Supporting Online Materials Materials and Methods Homogenates of semitendinosus/semimembranosus muscle (10% w/v in phosphate buffered saline) were prepared from five emaciated and somnolent mule deer, infected with CWD at the Colorado Division of Wildlife, Wildlife Research These deer were identified as D10, D08, 33968, H92, and 5941. CWD infection confirmed in all cases by the presence of histologic lesions in the brain spongiform degeneration of the perikaryon, the immunohistochemical detection disease-associated PrP in brain and tonsil, or by immunoblotting of disease associated PrP (CerPrPSc). Semitendinosus/semimembranosus muscle was obtained from two asymptomatic, mock inoculated deer, referred to as FPS that originated from a CWD non-endemic area and which were held indoors at State University from ten days of age. These control deer were confirmed CWD by histopathological and immunohistochemical analysis of brain tissue at The utmost care was taken to avoid inclusion of obvious nervous tissue when biopsies were prepared and to ensure that contamination of skeletal muscle CNS tissue did not occur. Fresh, single-use instruments were used to collect biopsy and a central piece from each sample was prepared with fresh, instruments to further isolate muscle tissue for inoculum preparation. Brain transmission were prepared separately from muscle as additional insurance contamination. 1 Groups of anesthetized Tg(CerPrP)1536 mice were inoculated intracerebrally of 1 % skeletal muscle or brain extracts prepared in phosphate buffered Inoculated Tg(CerPrP) mice were diagnosed with prion disease following the development of at least three neurologic symptoms including truncal ataxia, loss of extensor reflex, difficultly righting, and slowed movement. The time inoculation to the onset of clinical signs is referred to as the incubation For PrP analysis in brain extracts of Tg(CerPrP)1536 mice, 10 % homogenates in PBS were either untreated (-) or treated (+) with 40 µg/ml proteinase K hour at 37oC in the presence of 2% sarkosyl. Proteins were separated by sulfate polyacrylamide gel electrophoresis, analyzed by immunoblotting using monoclonal antibody 6H4 (Prionics AG, Switzerland), incubated with secondary antibody, developed using ECL-plus detection (Amersham), and using a FLA-5000 scanner (Fuji). 2 Fig. S1 PrP in brain extracts from representative Tg(CerPrP)1536 mice receiving tissue inocula from CWD-affected or CWD-negative deer. Extracts were either (+) or untreated (-) with proteinase K (PK) as indicated. The positions of molecular weight markers at 21.3, 28.7, 33.5 kDa (from bottom to top) are left of the immunoblot. 3 snip... http://www.bseinquiry.gov.uk/files/yb/1991/01/04004001.pdf Suggested citation for this article: Belay ED, Maddox RA, Williams ES, Miller MW, Gambetti P, Schonberger LB. Chronic wasting disease and potential transmission to humans. Emerg Infect Dis [serial on the Internet]. 2004 Jun [date cited]. Available from: http://www.cdc.gov/ncidod/EID/vol10no6/03-1082.htm Research Environmental Sources of Prion Transmission in Mule Deer Suggested citation for this article: Miller MW, Williams ES, Hobbs NT, Wolfe LL. Environmental sources of prion transmission in mule deer. Emerg Infect Dis [serial on the Internet]. 2004 Jun [date cited]. Available from: http://www.cdc.gov/ncidod/EID/vol10no6/04-0010.htm NEW STRAIN OF TSE USA CATTLE OR JUST INCOMPETENCE IN TESTING??? CJD WATCH http://www.fortunecity.com/healthclub/cpr/349/part1cjd.htm CJD WATCH MESSAGE BOARD http://disc.server.com/Indices/167318.html Terry S. Singeltary Sr.
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