Derlin-1 regulates mutant VCP-linked pathogenesis and endoplasmic reticulum stress-induced apoptosis.

Mutations in VCP (Valosin-containing protein), an AAA ATPase critical for ER-associated degradation, are linked to IBMPFD (Inclusion body myopathy with Paget disease and frontotemporal dementia). Using a Drosophila IBMPFD model, we have identified the ER protein Derlin-1 as a modifier of pathogenic...

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Main Authors: Cyong-Jhih Liang, Ya-Chu Chang, Henry C Chang, Chung-Kang Wang, Yu-Chien Hung, Ying-Er Lin, Chia-Ching Chan, Chun-Hong Chen, Hui-Yun Chang, Tzu-Kang Sang
Format: Article
Language:English
Published: Public Library of Science (PLoS) 2014-09-01
Series:PLoS Genetics
Online Access:http://europepmc.org/articles/PMC4177747?pdf=render