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Article

Familial prion protein mutants inhibit Hrd1-mediated retrotranslocation of misfolded proteins by depleting misfolded protein sensor BiP.

Human molecular genetics - 1 Mar 2016

Peters Sarah L, Déry Marc-André, LeBlanc Andrea C

Abstract excerpt

Similar to many proteins trafficking through the secretory pathway, cellular prion protein (PrP) partly retrotranslocates from the endoplasmic reticulum to the cytosol through the endoplasmic reticulum-associated degradation (ERAD) pathway in an attempt to alleviate accumulation of cellular misfolded PrP. Surprisingly, familial PrP mutants fail to retrotranslocate and simultaneously block normal cellular PrP...

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Identifiers

PMID: 26740554
DOI: 10.1093/hmg/ddv630
PMCID: PMC4754050

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TTR

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