Transmission Properties of Human PrP 102L Prions Challenge the Relevance of Mouse Models of GSS: e1004953

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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Veröffentlicht in:	PLoS pathogens 2015-07, Vol.11 (7)
Hauptverfasser:	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 DF, Collinge, John
Format:	Artikel
Sprache:	eng
Schlagworte:	Biomedical research Brain research Creutzfeldt-Jakob disease Funding Hypotheses Immunoglobulins Mutation Prions Proteins Rodents Transgenic animals
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