Ontology highlight
ABSTRACT:
SUBMITTER: Yang H
PROVIDER: S-EPMC2811200 | biostudies-other | 2010 Jan
REPOSITORIES: biostudies-other
Yang Hui H Liu Chao C Zhong Yongwang Y Luo Shouqing S Monteiro Mervyn J MJ Fang Shengyun S
PloS one 20100126 1
Huntington's disease (HD) is caused by polyglutamine expansion in huntingtin (htt) protein, but the exact mechanism of HD pathogenesis remains uncertain. Recent evidence suggests that htt proteins with expanded polyglutamine tracts induce endoplasmic reticulum (ER) stress, probably by interfering with ER-associated degradation (ERAD). Here we report that mutant htt interacts and interferes with the function of gp78, an ER membrane-anchored ubiquitin ligase (E3) involved in ERAD. Mapping studies ...[more]