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Title: Spontaneous generation of infectious prion disease in transgenic mice
Author: Torres, Juan Maria
Castilla, Joaquín
Pintado, Belén
Gutiérrez-Adan, Alfonso
Andréoletti, Olivier
Aguilar-Calvo, Patricia
Arroba, Ana-Isabel
Parra-Arrondo, Beatriz
Ferrer, Isidro (Ferrer Abizanda)
Manzanares, Jorge
Espinosa, Juan Carlos
Keywords: Cribratge
Malalties per prions
Ratolins transgènics
Medical screening
Prion diseases
Transgenic mice
Issue Date: Dec-2013
Publisher: Centers for Disease Control and Prevention
Abstract: 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 intracerebral inoculation of brain homogenate from ill mice expressing mutant bovine PrP to mice expressing wild-type bovine PrP, which indicated de novo generation of infectious prions. Our findings demonstrate that a single amino acid change in the PrP(C) sequence can induce spontaneous generation of an infectious prion disease that differs from all others identified in hosts expressing the same PrP(C) sequence. These observations support the view that a variety of infectious prion strains might spontaneously emerge in hosts displaying random genetic PrP(C) mutations.
Note: Reproducció del document publicat a:
It is part of: Emerging Infectious Diseases, 2013, vol. 19, num. 12, p. 1938-1947
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ISSN: 1080-6040
Appears in Collections:Articles publicats en revistes (Patologia i Terapèutica Experimental)

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