Journal
VETERINARY RESEARCH
Volume 47, Issue -, Pages -Publisher
BIOMED CENTRAL LTD
DOI: 10.1186/s13567-016-0380-7
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Funding
- Spanish Ministerio de Ciencia e Innovacion [AGL2009-11553-C02-02, AGL2012-37988-C04-04]
- European Union [FOOD-CT-2006-36353, 219235 ERA-NET EMIDA]
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Host prion (PrPC) genotype is a major determinant for the susceptibility to prion diseases. The Q/K-222-PrPC polymorphic variant provides goats and mice with high resistance against classical scrapie and bovine spongiform encephalopathy (BSE); yet its effect against atypical scrapie is unknown. Here, transgenic mice expressing the goat wild-type (wt) or the K-222-PrPC variant were intracerebrally inoculated with several natural cases of atypical scrapie from sheep and goat and their susceptibility to the prion disease was determined. Goat wt and K-222-PrPC transgenic mice were 100% susceptible to all the atypical scrapie isolates, showing similar survival times and almost identical disease phenotypes. The capacity of the K-222-PrPC variant to replicate specifically the atypical scrapie strain as efficiently as the goat wt PrPC, but not the classical scrapie or cattle-BSE as previously reported, further suggests the involvement of concrete areas of the host PrPC in the strain-dependent replication of prions.
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