*  Frontiers | Objects in Contact with Classical Scrapie Sheep Act as a Reservoir for Scrapie Transmission | Veterinary Science
Classical scrapie is an environmentally transmissible prion disease of sheep and goats. Prions can persist and remain potentially infectious in the environment for many years and thus pose a risk of infecting animals after re-stocking. In vitro studies using serial Protein Misfolding Cyclic Amplification (sPMCA) have suggested that objects on a scrapie-affected sheep farm could contribute to disease transmission. This in vivo study aimed to determine the role of field furniture (water troughs, feeding troughs, fencing and other objects that sheep may rub against) used by a scrapie-infected sheep flock as a vector for disease transmission to scrapie-free lambs with the prion protein genotype VRQ/VRQ, which is associated with high susceptibility to classical scrapie. When the field furniture was placed in clean accommodation sheep became infected when exposed to either a water trough (four out of five) or to objects used for ...
  https://www.frontiersin.org/articles/10.3389/fvets.2015.00032/full
*  Clinical findings in two cases of atypical scrapie in sheep: a case report | BMC Veterinary Research | Full Text
A new form of scrapie, later termed atypical scrapie, has first been described in Norway in 2003 [5]. The diagnosis of atypical scrapie is made if the TeSeE sheep/goat Western blot reveals a recognisable protein band with a molecular mass of less than 15 kDa and conspicuous PrPsc immunolabelling is detectable by IHC in the cerebellum and - at the obex level - in the nucleus of the spinal tract of the trigeminal nerve but absent in the dorsal motor nucleus of the vagal nerve [16]. Both cases fulfilled the criteria for atypical scrapie. In addition, both sheep were older sheep and carried the A F141RQ/AHQ PrP genotype, which has also been observed in other atypical scrapie cases [13, 14, 16].. Clinically, all affected animals in Norway reportedly displayed ataxia in the absence of pruritus; other infrequent signs were anxiety and loss of bodily condition [5]. The two cases reported as clinical suspects in Ireland all shared ...
  https://bmcvetres.biomedcentral.com/articles/10.1186/1746-6148-3-2
*  Table - Transgenic Mice Expressing Porcine Prion Protein Resistant to Classical Scrapie but Susceptible to Sheep Bovine...
How susceptible pigs are to infection with sheep prions is unknown. We show, through transmission experiments in transgenic mice expressing porcine prion protein (PrP), that the susceptibility of this mouse model to bovine spongiform encephalopathy (BSE) can be enhanced after its passage in ARQ sheep, indicating that the pathogenicity of the BSE agent is modified after passage in sheep. Transgenic mice expressing porcine PrP were, nevertheless, completely resistant to infection with a broad panel of classical scrapie isolates from different sheep PrP genotypes and with different biochemical characteristics. The atypical (Nor98 like) isolate (SC-PS152) was the only scrapie isolate capable of transmission in these mice, although with a marked transmission barrier. Unexpectedly, the atypical scrapie agent appeared to undergo a strain phenotype shift upon transmission to porcine-PrP transgenic mice and acquired new strain properties, suggesting that atypical ...
  https://wwwnc.cdc.gov/eid/article/15/8/08-1218-t1
*  Epidemiology and control of scrapie within a sheep flock. - PSY
Mathematical models of the transmission dynamics of scrapie are used to explore the expected course of an outbreak in a sheep flock, and the potential impacts of different control measures. All models incorporate sheep demography, a long and variable scrapie incubation period, horizontal and vertical routes of transmission and genetic variation in susceptibility. Outputs are compared for models which do and do not incorporate an environmental reservoir of infectivity, and which do and do not incorporate carrier genotypes. Numerical analyses using parameter values consistent with available data indicate that, in a closed flock, scrapie outbreaks may have a duration of several decades, reduce the frequency of susceptible genotypes, and may become endemic if carrier genotypes are present. In an open flock, endemic scrapie is possible even in the absence of carriers. Control measures currently or likely to become available may reduce the ...
