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Mixtures of prion substrains in natural scrapie cases revealed by ovinised murine models

Barrio, Tomás ; Filali, Hicham ; Otero, Alicia ; Sheleby-Elías, Jessica ; Marín, Belén ; Vidal, Enric ; Béringue, Vincent ; Torres, Juan María ; Groschup, Martin ; Andréoletti, Olivier ; Badiola, Juan José ; Bolea, Rosa

Scientific reports, 2020-03, Vol.10 (1), p.5042-5042, Article 5042 [Periódico revisado por pares]

England: Nature Publishing Group

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Título:
Mixtures of prion substrains in natural scrapie cases revealed by ovinised murine models
Autor: Barrio, Tomás ; Filali, Hicham ; Otero, Alicia ; Sheleby-Elías, Jessica ; Marín, Belén ; Vidal, Enric ; Béringue, Vincent ; Torres, Juan María ; Groschup, Martin ; Andréoletti, Olivier ; Badiola, Juan José ; Bolea, Rosa
Assuntos: Animal models ; Animals ; Bioassays ; Brain - metabolism ; Cattle ; Disease Models, Animal ; Endopeptidase K ; France ; Genetic variability ; Life Sciences ; Mice, Transgenic ; Prion protein ; Prion Proteins - metabolism ; Proteinase ; PrPSc Proteins - metabolism ; PrPSc Proteins - pathogenicity ; Scrapie ; Scrapie - etiology ; Scrapie - metabolism ; Scrapie - pathology ; Scrapie - prevention & control ; Sheep ; Spain ; Transgenic mice
É parte de: Scientific reports, 2020-03, Vol.10 (1), p.5042-5042, Article 5042
Notas: ObjectType-Article-1
SourceType-Scholarly Journals-1
ObjectType-Feature-2
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Descrição: Phenotypic variability in prion diseases, such as scrapie, is associated to the existence of prion strains, which are different pathogenic prion protein (PrP ) conformations with distinct pathobiological properties. To faithfully study scrapie strain variability in natural sheep isolates, transgenic mice expressing sheep cellular prion protein (PrP ) 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 ) banding patterns, lesion profiles and PrP distribution were studied. Inocula showed a remarkable homogeneity on banding patterns, all of them but one showing 19-kDa PrP . However, a number of isolates caused accumulation of 21-kDa PrP in TgShp XI. A different subgroup of isolates caused long survival periods and presence of 21-kDa PrP 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 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.
Editor: England: Nature Publishing Group
Idioma: Inglês

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Mixtures of prion substrains in natural scrapie cases revealed by ovinised murine models

Barrio, Tomás ; Filali, Hicham ; Otero, Alicia ; Sheleby-Elías, Jessica ; Marín, Belén ; Vidal, Enric ; Béringue, Vincent ; Torres, Juan María ; Groschup, Martin ; Andréoletti, Olivier ; Badiola, Juan José ; Bolea, Rosa

England: Nature Publishing Group

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