Monday, January 28, 2013

Brazil MAD COW BSE ban to stay

Brazil BSE ban to stay




XAVIER PORTER27 Jan, 2013 04:00 AM




BRAZIL's issues with bovine spongiform encephalopathy (BSE) are not going away in a hurry.


Since the announcement of its first case of BSE, describing it as "atypical" on December 7, 10 countries have imposed full or partial bans.


Many export and import countries were concerned about the two-year delay between when the BSE cow was first tested and the finding which was only released in early December, 2012.


The concerns raised by these countries has now resulted in Brazil's government and the World Organisation for Animal Health (OIE) to further investigate Brazil's transparency in their BSE surveillance system and testing processes.


The following are the countries who have introduced partial and full bans since the announcement South Korea, China, Japan, South Africa, Saudi Arabia, Peru (full ban for 90 days), Chile, Jordan (only beef from Parana State), Taiwan (processed beef-only) and Lebanon (Parana State).


The combined export volume of these countries equates to 150,000 tonnes of beef per year or 12.5 per cent of Brazil's total exports.


The export opportunities for Australia may be limited due to the other cheaper beef supply countries, with FMD (foot and mouth disease) beef, that export to the same countries as Brazil.


Russia being the potential exception for Australia due to the heavy tariffs imposed on countries like India at 31pc duty, its annual import needs of 220,000t of beef could prove to be a boon for Australian exporters.




Brazil makes WTO threat


On December 21, Brazil threatened to take action through the World Trade Organisation (WTO) unless the countries that have imposed bans on Brazilian beef reverse their decisions by March.


These actions had the opposite impact, with five additional countries imposing bans since the WTO threat was announced.


There is no doubt the initial response by Brazil was to try to as quickly as possible play down the impact of the BSE case and to 'pull into line' the importers that had banned Brazilian beef by threatening to take them to the WTO.


But instead raised further concerns by more countries about Brazil's BSE surveillance procedures, with more country's asking the same question why was there a two-year delay on results being made known?


The US Ranchers-Cattlemen Action Legal Fund (R-CALF) raised directly with the OIE their additional procedure concerns about the long delay between the confirmed two positive tests six months to be exact.


After the brain sample tested positive in mid-2012, it was sent to the OIE reference laboratory in the United Kingdom, where it again tested positive for BSE on December 6, 2012.


Officials allegedly claim the long delay between the two primary tests was due to a combination of a work overload at the testing laboratory and OIE rules that caused Brazil to lower the priority of testing the suspect cow.




BSE audits to be conducted




On January 5, Brazil's agriculture ministry confirmed the country's beef industry would be thoroughly audited.


State government teams would be sent to beef producers to ensure procedures to safeguard cows' health were being properly followed.


A review on all operational procedures would be made in accordance with OIE guidelines.


Ministry of Agriculture international relations secretary Celio Porto said the audit will start by looking at what possible failures may exist in the fight against BSE.


This decision seems to be made on the back of the OIE scientific committee announcing it will examine Brazil's BSE risk status and conduct an audit next month.


The decision by the OIE to do this audit seems to be related to R-CALF questioning of not just Brazil's BSE surveillance systems but also the OIE's procedures.


R-CALF regards the two-year delay as a symptom of the failure of the OIE's global system that they believe incorrectly assumes foreign countries, particularly developing countries, have the same means, commitment and capabilities as the US to control and eradicate diseases.


The OIE annual meeting is to be held in May at which the findings of their Brazilian audit and any recommendation would be tabled and considered.





In essence, the credibility of not only Brazil's BSE surveillance system has been questioned but also the OIE's global system.






A negative OIE review would be disastrous for Brazil, which could lead to potentially more bans from import countries or at best the current bans remaining in place for an extended period.


It would seem obvious to me that no country is likely to remove bans before May until the findings of the OIE review are made public.


The message is clear those countries that have imposed bans are needing transparency in the current Brazil BSE surveillance system and a credible reason why there was a two-year delay in the findings.






Russia to review its position





Russia's decision to wait and evaluate the situation is based on the comforting fact that back in 2011 Russia imposed bans on three Brazilian States Mato Grosso, Parana, Rio and Granda do Sul.


