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    The ARR allele is considered to provide a very strong resistance against classical scrapie infection in sheep. In this study, we report the occurrence of clinical transmissible spongiform encephalopathy in ARR/ARR sheep, following their inoculation by the intracerebral route with a classical scrapie isolate. On first passage, the disease displayed an incomplete attack rate transmission, with incubation periods exceeding 6 years. On second passage, the obtained prion did not display better abilities to propagate than the original isolate. These transmission results contrasted with the 100 % attack rate and the short incubation periods observed in ARQ/ARQ sheep challenged with the same isolate. These data confirm that ARR/ARR sheep cannot be considered to be fully resistant to classical scrapie. However, they also support the contention that classical scrapie has a very limited capacity to transmit and adapt to ARR/ARR sheep.


    Caroline Lacroux, Hervé Cassard, Hugh Simmons, Jean Yves Douet, Fabien Corbière, Severine Lugan, Pierette Costes, Naima Aron, Alvina Huor, Cécile Tillier, Francois Schelcher, Olivier Andreoletti. Classical scrapie transmission in ARR/ARR genotype sheep. The Journal of general virology. 2017 Aug;98(8):2200-2204

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    PMID: 28721847

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