  https://www.psy.ox.ac.uk/publications/17039
*  NASDA - National Scrapie Eradication Program 2016 Annual Report
The National Scrapie Eradication Program (NSEP) annual report for FY 2016 is now available. The annual report is available in both PowerPoint and PDF forms. Highlights of the Report include: over 40,000 sheep and goats were tested for scrapie in FY 2016, with only 13 animals confirmed positive for classical scrapie; the last case of scrapie was reported in April 2016; and only two infected and three source flocks were identified in FY 2016. Additional information about scrapie, the disease, and the national scrapie eradication program can be found on the APHIS VS Scrapie Website and www.eradicatescrapie.org.. ...
  http://nasda.org/News/47081.aspx
*  Pathological Prion Protein in the Tongues of Sheep Infected with Naturally Occurring Scrapie
Naturally Occurring Scrapie. Cristina Casalone, Cristiano Corona, Maria Ines Crescio, Francesca Martucci, Maria Mazza, Giuseppe Ru, Elena Bozzetta, Pier Luigi Acutis, and Maria Caramelli*. CEA, Italian Reference Laboratory for TSE, Istituto Zooprofilattico Sperimentale del Piemonte, Liguria e Valle d'Aosta, Turin, Italy. Received 12 July 2004/ Accepted 1 December 2004. Tongue involvement by prion spreading was shown to be a common outcome after oral or intracranial experimental challenge with scrapie and transmissible mink encephalopathy sources in rodent models. It is also known that bovine spongiform encephalopathy, which is pathogenic for humans, is experimentally transmissible to sheep and can lead to a disease indistinguishable from scrapie. A recent European Food Safety Authority opinion recommended research into PrPsc accumulation in the tongues of ruminants. We report on the detection of PrPsc in the tongues of seven scrapie-infected ...
  http://www.vegsource.com/talk/madcow/messages/94355.html
*  Publication : USDA ARS
Citation: Herrmann, L.M., Cheevers, W.P., Davis, W.C., Knowles, Jr., D.P., O'Rourke, K.I. 2005. CD21 positive follicular dendritic cells: A possible source of PrPSc in lymph node macrophages of scrapie-infected sheep. American Journal of Pathology. 162(4):1075-1081. Interpretive Summary: Natural sheep scrapie is part of a group of neurodegenerative diseases called transmissible spongiform encephopathies (TSE's) and continues to be spread in flocks in the United States. The presence of prion protein (PrPSc) in germinal centers of lymphoid tissues of scrapie-infected sheep is a marker for scrapie. But, little information exists regarding the specific cell types in lymphoid tissue that accumulate PrPSc. Using dual immunohistochemistry, follicular macrophages cells involved in the immune response of lymph nodes from scrapie-infected sheep were found to accumulate PrPSc. Technical Abstract: Natural sheep scrapie ...
  https://www.ars.usda.gov/research/publications/publication/?seqNo115=125256
*  Welcome to CDC stacks | Experimental Oral Transmission of Atypical Scrapie to Sheep - 24371 | Emerging Infectious Diseases
To investigate the possibility of oral transmission of atypical scrapie in sheep and determine the distribution of infectivity in the animals' peripheral tissues, we challenged neonatal lambs orally with atypical scrapie; they were then killed at 12 or 24 months. Screening test results were negative for disease-specific prion protein in all but 2 recipients; they had positive results for examination of brain, but negative for peripheral tissues. Infectivity of brain, distal ileum, and spleen from all animals was assessed in mouse bioassays; positive results were obtained from tissues that had negative results on screening. These findings demonstrate that atypical scrapie can be transmitted orally and indicate that it has the potential for natural transmission and iatrogenic spread through animal feed. Detection of infectivity in tissues negative by current surveillance methods indicates that diagnostic sensitivity is suboptimal for atypical ...
  https://stacks.cdc.gov/view/cdc/24371
*  Enteroglial and neuronal involvement without apparent neuron loss in ileal enteric nervous system plexuses from scrapie...