These bans are still in place and Parana, which was the State that the atypical case was found in, has not shipped beef to Russia in almost 18 months.


The concerns at the time related to sanitary issues and since then 85 export establishments from these three States have been prevented from exporting.


The need for transparency in any countries BSE surveillance system and testing processes can not be underestimated and Brazil is no exception.


An independent and credible body, such as the OIE, is critical to ensure that all importing countries can see correct procedures have been followed and similar occurrences won't be repeated.


Until these concerns are met, Brazil's beef exports future among the 10 countries with bans in place will remain uncertain.












Friday, December 07, 2012



ATYPICAL BSE BRAZIL 2010 FINALLY CONFIRMED OIE 2012














Wednesday, December 19, 2012



Scientific Report of the European Food Safety Authority on the Assessment of the Geographical BSE Risk (GBR) of Brazil

















Friday, January 25, 2013



Japan may relax US Mad Cow BSE beef import rules in Feb 2013
















Monday, January 28, 2013



U.S. Trade Representative Ron Kirk and Agriculture Secretary Tom Vilsack Announce Agreement to Further Open Japan’s Market to U.S. Beef


















Thursday, January 17, 2013



Canada, U.S. agree on animal-disease measures to protect trade, while reducing human and animal health protection














IT is of my opinion, that the OIE and the USDA et al, are the soul reason, and responsible parties, for Transmissible Spongiform Encephalopathy TSE prion diseases, including typical and atypical BSE, typical and atypical Scrapie, and all strains of CWD, and human TSE there from, spreading around the globe.




I have lost all confidence of this organization as a regulatory authority on animal disease, and consider it nothing more than a National Trading Brokerage for all strains of animal TSE, just to satisfy there commodity. AS i said before, OIE should hang up there jock strap now, since it appears they will buckle every time a country makes some political hay about trade protocol, commodities and futures. IF they are not going to be science based, they should do everyone a favor and dissolve there organization.




JUST because of low documented human body count with nvCJD and the long incubation periods, the lack of sound science being replaced by political and corporate science in relations with the fact that science has now linked some sporadic CJD with atypical BSE and atypical scrapie, and the very real threat of CWD being zoonosis, I believed the O.I.E. has failed terribly and again, I call for this organization to be dissolved. ...







Tuesday, July 17, 2012


O.I.E. BSE, CWD, SCRAPIE, TSE PRION DISEASE Final Report of the 80th General Session, 20 - 25 May 2012










Thursday, December 20, 2012


OIE GROUP RECOMMENDS THAT SCRAPE PRION DISEASE BE DELISTED AND SAME OLD BSe WITH BOVINE MAD COW DISEASE










Wednesday, May 25, 2011


O.I.E. Terrestrial Animal Health Standards Commission and prion (TSE) disease reporting 2011


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


From: Terry S. Singeltary Sr.


To: BSE-L@LISTS.AEGEE.ORG


Cc: trade@oie.int ; oie@oie.int ; f.diaz@oie.int ; scientific.dept@oie.int ; cjdvoice@yahoogroups.com ; BLOODCJD@YAHOOGROUPS.COM


Sent: Tuesday, May 24, 2011 2:24 PM


Subject: O.I.E. Terrestrial Animal Health Standards Commission and prion (TSE) disease reporting 2011










Saturday, December 18, 2010


OIE Global Conference on Wildlife Animal Health and Biodiversity - Preparing for the Future (TSE AND PRIONS) Paris (France), 23-25 February 2011










Monday, November 23, 2009


BSE GBR RISK ASSESSMENTS UPDATE NOVEMBER 23, 2009 COMMISSION OF THE EUROPEAN COMMUNITIES AND O.I.E. COMMISSION DECISION of 11 November 2009 amending the Annex to Decision 2007/453/EC as regards the BSE status of Chile, Colombia and Japan (notified under document C(2009) 8590)










Tuesday, January 1, 2008


BSE OIE USDA


Subject: OIE BSE RECOMMENDATION FOR USA, bought and paid for by your local cattle dealers i.e. USDA


Date: May 14, 2007 at 9:00 am PST


OIE BSE RECOMMENDATION FOR USA, bought and paid for by your local cattle dealers i.e. USDA


