Article
Gerstmann-Sträussler-Scheinker Disease with F198S Mutation Induces Independent Tau and Prion Protein Pathologies in Bank Voles.
Biomolecules - 21 Oct 2022
Bruno Rosalia, Pirisinu Laura, Riccardi Geraldina, D'Agostino Claudia, De Cecco Elena, Legname Giuseppe, Cardone Franco, Gambetti Pierluigi, Nonno Romolo, Agrimi Umberto, Di Bari Michele Angelo
Abstract excerpt
Gerstmann-Sträussler-Scheinker disease (GSS) is a rare genetic prion disease. A large GSS kindred linked to the serine-for-phenylalanine substitution at codon 198 of the prion protein gene (GSS-F198S) is characterized by conspicuous accumulation of prion protein (PrP)-amyloid deposits and neurofibrillary tangles. Recently, we demonstrated the transmissibility of GSS-F198S prions to bank vole carrying isoleucine...
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