NMR structure of the human prion protein with the pathological Q212P mutation reveals unique structural features.

Prion diseases are fatal neurodegenerative disorders caused by an aberrant accumulation of the misfolded cellular prion protein (PrP(C)) conformer, denoted as infectious scrapie isoform or PrP(Sc). In inherited human prion diseases, mutations in the open reading frame of the PrP gene (PRNP) are hypo...

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Bibliographic Details
Main Authors: Gregor Ilc, Gabriele Giachin, Mariusz Jaremko, Łukasz Jaremko, Federico Benetti, Janez Plavec, Igor Zhukov, Giuseppe Legname
Format: Article
Language:English
Published: Public Library of Science (PLoS) 2010-01-01
Series:PLoS ONE
Online Access:http://europepmc.org/articles/PMC2908606?pdf=render