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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.

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    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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