The enteric nervous system (ENS) probably plays a dominant role in sheep scrapie pathogenesis, but little is known about the cell types involved. We investigated the ileal myenteric and submucosal plexuses of four naturally and four orally experimentally scrapie-affected ARQ/ARQ Sarda sheep, as well as those of 12 healthy-control Sarda sheep carrying different PrP genotypes. All scrapie-affected animals, euthanized at clinical-disease end stage, showed PrPd deposition within enteric glial cells (EGCs) and calbindin-immunoreactive (CALB-IR) and neuronal nitric oxide synthase (nNOS)-IR neurons. Whole-mount investigations revealed no significant differences between the densities of total, CALB-IR and nNOS-IR neurons in scrapie-affected versus healthy sheep, irrespective of PrP genotype. Our results suggest that EGCs and CALB-IR and nNOS-IR neurons are probably involved in the pathogenesis of natural and oral experimental sheep ...
  http://www.zora.uzh.ch/id/eprint/60878/
*  NOR-98 ATYPICAL SCRAPIE USA UPDATE AS AT OCT 2007: National Scrapie Eradication Program May 2017 Monthly Report Fiscal Year 2017
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 ...
  http://nor-98.blogspot.com/2017/07/national-scrapie-eradication-program.html
*  PLOS ONE: The Limits of Test-Based Scrapie Eradication Programs in Goats
Small ruminant post-mortem testing programs were initially designed for monitoring the prevalence of prion disease. They are now considered as a potential alternative to genetic selection for eradicating/controlling classical scrapie at population level. If such policy should be implemented, its success would be crucially dependent on the efficiency of the surveillance system used to identify infected flocks. In this study, we first determined the performance of post-mortem classical scrapie detection in eight naturally affected goat herds (total n = 1961 animals) according to the age at culling. These results provided us with necessary parameters to estimate, through a Monte Carlo simulation model, the performance of scrapie detection in a commercial population. According to this model, whatever the number of tests performed, post mortem surveillance will have limited success in identifying infected herds. These data support the contention that ...
  http://journals.plos.org/plosone/article/comments?id=10.1371/journal.pone.0054911
*  USDA APHIS | National Scrapie Eradication Program
The program's goals are to eradicate classical scrapie from the United States and to meet World Organization for Animal Health (OIE) criteria for disease freedom. Since 2002, the prevalence of scrapie has decreased significantly through existing eradication efforts, largely a result of effective slaughter surveillance.. Since slaughter surveillance stared in FY 2003, the percent of cull sheep found positive at slaughter (once adjusted for face color) has decreased 90 percent. However, in order to declare the U.S. "scrapie free", we must be able to prove to the world that we have conducted testing in all sheep and goat populations. This is why your submission of samples from sheep/goats over 18 months of age found dead or euthanized on your farm is extremely important. Without your help, we will not be able to declare the US free of scrapie, costing the sheep and goat industries approximately $10 to $20 million, annually.. Remember: Educate, ...
  http://www.aphis.usda.gov/aphis/ourfocus/animalhealth/animal-disease-information/sheep-and-goat-health/national-scrapie-eradication-program/ct_scrapie_home/
*  Goat Genetics and Scrapie - Canadian Food Inspection Agency
Genotyping sheep for susceptibility to scrapie infection is an internationally recognized tool in scrapie disease control programs in many countries, including Canada's National Scrapie Eradication Program.
  http://www.inspection.gc.ca/animals/terrestrial-animals/diseases/reportable/scrapie/goat-genetics-and-scrapie/eng/1469836852107/1469836924249
*  scrapie
If the reader does not believe that viroids and satellites are distinctive, then surely prions, infectious agents composed only of protein, must impress.. The question of whether infectious agents exist without genomes arose with the discovery and characterization of infectious agents associated with a group of diseases called transmissible spongiform encephalopathies (TSEs). These diseases are rare, but always fatal, neurodegenerative disorders that afflict humans and other mammals. They are characterized by long incubation periods, spongiform changes in the brain associated with loss of neurons, and the absence of host responses. TSEs are caused by infectious proteins called prions.. The first TSE recognized was scrapie, so called because infected sheep tend to scrape their bodies on fences so much that they rub themselves raw. Scrapie has been recognized as a disease of European sheep for more than 250 years. It is endemic in some countries, for example, the United ...
  http://www.virology.ws/tag/scrapie/
*  Distinct patterns of spread of prion infection in brains of mice expressing anchorless or anchored forms of prion protein |...