STATEMENT BY DR. RON DEHAVEN REGARDING OIE RISK RECOMMENDATION


March 9, 2007











Tuesday, June 26, 2012


Creutzfeldt Jakob Disease Human TSE report update North America, Canada, Mexico, and USDA PRION UNIT as of May 18, 2012


type determination pending Creutzfeldt Jakob Disease (tdpCJD), is on the rise in Canada and the USA












Monday, December 31, 2012


Creutzfeldt Jakob Disease and Human TSE Prion Disease in Washington State, 2006–2011-2012









16 year old with mad cow type TSE prion disease USA, course Gambetti et al changed the name again...






Monday, January 14, 2013


Gambetti et al USA Prion Unit change another highly suspect USA mad cow victim to another fake name i.e. sporadic FFI at age 16 CJD Foundation goes along with this BSe









Monday, December 31, 2012


Creutzfeldt Jakob Disease and Human TSE Prion Disease in Washington State, 2006–2011-2012









Tuesday, December 25, 2012


CREUTZFELDT JAKOB TSE PRION DISEASE HUMANS END OF YEAR REVIEW DECEMBER 25, 2012








Friday, November 23, 2012


sporadic Creutzfeldt-Jakob Disease update As at 5th November 2012 UK, USA, AND CANADA








Sunday, December 2, 2012


CANADA 19 cases of mad cow disease SCENARIO 4: ‘WE HAD OUR CHANCE AND WE BLEW IT’








Tuesday, November 02, 2010


IN CONFIDENCE


The information contained herein should not be disseminated further except on the basis of "NEED TO KNOW".


BSE - ATYPICAL LESION DISTRIBUTION (RBSE 92-21367) statutory (obex only) diagnostic criteria CVL 1992









2009 UPDATE ON ALABAMA AND TEXAS MAD COWS 2005 and 2006









Comments on technical aspects of the risk assessment were then submitted to FSIS.




Comments were received from Food and Water Watch, Food Animal Concerns Trust (FACT), Farm Sanctuary, R-CALF USA, Linda A Detwiler, and Terry S. Singeltary.


This document provides itemized replies to the public comments received on the 2005 updated Harvard BSE risk assessment. Please bear the following points in mind:









Owens, Julie


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


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


To: FSIS RegulationsComments


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


Page 1 of 98









FSIS, USDA, REPLY TO SINGELTARY









U.S.A. 50 STATE BSE MAD COW CONFERENCE CALL Jan. 9, 2001













2012 atypical L-type BSE BASE California reports


Saturday, August 4, 2012


Final Feed Investigation Summary - California BSE Case - July 2012








SUMMARY REPORT CALIFORNIA BOVINE SPONGIFORM ENCEPHALOPATHY CASE INVESTIGATION JULY 2012


Summary Report BSE 2012


Executive Summary









Saturday, August 4, 2012


Update from APHIS Regarding Release of the Final Report on the BSE Epidemiological Investigation









CENSORSHIP IS A TERRIBLE THING $$$





Canada has had a COVER-UP policy of mad cow disease since about the 17th case OR 18th case of mad cow disease. AFTER THAT, all FOIA request were ignored $$$


THIS proves there is indeed an epidemic of mad cow disease in North America, and it has been covered up for years and years, if not for decades, and it’s getting worse $$$


Thursday, February 10, 2011


TRANSMISSIBLE SPONGIFORM ENCEPHALOPATHY REPORT UPDATE CANADA FEBRUARY 2011 and how to hide mad cow disease in Canada Current as of: 2011-01-31









Wednesday, August 11, 2010


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








Thursday, August 19, 2010


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









Friday, March 4, 2011


Alberta dairy cow found with mad cow disease









Reasons for the New Regulation Order No. 23 (as well as amending Order No. 149) of the State Committee for Veterinary Medicine name BSE as the reason for new import requirement. The legal title for Order No. 23 is "On Urgent Measures Aimed at Prevention and Elimination of BSE and Other Prion Infections in Cattle”. Neither Order explains how the threat of introduction of BSE can be addressed through the inspection of producers of all products of animal origin including fish, dairy products, poultry and pork. It is not clear what other concerns are addressed through the proposed inspections. Formal Notification of Trading Partners On August 3rd, Ukraine's Notification and Enquiry Point issued a legal Notification G/SPS/N/UKR/3/Rev.1 found on the Official WTO Website (Committee on Sanitary and Phytosanitary Measures)









