Article
Spontaneous generation of infectious prion disease in transgenic mice.
Emerging infectious diseases - 1 Dec 2013
Torres Juan-María, Castilla Joaquín, Pintado Belén, Gutiérrez-Adan Alfonso, Andréoletti Olivier, Aguilar-Calvo Patricia, Arroba Ana-Isabel, Parra-Arrondo Beatriz, Ferrer Isidro, Manzanares Jorge, Espinosa Juan-Carlos
Abstract excerpt
We generated transgenic mice expressing bovine cellular prion protein (PrP(C)) with a leucine substitution at codon 113 (113L). This protein is homologous to human protein with mutation 102L, and its genetic link with Gerstmann-Sträussler-Scheinker syndrome has been established. This mutation in bovine PrP(C) causes a fully penetrant, lethal, spongiform encephalopathy. This genetic disease was transmitted by...
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