In the present experiments tg44 mice expressing anchorless PrP and C57 mice expressing anchored PrP were found to be equal in susceptibility when tested by limiting dilution i.c. infection using RML scrapie (Figure 1). Therefore, the differences previously noted between these mouse strains in scrapie incubation period, types of clinical signs and neuropathology[28] are likely due to factors other than susceptibility to infection. In tg44 mice the lack of carbohydrates and GPI anchor on the anchorless PrP molecules as well as the lack of attachment to the plasma membrane would all be expected to contribute to an increased susceptibility to amyloid formation during the generation of PrPres[8, 36]. However, it is less clear whether the amyloid and non-amyloid forms of PrPres might differ in their neurotoxic potential. Furthermore, the cell surface expression of anchored PrPsen might also be an important factor in susceptibility of cells and tissues to toxic effects induced by ...
  https://actaneurocomms.biomedcentral.com/articles/10.1186/2051-5960-2-8
*  Publication : USDA ARS
Citation: Dassanayake, R.P., Schneider, D.A., Hoesing, L.M., Truscott, T.C., Davis, W.C., Orourke, K.I. 2012. Cell surface expression of PrP-c and the presence of scrapie prions in the blood of goats. Journal of General Virology. 1127-1131. Interpretive Summary: Classical scrapie is a naturally occurring fatal brain disease of goats and sheep which is caused by prions, a novel class of infectious agent. Presently, laboratory diagnosis of scrapie disease in live animals is limited to the detection of a misfolded form of prion protein (PrP-Sc) in biopsies of certain lymphoid tissues. Although collection of a blood sample for testing would be more convenient and allow more opportunity for repeat testing, the sensitivity for detecting PrP-Sc in blood samples of sheep has been unsuitably low. Furthermore, it is unknown whether scrapie-infected goat blood harbors infectious prions or PrP-Sc. In the research setting, we have confirmed the presence ...
  https://www.ars.usda.gov/research/publications/publication/?seqNo115=271530
*  Do we need to explain the occurrence of atypical scrapie?
SUNDAY, JULY 30, 2017 Do we need to explain the occurrence of atypical scrapie? ABSOLUTELY, AND HERE'S WHY... http://nor-98.blogspot.com/2017/07/do-we-need-to-explain-occurrence-of.html kind regards, terry
  http://braintalkcommunities.org/showthread.php/101782-Do-we-need-to-explain-the-occurrence-of-atypical-scrapie
*  Scrapie cash award - Farmers Weekly
20 June 1997 Scrapie cash award SCRAPIE research is to benefit from £3.8m funding, awarded to the Institute for Animal Health.Funding from the Biotech
  http://www.fwi.co.uk/news/scrapie-cash-award.htm
*  Case-control study on the use of pituitary-derived hormones from sheep as a potential risk factor for the occurrence of...
A case-control study was conducted in 2013 to investigate the use of pituitary-derived hormones from sheep as a potential risk factor for the presence of atypical scrapie in Great Britain sheep holdings. One hundred and sixty-five holdings were identified as cases. Two equal sets of controls were selected: no case of scrapie and cases of classical scrapie. A total of 495 holdings were selected for the questionnaire survey, 201 responses were received and 190 (38.3 per cent) were suitable for analysis. The variables 'use-of-heat-synchronisation/superovulation' and 'flock size' were significantly associated with the occurrence of atypical scrapie. Farms with atypical cases were less likely (OR 0.25, 95 per cent CI 0.07 to 0.89) to implement heat synchronisation/superovulation in the flock than the control group. Atypical cases were 3.3 times (95 per cent CI 1.38 to 8.13) more likely to occur in large holdings (,879 sheep) than in small flocks ...
  http://veterinaryrecord.bmj.com/content/180/16/403
*  CREUTZFELDT JAKOB DISEASE: July 2015
In addition to previous studies on scrapie transmission to primate1,8,9 and the recently published study on transgenic humanized mice13, our results constitute new evidence for recommending that the potential risk of scrapie for human health should not be dismissed. Indeed, human PrP transgenic mice and primates are the most relevant models for investigating the human transmission barrier. To what extent such models are informative for measuring the zoonotic potential of an animal TSE under field exposure conditions is unknown. During the past decades, many protective measures have been successfully implemented to protect cattle from the spread of c-BSE, and some of these measures have been extended to sheep and goats to protect from scrapie according to the principle of precaution. Since cases of c-BSE have greatly reduced in number, those protective measures are currently being challenged and relaxed in the absence of other known zoonotic animal prion ...