Thursday, March 29, 2012


atypical Nor-98 Scrapie has spread from coast to coast in the USA 2012


NIAA Annual Conference April 11-14, 2011 San Antonio, Texas









Monday, November 30, 2009


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








Monday, December 1, 2008


When Atypical Scrapie cross species barriers








EVIDENCE OF SCRAPIE IN SHEEP AS A RESULT OF FOOD BORNE EXPOSURE


This is provided by the statistically significant increase in the incidence of sheep scrape from 1985, as determined from analyses of the submissions made to VI Centres, and from individual case and flock incident studies. ........









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










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







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).








Epidemiology of Scrapie in the United States 1977











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 ?








Friday, February 11, 2011


Atypical/Nor98 Scrapie Infectivity in Sheep Peripheral Tissues








Sunday, December 12, 2010


EFSA reviews BSE/TSE infectivity in small ruminant tissues News Story 2 December 2010







Wednesday, January 18, 2012


Selection of Distinct Strain Phenotypes in Mice Infected by Ovine Natural Scrapie Isolates Similar to CH1641 Experimental Scrapie


Journal of Neuropathology & Experimental Neurology: February 2012 - Volume 71 - Issue 2 - p 140–147








Thursday, July 14, 2011


Histopathological Studies of "CH1641-Like" Scrapie Sources Versus Classical Scrapie and BSE Transmitted to Ovine Transgenic Mice (TgOvPrP4)








Wednesday, January 18, 2012


BSE IN GOATS CAN BE MISTAKEN FOR SCRAPIE


February 1, 2012








Thursday, December 23, 2010


Molecular Typing of Protease-Resistant Prion Protein in Transmissible Spongiform Encephalopathies of Small Ruminants, France, 2002-2009


Volume 17, Number 1 January 2011









Thursday, November 18, 2010


Increased susceptibility of human-PrP transgenic mice to bovine spongiform encephalopathy following passage in sheep









Wednesday, February 16, 2011


IN CONFIDENCE


SCRAPIE TRANSMISSION TO CHIMPANZEES


IN CONFIDENCE









why do we not want to do TSE transmission studies on chimpanzees $





5. A positive result from a chimpanzee challenged severly would likely create alarm in some circles even if the result could not be interpreted for man. I have a view that all these agents could be transmitted provided a large enough dose by appropriate routes was given and the animals kept long enough. Until the mechanisms of the species barrier are more clearly understood it might be best to retain that hypothesis.


snip...


R. BRADLEY










Friday, February 11, 2011


Atypical/Nor98 Scrapie Infectivity in Sheep Peripheral Tissues









Monday, April 25, 2011


Experimental Oral Transmission of Atypical Scrapie to Sheep


Volume 17, Number 5-May 2011









Sunday, April 18, 2010


SCRAPIE AND ATYPICAL SCRAPIE TRANSMISSION STUDIES A REVIEW 2010









Thursday, March 29, 2012


atypical Nor-98 Scrapie has spread from coast to coast in the USA 2012


NIAA Annual Conference April 11-14, 2011San Antonio, Texas









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.







another atypical Nor-98 Scrapie case documented in Canada for 2012





Date confirmed Location Animal type infected May 31* Quebec Sheep










Sunday, April 29, 2012


Scrapie confirmed at quarantined sheep farm Canada CFIA






Wednesday, April 4, 2012


20120402 - Breach of quarantine/Violation de la mise en quarantaine of an ongoing Scrapie investigation









Thursday, February 23, 2012


Atypical Scrapie NOR-98 confirmed Alberta Canada sheep January 2012









Tuesday, November 6, 2012


Transmission of New Bovine Prion to Mice, Atypical Scrapie, BSE, and Sporadic CJD, November-December 2012 update









Monday, March 19, 2012


Infectivity in Skeletal Muscle of Cattle with Atypical Bovine Spongiform Encephalopathy


PLoS One. 2012; 7(2): e31449.











