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dc.contributor.authorBarrio, Tomás
dc.contributor.authorFilali, Hicham
dc.contributor.authorOtero, Alicia
dc.contributor.authorSheleby-Elías, Jessica
dc.contributor.authorMarín, Belén
dc.contributor.authorVidal, Enric
dc.contributor.authorBéringue, Vincent
dc.contributor.authorTorres, Juan María
dc.contributor.authorGroschup, Martin
dc.contributor.authorAndréoletti, Olivier
dc.contributor.authorBadiola, Juan José
dc.contributor.authorBolea, Rosa
dc.contributor.otherProducció Animalca
dc.date.accessioned2021-02-17T08:18:05Z
dc.date.available2021-02-17T08:18:05Z
dc.date.issued2020-03-19
dc.identifier.citationBarrio, Tomás, Hicham Filali, Alicia Otero, Jessica Sheleby-Elías, Belén Marín, Enric Vidal, and Vincent Béringue et al. 2020. "Mixtures Of Prion Substrains In Natural Scrapie Cases Revealed By Ovinised Murine Models". Scientific Reports 10 (1). doi:10.1038/s41598-020-61977-1.ca
dc.identifier.issn2045-2322ca
dc.identifier.urihttp://hdl.handle.net/20.500.12327/1094
dc.description.abstractPhenotypic variability in prion diseases, such as scrapie, is associated to the existence of prion strains, which are different pathogenic prion protein (PrPSc) conformations with distinct pathobiological properties. To faithfully study scrapie strain variability in natural sheep isolates, transgenic mice expressing sheep cellular prion protein (PrPC) 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 (PrPres) banding patterns, lesion profiles and PrPSc distribution were studied. Inocula showed a remarkable homogeneity on banding patterns, all of them but one showing 19-kDa PrPres. However, a number of isolates caused accumulation of 21-kDa PrPres in TgShp XI. A different subgroup of isolates caused long survival periods and presence of 21-kDa PrPres 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 PrPC 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.ca
dc.format.extent15ca
dc.language.isoengca
dc.publisherNature Researchca
dc.relation.ispartofScientific Reportsca
dc.rightsAttribution 4.0 Internationalca
dc.rights.urihttp://creativecommons.org/licenses/by/4.0/*
dc.titleMixtures of prion substrains in natural scrapie cases revealed by ovinised murine modelsca
dc.typeinfo:eu-repo/semantics/articleca
dc.description.versioninfo:eu-repo/semantics/publishedVersionca
dc.rights.accessLevelinfo:eu-repo/semantics/openAccess
dc.embargo.termscapca
dc.relation.projectIDMINECO/Programa Estatal de I + D + I orientada a los retos de la sociedad/AGL2015-65560-R/ES/Implicación de la glicosilación en la transmisión de las enfermedades priónicas, biomarcadores moleculares y papel de las neurotrofinas en la neurodegeneración/ca
dc.relation.projectIDEC/INTERREG-POCTEFA/EU/EFA-148-16/ /REDPRIONca
dc.subject.udc619ca
dc.identifier.doihttps://doi.org/10.1038/s41598-020-61977-1ca
dc.contributor.groupSanitat Animalca


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