Abstract
Because variant Creutzfeldt–Jakob disease (vCJD) in humans probably results from consumption of products contaminated with tissue from animals with bovine spongiform encephalopathy, whether infectious prion protein is present in ruminant muscles is a crucial question. Here we show that experimentally and naturally scrapie-affected sheep accumulate the prion protein PrPSc in a myocyte subset. In naturally infected sheep, PrPSc is detectable in muscle several months before clinical disease onset. The relative amounts of PrPSc suggest a 5,000-fold lower infectivity for muscle as compared to brain.
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Acknowledgements
The authors thank the INRA domain of Langlade & VLA Weybridge for producing and breeding the animals used in this study, Bio-Rad for providing the TeSeE sheep-goat kits and G. Hunsmann (German Primate Center, Göttingen, Germany) for providing mouse monoclonal antibody 8G8. This work was supported financially by 'GIS infections à prion' (French research ministry), the European Union (QLK3-CT-2002-01309) and the Midi-Pyrénées Region, France.
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Andréoletti, O., Simon, S., Lacroux, C. et al. PrPSc accumulation in myocytes from sheep incubating natural scrapie. Nat Med 10, 591–593 (2004). https://doi.org/10.1038/nm1055
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DOI: https://doi.org/10.1038/nm1055
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