Thursday, March 29, 2012


atypical Nor-98 Scrapie has spread from coast to coast in the USA 2012


NIAA Annual Conference April 11-14, 2011San Antonio, Texas











Monday, April 25, 2011


Experimental Oral Transmission of Atypical Scrapie to Sheep


Volume 17, Number 5-May 2011 However, work with transgenic mice has demonstrated the potential susceptibility of pigs, with the disturbing finding that the biochemical properties of the resulting PrPSc have changed on transmission (40).










***The pathology features of Nor98 in the cerebellum of the affected sheep showed similarities with those of sporadic Creutzfeldt-Jakob disease in humans.










*** Intriguingly, these conclusions suggest that some pathological features of Nor98 are reminiscent of Gerstmann-Sträussler-Scheinker disease.


119








*** 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.










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.











Monday, November 30, 2009


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









I strenuously urge the USDA and the OIE et al to revoke the exemption of the legal global trading of atypical Nor-98 scrapie TSE. ...TSS







Thursday, February 23, 2012


EIGHT FORMER SECRETARIES OF AGRICULTURE SPEAKING AT USDA'S 2012 AGRICULTURE OUTLOOK FORUM INDUCTED INTO USA MAD COW HALL OF SHAME









TSS

Saturday, January 05, 2013

Immunohistochemical Detection of Disease- Associated Prion Protein in the Peripheral Nervous System in Experimental H-Type Bovine Spongiform Encephalopathy

Immunohistochemical Detection of Disease- Associated Prion Protein in the Peripheral Nervous System in Experimental H-Type Bovine Spongiform Encephalopathy



H. Okada1, Y. Iwamaru1, T. Yokoyama1, and S. Mohri1



Abstract



H-type bovine spongiform encephalopathy (BSE) has been identified in aged cattle in Europe and North America. To determine the localization of disease-associated prion protein (PrPSc) in the peripheral nerve tissues of cattle affected with H-type BSE, we employed highly sensitive immunohistochemical and immunofluorescence techniques with the tyramide signal amplification (TSA) system. PrPSc deposition was detected in the inferior ganglia, sympathetic nerve trunk, vagus nerve, spinal nerves, cauda equina, and adrenal medulla, using this system. Notably, granular PrPSc deposits were present mainly in the Schwann cells and fibroblastlike cells and occasionally along certain nerve fibers at the surface of the axons. In the adrenal gland, PrPSc immunolabeling was observed within the sympathetic nerve fibers and nerve endings in the adrenal medulla. Although our results were limited to only 3 experimental cases, these results suggest that the TSA system, a highly sensitive immunohistochemical procedure, may help in elucidating the peripheral pathogenesis of H-type BSE.



Keywords



atypical bovine spongiform encephalopathy, H-type, prion, tyramide amplification, peripheral nervous system



snip...




Neuropil, but not neuronal, vacuolation was obvious in all brain areas, especially in the nuclei of the thalamus and brainstem. Immunolabeled PrPSc was widely distributed throughout the brain and spinal cord (Fig. 1). Eight patterns of PrPSc immunolabeling including intraneuronal, perineuronal, intraglial, linear, fine particulate, coarse granular, stellate, and plaques were identified in the brain. The most conspicuous pattern of PrPSc was that of fine particulate and coarse granular deposits in the cerebral and cerebellar cortices and the nuclei of the thalamus, brainstem, and cerebellum. Stellate-type PrPSc deposition was detected in the cerebral and cerebellar cortices, basal ganglia, thalamus, hypothalamus, and hippocampus. Intraglial-type PrPSc deposition was conspicuous throughout the white matter of the brain and spinal cord. Spherical PrPSc plaques were sparsely located in the thalamus, basal ganglia, midbrain, pons, deeper layers of the cerebral cortices, and subcortical white matter. Immunolabeled PrPSc was observed in the retina, neurohypophysis, and optic nerve. In the trigeminal and dorsal root ganglia, PrPSc was mainly found accumulated in both ganglionic and satellite cells, using the conventional polymer immunodetection method as described previously.9 In addition to the extracerebral tissues described above, positive PrPSc immunolabeling was detected in the adrenal gland, cauda equina, cervical spinal nerves, facial nerve, hypoglossal nerve, vagus nerve, sciatic nerve, and ganglia, such as the inferior ganglion of the vagus nerve, superior cervical ganglion, stellate ganglion, ganglia of the sympathetic trunk, and celiac and mesenteric ganglion complex, with the use of the TSA-biotin system. In these ganglia, not all neurons of ganglia exhibited intracytoplasmic labeling, but labeling was evident in the Schwann cells or fibroblast-like cells (Figs. 2, 3). In the cauda equina, dorsal roots of the cervical spinal nerves, facial nerve, hypoglossal nerve, vagus nerve, and sciatic nerve, granular PrPSc aggregate was mainly located in Schwann cells. In the adrenal gland, the PrPSc signal was evident at the intercellular fine processes of the nerve endings between the chromaffin cells of the adrenal medulla (Fig. 4). Immunolabeled PrPSc was not detected in the enteric nervous system such as the myenteric and submucosal plexi of the digestive tract. No specific immunolabeling was detectable in the lymphoid tissues with the TSA-biotin system.




