Article
Generation of a new infectious recombinant prion: a model to understand Gerstmann-Sträussler-Scheinker syndrome.
Scientific reports - 29 Aug 2017
Elezgarai Saioa R, Fernández-Borges Natalia, Eraña Hasier, Sevillano Alejandro M, Charco Jorge M, Harrathi Chafik, Saá Paula, Gil David, Kong Qingzhong, Requena Jesús R, Andréoletti Olivier, Castilla Joaquín
Abstract excerpt
Human transmissible spongiform encephalopathies (TSEs) or prion diseases are a group of fatal neurodegenerative disorders that include Kuru, Creutzfeldt-Jakob disease, Gerstmann-Sträussler-Scheinker syndrome (GSS), and fatal familial insomnia. GSS is a genetically determined TSE caused by a range of mutations within the prion protein (PrP) gene. Several animal models, based on the expression of PrPs carrying...
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