Transgenic fatal familial insomnia mice indicate prion infectivity-independent mechanisms of pathogenesis and phenotypic expression of disease.

Transgenic fatal familial insomnia mice indicate prion infectivity-independent mechanisms of pathogenesis and phenotypic expression of disease.

Fatal familial insomnia (FFI) and a genetic form of Creutzfeldt-Jakob disease (CJD178) are clinically different prion disorders linked to the D178N prion protein (PrP) mutation. The disease phenotype is determined by the 129 M/V polymorphism on the mutant allele, which is thought to influence D178N...

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Main Authors: Ihssane Bouybayoune, Susanna Mantovani, Federico Del Gallo, Ilaria Bertani, Elena Restelli, Liliana Comerio, Laura Tapella, Francesca Baracchi, Natalia Fernández-Borges, Michela Mangieri, Cinzia Bisighini, Galina V Beznoussenko, Alessandra Paladini, Claudia Balducci, Edoardo Micotti, Gianluigi Forloni, Joaquín Castilla, Fabio Fiordaliso, Fabrizio Tagliavini, Luca Imeri, Roberto Chiesa
Format: Article
Language:English
Published: Public Library of Science (PLoS) 2015-04-01
Series:PLoS Pathogens
Online Access:https://doi.org/10.1371/journal.ppat.1004796
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https://doi.org/10.1371/journal.ppat.1004796

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