Using the dual or triple immunofluorescence technique, localization of PrPSc was visible in the ganglionic and satellite cells of the ganglia in the merged images (Fig. 5). In addition, the merged image showed that PrPSc granules were rarely located at the periphery of axons or within the axons adjacent to the ganglia (Fig. 6). Moreover, granular PrPSc was observed outside S100-positive cells (Fig. 7). In the cauda equina and spinal nerves, PrPSc coexisted mostly in the periphery of Schwann cells labeled with MBP and S100 (Fig. 8). No PrPSc immunolabeling was detected in the compact layers of the myelin sheath. In addition, no positive signal was detected in the sections from uninfected controls incubated with mAb F99/97.6.1 by both TSA-biotin and TSA-fluorescence methods, and no background immunostaining was observed in any sections of H-type infected animals by both methods when non-immune mouse and rabbit IgG, or PBS, were applied to the sections instead of the primary PrP-antibody (Figs. 9, 10).





snip...see full text ;














snip...




Unfortunately, a detailed and all-encompassing analysis of neuropathology and topographical distribution of immunolabeled PrPSc in H-type BSE-affected cattle could not be performed, since only the obex region is routinely sampled for BSE surveillance testing and the remaining brain as well as the carcasses are not available in most countries [3,10,12,13,24-27]. Recently, clinical signs and biochemical properties of experimental German H-type BSE cases have been reported [20]. The primary objective of this study was to investigate the transmissibility of H-type BSE, using a field isolate detected in the active surveillance program in Canada [12]. The secondary objective was to extend the knowledge of the topographical distribution and deposition patterns of immunolabeled PrPSc in H-type BSE.




snip...






In addition, the present data will support risk assessments in some peripheral tissues derived from cattle affected with H-type BSE.











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








Thursday, June 23, 2011


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







P.4.23 Transmission of atypical BSE in humanized mouse models



Liuting Qing1, Wenquan Zou1, Cristina Casalone2, Martin Groschup3, Miroslaw Polak4, Maria Caramelli2, Pierluigi Gambetti1, Juergen Richt5, Qingzhong Kong1 1Case Western Reserve University, USA; 2Instituto Zooprofilattico Sperimentale, Italy; 3Friedrich-Loeffler-Institut, Germany; 4National Veterinary Research Institute, Poland; 5Kansas State University (Previously at USDA National Animal Disease Center), USA



Background: Classical BSE is a world-wide prion disease in cattle, and the classical BSE strain (BSE-C) has led to over 200 cases of clinical human infection (variant CJD). Atypical BSE cases have been discovered in three continents since 2004; they include the L-type (also named BASE), the H-type, and the first reported case of naturally occurring BSE with mutated bovine PRNP (termed BSE-M). The public health risks posed by atypical BSE were largely undefined.



Objectives: To investigate these atypical BSE types in terms of their transmissibility and phenotypes in humanized mice.



Methods: Transgenic mice expressing human PrP were inoculated with several classical (C-type) and atypical (L-, H-, or Mtype) BSE isolates, and the transmission rate, incubation time, characteristics and distribution of PrPSc, symptoms, and histopathology were or will be examined and compared.



