Article
Inhibition of mitochondrial protein import by mutant huntingtin.
Nature neuroscience - 1 Jun 2014
Yano Hiroko, Baranov Sergei V, Baranova Oxana V, Kim Jinho, Pan Yanchun, Yablonska Svitlana, Carlisle Diane L, Ferrante Robert J, Kim Albert H, Friedlander Robert M
Abstract excerpt
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...
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