Ontology highlight
ABSTRACT:
SUBMITTER: Liang CJ
PROVIDER: S-EPMC4177747 | biostudies-literature | 2014 Sep
REPOSITORIES: biostudies-literature
Liang Cyong-Jhih CJ Chang Ya-Chu YC Chang Henry C HC Wang Chung-Kang CK Hung Yu-Chien YC Lin Ying-Er YE Chan Chia-Ching CC Chen Chun-Hong CH Chang Hui-Yun HY Sang Tzu-Kang TK
PLoS genetics 20140925 9
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 TER94 (the fly VCP homolog) mutants. Derlin-1 binds to TER94 directly, and this interaction is essential for Derlin-1 overexpression to suppress the pathogenic TER94-induced neurodegeneration. Derlin- ...[more]