Results: Sixty percent of BASE-inoculated humanized mice became infected with minimal spongiosis and an average incubation time of 20-22 months, whereas only one of the C-type BSE-inoculated mice developed prion disease after more than 2 years. Protease-resistant PrPSc in BASE-infected humanized Tg mouse brains was biochemically different from bovine BASE or sCJD. PrPSc was also detected in the spleen of 22% of BASE-infected humanized mice, but not in those infected with sCJD. Secondary transmission of BASE in the humanized mice led to a small reduction in incubation time.



*** The atypical BSE-H strain is also transmissible with distinct phenotypes in the humanized mice, but no BSE-M transmission has been observed so far.




Discussion: Our results demonstrate that BASE is more virulent than classical BSE, has a lymphotropic phenotype, and displays a modest transmission barrier in our humanized 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.









P26 TRANSMISSION OF ATYPICAL BOVINE SPONGIFORM ENCEPHALOPATHY (BSE) IN HUMANIZED MOUSE MODELS



Liuting Qing1, Fusong Chen1, Michael Payne1, Wenquan Zou1, Cristina Casalone2, Martin Groschup3, Miroslaw Polak4, Maria Caramelli2, Pierluigi Gambetti1, Juergen Richt5*, and Qingzhong Kong1 1Department of Pathology, Case Western Reserve University, Cleveland, OH 44106, USA; 2CEA, Istituto Zooprofilattico Sperimentale, Italy; 3Friedrich-Loeffler-Institut, Germany; 4National Veterinary Research Institute, Poland; 5Kansas State University, Diagnostic Medicine/Pathobiology Department, Manhattan, KS 66506, USA. *Previous address: USDA National Animal Disease Center, Ames, IA 50010, USA



Classical BSE is a world-wide prion disease in cattle, and the classical BSE strain (BSE-C) has led to over 200 cases of clinical human infection (variant CJD). Two atypical BSE strains, BSE-L (also named BASE) and BSE-H, have been discovered in three continents since 2004. The first case of naturally occurring BSE with mutated bovine PrP gene (termed BSE-M) was also found in 2006 in the USA. The transmissibility and phenotypes of these atypical BSE strains/isolates in humans were unknown. We have inoculated humanized transgenic mice with classical and atypical BSE strains (BSE-C, BSE-L, BSE-H) and the BSE-M isolate. We have found that the atypical BSE-L strain is much more virulent than the classical BSE-C.



*** The atypical BSE-H strain is also transmissible in the humanized transgenic mice with distinct phenotype, but no transmission has been observed for the BSE-M isolate so far.



III International Symposium on THE NEW PRION BIOLOGY: BASIC SCIENCE, DIAGNOSIS AND THERAPY 2 - 4 APRIL 2009, VENEZIA (ITALY)











I ask Professor Kong ;



Thursday, December 04, 2008 3:37 PM


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



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



Professor Kong reply ;



.....snip



''As to the H-BSE, we do not have sufficient data to say one way or another, but we have found that H-BSE can infect humans. I hope we could publish these data once the study is complete. Thanks for your interest.'' Best regards, Qingzhong Kong, PhD Associate Professor Department of Pathology Case Western Reserve University Cleveland, OH 44106 USA




END...TSS




Thursday, December 04, 2008 2:37 PM


"we have found that H-BSE can infect humans." personal communication with Professor Kong. ...TSS


BSE-H is also transmissible in our humanized Tg mice.


The possibility of more than two atypical BSE strains will be discussed.


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








SNIP...




Friday, May 11, 2012


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






Thursday, June 21, 2012


Clinical and Pathologic Features of H-Type Bovine Spongiform Encephalopathy Associated with E211K Prion Protein Polymorphism







Tuesday, November 02, 2010



IN CONFIDENCE



The information contained herein should not be disseminated further except on the basis of "NEED TO KNOW".



BSE - ATYPICAL LESION DISTRIBUTION (RBSE 92-21367) statutory (obex only) diagnostic criteria CVL 1992









2009 UPDATE ON ALABAMA AND TEXAS MAD COWS 2005 and 2006








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.














her healthy calf also carried the mutation (J. A. Richt and S. M. Hall PLoS Pathog. 4, e1000156; 2008).




