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

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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Main Authors: Emmanuel A Asante, Andrew Grimshaw, Michelle Smidak, Tatiana Jakubcova, Andrew Tomlinson, Asif Jeelani, Shyma Hamdan, Caroline Powell, Susan Joiner, Jacqueline M Linehan, Sebastian Brandner, Jonathan D F Wadsworth, John Collinge
Format: Article
Language:English
Published: Public Library of Science (PLoS) 2015-07-01
Series:PLoS Pathogens
Online Access:https://doi.org/10.1371/journal.ppat.1004953
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https://doi.org/10.1371/journal.ppat.1004953

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