Polymorphisms within the prion protein (PrP) gene are associated with scrapie susceptibility. We analysed the PrP genes of 140 Romney Marsh sheep, the dominant breed in New Zealand, a country free from scrapie. We found PrP alleles that are associated with a high susceptibility to scrapie. Sheep with these PrP genotypes would probably succumb to scrapie when born and raised in a scrapie endemic environment. These findings correspond to those obtained in minor breeds from New Zealand. We conclude that scrapie development not only depends on host genetic factors but also requires exogenous factors. Our findings demonstrate the effectiveness of the measures taken by New Zealand to maintain free from scrapie.
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PMCA to demonstrate the efficacy of prion inactivation methods on reusable medical devices: a relevant alternative to animal bioassays.
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Molecular Virology Immunology Unit, Université Paris-Saclay, INRAE, UVSQ, Jouy-en-Josas, France. Electronic address:
Validation of prion inactivation processes for medical devices relies on in-vivo experimental protocols. However, bioassays are costly, long (1-2 years) and ethically disputable. Additionally, results obtained with one prion strain - for example, 263K (hamster-adapted strain originating from sheep scrapie) - cannot be easily extrapolated to relevant human prion strains, further questioning the utility of bioassays.
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Article Synopsis
- Lethal prion diseases are caused by misfolded prion proteins (PrP) that resist breakdown, and the study investigates how molecular chaperones like Grp78 can influence the accumulation of these proteins in different cellular environments.
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