Selective processing and metabolism of disease-causing mutant prion proteins.

Prion diseases are fatal neurodegenerative disorders caused by aberrant metabolism of the cellular prion protein (PrP(C)). In genetic forms of these diseases, mutations in the globular C-terminal domain are hypothesized to favor the spontaneous generation of misfolded PrP conformers (including the t...

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Bibliographic Details
Main Authors: Aarthi Ashok, Ramanujan S Hegde
Format: Article
Language:English
Published: Public Library of Science (PLoS) 2009-06-01
Series:PLoS Pathogens
Online Access:http://europepmc.org/articles/PMC2691595?pdf=render