  http://creutzfeldt-jakob-disease.blogspot.com/2015_07_01_archive.html
*  The SEAC Sheep Subgroup Position Statement
Date: March 2, 2006 at 7:12 am PST. SEAC SHEEP SUBGROUP. POSITION STATEMENT. In view of SEAC s commitment to keep the scientific evidence. underpinning the strategy of the National Scrapie Plan (NSP) under. review1, and in light of rapidly emerging scientific findings relating to. atypical scrapie, the opinion of the sheep subgroup was sought by. SEAC in consultation with Defra. A meeting of the SEAC sheep. subgroup was held on January 24th 2006, with the following aims:. To give the best interpretation of the current data on atypical. scrapie and of the potential risks for a) animal health and b). human health. To consider whether new data change the risk. basis underpinning the NSP, flock control, or relevant sections. of the TSE roadmap.. To consider what additional information is necessary in order. to improve assessment of the risk for animal and human. health.. To produce a statement for consideration at SEAC 91. The meeting brought together European ...
  http://www.vegsource.com/talk/madcow/messages/1000499.html
*  Ovine reference materials and assays for prion genetic testing
Background: Genetic predisposition to scrapie in sheep is associated with several variations in the peptide sequence of the prion protein gene (PRNP). DNA-based tests for scoring PRNP codons are essential tools for eradicating scrapie and for evaluating rare alleles for increased resistance to disease. In addition to those associated with scrapie, there are dozens more PRNP polymorphisms that may occur in various flocks. If not accounted for, these sites may cause base-pair mismatching with oligonucleotides used in DNA testing. Thus, the fidelity of scrapie genetic testing is enhanced by knowing the position and frequency of PRNP polymorphisms in targeted flocks. Results: An adaptive DNA sequencing strategy was developed to determine the 771 bp PRNP coding sequence for any sheep and thereby produce a consensus sequence for targeted flocks. The strategy initially accounted for 43 known polymorphisms and facilitates the detection of unknown ...
  http://connection.ebscohost.com/c/articles/51089981/ovine-reference-materials-assays-prion-genetic-testing
*  BSE BASE MAD COW TESTING TEXAS, USA, AND CANADA: August 2008
The most frightening thing I have read all day is the report of Gambetti's finding of a new strain of sporadic cjd in young people...Dear God, what in the name of all that is holy is that!!! If the US has different strains of scrapie.....why????than the UK...then would the same mechanisms that make different strains of scrapie here make different strains of BSE...if the patterns are different in sheep and mice for scrapie.....could not the BSE be different in the cattle, in the mink, in the humans.......I really think the slides or tissues and everything from these young people with the new strain of sporadic cjd should be put up to be analyzed by many, many experts in cjd........bse.....scrapie Scrape the damn slide and put it into mice.....wait.....chop up the mouse brain and and spinal cord........put into some more mice.....dammit amplify the thing and start the damned research.....This is NOT rocket science...we need to use what we know ...
  http://madcowtesting.blogspot.com/2008/08/
*  BSE BASE MAD COW TESTING TEXAS, USA, AND CANADA: August 2008
The most frightening thing I have read all day is the report of Gambetti's finding of a new strain of sporadic cjd in young people...Dear God, what in the name of all that is holy is that!!! If the US has different strains of scrapie.....why????than the UK...then would the same mechanisms that make different strains of scrapie here make different strains of BSE...if the patterns are different in sheep and mice for scrapie.....could not the BSE be different in the cattle, in the mink, in the humans.......I really think the slides or tissues and everything from these young people with the new strain of sporadic cjd should be put up to be analyzed by many, many experts in cjd........bse.....scrapie Scrape the damn slide and put it into mice.....wait.....chop up the mouse brain and and spinal cord........put into some more mice.....dammit amplify the thing and start the damned research.....This is NOT rocket science...we need to use what we know ...
  http://madcowtesting.blogspot.com/2008_08_01_archive.html
*  Shepherd's Notebook: Scrapie Update
Wild & Woolly is a quarterly newsletter for sheep and goat producers and anyone else interested in small ruminants. Subscribe to the newsletter listserv if you want to receive an email when a new issue of the newsletter has been posted to the web. To subscribe, send an email to listserv@listserv.umd.edu. In the body of the message, write subscribe sheepandgoatnews. Mailed copies of the newsletter are available for $10/year. ...
  http://mdsheepgoat.blogspot.com/2014/03/scrapie-update.html