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http://hdl.handle.net/2445/134068
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 Prions Malalties per prions Ratolins transgènics Medical screening Prions 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: https://doi.org/10.3201/eid1912.130106 |
It is part of: | Emerging Infectious Diseases, 2013, vol. 19, num. 12, p. 1938-1947 |
URI: | http://hdl.handle.net/2445/134068 |
Related resource: | https://doi.org/10.3201/eid1912.130106 |
ISSN: | 1080-6040 |
Appears in Collections: | Articles publicats en revistes (Patologia i Terapèutica Experimental) |
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