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 (CJD(178)) 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 D178...

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Publicat a:PLoS Pathog
Autors principals: Bouybayoune, Ihssane, Mantovani, Susanna, Del Gallo, Federico, Bertani, Ilaria, Restelli, Elena, Comerio, Liliana, Tapella, Laura, Baracchi, Francesca, Fernández-Borges, Natalia, Mangieri, Michela, Bisighini, Cinzia, Beznoussenko, Galina V., Paladini, Alessandra, Balducci, Claudia, Micotti, Edoardo, Forloni, Gianluigi, Castilla, Joaquín, Fiordaliso, Fabio, Tagliavini, Fabrizio, Imeri, Luca, Chiesa, Roberto
Format: Artigo
Idioma:Inglês
Publicat: Public Library of Science 2015
Matèries:
Research Article
Accés en línia:https://ncbi.nlm.nih.gov/pmc/articles/PMC4400166/
https://ncbi.nlm.nih.gov/pubmed/25880443
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1371/journal.ppat.1004796
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