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- W3011012803 abstract "Abstract Phenotypic variability in prion diseases, such as scrapie, is associated to the existence of prion strains, which are different pathogenic prion protein (PrP Sc ) conformations with distinct pathobiological properties. To faithfully study scrapie strain variability in natural sheep isolates, transgenic mice expressing sheep cellular prion protein (PrP C ) are used. In this study, we used two of such models to bioassay 20 scrapie isolates from the Spain-France-Andorra transboundary territory. Animals were intracerebrally inoculated and survival periods, proteinase K-resistant PrP (PrP res ) banding patterns, lesion profiles and PrP Sc distribution were studied. Inocula showed a remarkable homogeneity on banding patterns, all of them but one showing 19-kDa PrP res . However, a number of isolates caused accumulation of 21-kDa PrP res in TgShp XI. A different subgroup of isolates caused long survival periods and presence of 21-kDa PrP res in Tg338 mice. It seemed that one major 19-kDa prion isoform and two distinct 21-kDa variants coexisted in source inocula, and that they could be separated by bioassay in each transgenic model. The reason why each model favours a specific component of the mixture is unknown, although PrP C expression level may play a role. Our results indicate that coinfection with more than one substrain is more frequent than infection with a single component." @default.
- W3011012803 created "2020-03-23" @default.
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- W3011012803 date "2020-03-19" @default.
- W3011012803 modified "2023-10-18" @default.
- W3011012803 title "Mixtures of prion substrains in natural scrapie cases revealed by ovinised murine models" @default.
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- W3011012803 doi "https://doi.org/10.1038/s41598-020-61977-1" @default.
- W3011012803 hasPubMedCentralId "https://www.ncbi.nlm.nih.gov/pmc/articles/7081250" @default.
- W3011012803 hasPubMedId "https://pubmed.ncbi.nlm.nih.gov/32193445" @default.
- W3011012803 hasPublicationYear "2020" @default.
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