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Transgenic mice recapitulate the phenotypic heterogeneity of genetic prion diseases without developing prion infectivity: Role of intracellular PrP retention in neurotoxicity

Genetic prion diseases are degenerative brain disorders caused by mutations in the gene encoding the prion protein (PrP). Different PrP mutations cause different diseases, including Creutzfeldt-Jakob disease (CJD), Gerstmann-Sträussler-Scheinker (GSS) syndrome and fatal familial insomnia (FFI). The...

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Detalles Bibliográficos
Autores principales: Chiesa, Roberto, Restelli, Elena, Comerio, Liliana, Del Gallo, Federico, Imeri, Luca
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Taylor & Francis 2016
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4981194/
https://www.ncbi.nlm.nih.gov/pubmed/26864450
http://dx.doi.org/10.1080/19336896.2016.1139276