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Transmission Properties of Human PrP 102L Prions Challenge the Relevance of Mouse Models of GSS

Inherited prion disease (IPD) is caused by autosomal-dominant pathogenic mutations in the human prion protein (PrP) gene (PRNP). A proline to leucine substitution at PrP residue 102 (P102L) is classically associated with Gerstmann-Sträussler-Scheinker (GSS) disease but shows marked clinical and neur...

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Detalles Bibliográficos
Autores principales:	Asante, Emmanuel A., Grimshaw, Andrew, Smidak, Michelle, Jakubcova, Tatiana, Tomlinson, Andrew, Jeelani, Asif, Hamdan, Shyma, Powell, Caroline, Joiner, Susan, Linehan, Jacqueline M., Brandner, Sebastian, Wadsworth, Jonathan D. F., Collinge, John
Formato:	Online Artículo Texto
Lenguaje:	English
Publicado:	Public Library of Science 2015
Materias:	Research Article
Acceso en línea:	https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4489887/ https://www.ncbi.nlm.nih.gov/pubmed/26135918 http://dx.doi.org/10.1371/journal.ppat.1004953

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