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Article

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

PLoS pathogens - 1 Jun 2009

Ashok Aarthi, Hegde Ramanujan S

Abstract excerpt

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 transmissible PrP(Sc) form) that trigger downstream pathways leading to neuronal death. A mechanistic understanding...

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Identifiers

PMID: 19543376
DOI: 10.1371/journal.ppat.1000479
PMCID: PMC2691595

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