A novel Gerstmann-Sträussler-Scheinker disease mutation defines a precursor for amyloidogenic 8 kDa PrP fragments and reveals N-terminal structural changes shared by other GSS alleles.

A novel Gerstmann-Sträussler-Scheinker disease mutation defines a precursor for amyloidogenic 8 kDa PrP fragments and reveals N-terminal structural changes shared by other GSS alleles.

To explore pathogenesis in a young Gerstmann-Sträussler-Scheinker Disease (GSS) patient, the corresponding mutation, an eight-residue duplication in the hydrophobic region (HR), was inserted into the wild type mouse PrP gene. Transgenic (Tg) mouse lines expressing this mutation (Tg.HRdup) developed...

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Bibliographic Details
Main Authors: Robert C C Mercer, Nathalie Daude, Lyudmyla Dorosh, Ze-Lin Fu, Charles E Mays, Hristina Gapeshina, Serene L Wohlgemuth, Claudia Y Acevedo-Morantes, Jing Yang, Neil R Cashman, Michael B Coulthart, Dawn M Pearson, Jeffrey T Joseph, Holger Wille, Jiri G Safar, Gerard H Jansen, Maria Stepanova, Brian D Sykes, David Westaway
Format: Article
Language:English
Published: Public Library of Science (PLoS) 2018-01-01
Series:PLoS Pathogens
Online Access:http://europepmc.org/articles/PMC5786331?pdf=render

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http://europepmc.org/articles/PMC5786331?pdf=render

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