This raises the possibility that the disease could occasionally be genetic in origin. Indeed, the report of the UK BSE Inquiry in 2000 suggested that the UK epidemic had most likely originated from such a mutation and argued against the scrapierelated assumption. Such rare potential pathogenic PRNP mutations could occur in countries at present considered to be free of BSE, such as Australia and New Zealand. So it is important to maintain strict surveillance for BSE in cattle, with rigorous enforcement of the ruminant feed ban (many countries still feed ruminant proteins to pigs). Removal of specified risk material, such as brain and spinal cord, from cattle at slaughter prevents infected material from entering the human food chain. Routine genetic screening of cattle for PRNP mutations, which is now available, could provide additional data on the risk to the public. Because the point mutation identified in the Alabama animals is identical to that responsible for the commonest type of familial (genetic) CJD in humans, it is possible that the resulting infective prion protein might cross the bovine–human species barrier more easily. Patients with vCJD continue to be identified. The fact that this is happening less often should not lead to relaxation of the controls necessary to prevent future outbreaks. Malcolm A. Ferguson-Smith Cambridge University Department of Veterinary Medicine, Madingley Road, Cambridge CB3 0ES, UK e-mail: maf12@cam.ac.uk Jürgen A. Richt College of Veterinary Medicine, Kansas State University, K224B Mosier Hall, Manhattan, Kansas 66506-5601, USA




NATURE|Vol 457|26 February 2009











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)










Tuesday, November 6, 2012



***Transmission of New Bovine Prion to Mice, Atypical Scrapie, BSE, and Sporadic CJD, November-December 2012 update










Comments on technical aspects of the risk assessment were then submitted to FSIS.





Comments were received from Food and Water Watch, Food Animal Concerns Trust (FACT), Farm Sanctuary, R-CALF USA, Linda A Detwiler, and Terry S. Singeltary.


This document provides itemized replies to the public comments received on the 2005 updated Harvard BSE risk assessment. Please bear the following points in mind:









Owens, Julie


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


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


To: FSIS RegulationsComments



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










FSIS, USDA, REPLY TO SINGELTARY










U.S.A. 50 STATE BSE MAD COW CONFERENCE CALL Jan. 9, 2001
















2012 atypical L-type BSE BASE California reports





SUMMARY REPORT CALIFORNIA BOVINE SPONGIFORM ENCEPHALOPATHY CASE INVESTIGATION JULY 2012



Summary Report BSE 2012



Executive Summary










Saturday, August 4, 2012



Update from APHIS Regarding Release of the Final Report on the BSE Epidemiological Investigation










Saturday, August 4, 2012



*** Final Feed Investigation Summary - California BSE Case - July 2012










Saturday, December 15, 2012




Bovine spongiform encephalopathy: the effect of oral exposure dose on attack rate and incubation period in cattle -- an update 5 December 2012












Thursday, December 20, 2012



OIE GROUP RECOMMENDS THAT SCRAPE PRION DISEASE BE DELISTED AND SAME OLD BSe WITH BOVINE MAD COW DISEASE











Tuesday, December 25, 2012



A Quantitative Assessment of the Amount of Prion Diverted to Category 1 Materials and Wastewater During Processing











2011 Monday, September 26, 2011



L-BSE BASE prion and atypical sporadic CJD











Tuesday, June 26, 2012



Creutzfeldt Jakob Disease Human TSE report update North America, Canada, Mexico, and USDA PRION UNIT as of May 18, 2012



type determination pending Creutzfeldt Jakob Disease (tdpCJD), is on the rise in Canada and the USA










Sunday, December 2, 2012



CANADA 19 cases of mad cow disease SCENARIO 4: ‘WE HAD OUR CHANCE AND WE BLEW IT’









Monday, July 23, 2012



The National Prion Disease Pathology Surveillance Center July 2012









Tuesday, December 25, 2012



CREUTZFELDT JAKOB TSE PRION DISEASE HUMANS END OF YEAR REVIEW DECEMBER 25, 2012










Monday, December 31, 2012



Creutzfeldt Jakob Disease and Human TSE Prion Disease in Washington State, 2006–2011-2012









TSS