Mixtures of prion substrains in natural scrapie cases revealed by ovinised murine models
Barrio, Tomás (Universidad de Zaragoza. Centro de Encefalopatías y Enfermedades Transmisibles Emergentes)
Filali, Hicham (Universidad de Zaragoza. Centro de Encefalopatías y Enfermedades Transmisibles Emergentes)
Otero García, Alicia (Universidad de Zaragoza. Centro de Encefalopatías y Enfermedades Transmisibles Emergentes)
Sheleby-Elías, Jessica (Universidad de Zaragoza. Centro de Encefalopatías y Enfermedades Transmisibles Emergentes)
Marín, Belén (Universidad de Zaragoza. Centro de Encefalopatías y Enfermedades Transmisibles Emergentes)
Vidal Barba, Enric (Institut de Recerca i Tecnologia Agroalimentàries. Centre de Recerca en Sanitat Animal)
Béringue, Vincent (Université Paris-Saclay. Virologie Immunologie Moléculaires)
Torres, Juan María (Centro de Investigación en Sanidad Animal. CISA (Madrid, Espanya))
Groschup, Martin (Friedrich-Loeffler-Institute. Institute of Novel and Emerging Infectious Diseases)
Andreoletti, Olivier (École Nationale Vétérinaire de Toulouse)
Badiola, Juan José (Universidad de Zaragoza. Centro de Encefalopatías y Enfermedades Transmisibles Emergentes)
Bolea, Rosa (Universidad de Zaragoza. Centro de Encefalopatías y Enfermedades Transmisibles Emergentes)

Data:	2020
Resum:	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.
Drets:	Aquest document està subjecte a una llicència d'ús Creative Commons. Es permet la reproducció total o parcial, la distribució, la comunicació pública de l'obra i la creació d'obres derivades, fins i tot amb finalitats comercials, sempre i quan es reconegui l'autoria de l'obra original.
Llengua:	Anglès
Document:	Article ; recerca ; Versió publicada
Matèria:	Neurodegeneration ; Prion diseases
Publicat a:	Scientific reports, Vol. 10 (march 2020) , ISSN 2045-2322

DOI: 10.1038/s41598-020-61977-1
PMID: 32193445

15 p, 4.2 MB

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Documents de recerca > Documents dels grups de recerca de la UAB > Centres i grups de recerca (producció científica) > Ciències de la salut i biociències > Centre de Recerca en Sanitat Animal (CReSA-IRTA)
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