Ontology highlight
ABSTRACT:
SUBMITTER: Yano H
PROVIDER: S-EPMC4174557 | biostudies-literature | 2014 Jun
REPOSITORIES: biostudies-literature
Yano Hiroko H Baranov Sergei V SV Baranova Oxana V OV Kim Jinho J Pan Yanchun Y Yablonska Svitlana S Carlisle Diane L DL Ferrante Robert J RJ Kim Albert H AH Friedlander Robert M RM
Nature neuroscience 20140518 6
Mitochondrial dysfunction is associated with neuronal loss in Huntington's disease (HD), a neurodegenerative disease caused by an abnormal polyglutamine expansion in huntingtin (Htt). However, the mechanisms linking mutant Htt and mitochondrial dysfunction in HD remain unknown. We identify an interaction between mutant Htt and the TIM23 mitochondrial protein import complex. Remarkably, recombinant mutant Htt directly inhibited mitochondrial protein import in vitro. Furthermore, mitochondria from ...[more]