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Impaired mitochondrial trafficking in Huntington's disease.

TitleImpaired mitochondrial trafficking in Huntington's disease.
Publication TypeJournal Article
Year of Publication2010
AuthorsLi X-J, Orr AL, Li S
JournalBiochim Biophys Acta
Volume1802
Issue1
Pagination62-5
Date Published2010 Jan
ISSN0006-3002
KeywordsAnimals, Humans, Huntington Disease, Mitochondria, Mitochondrial Proteins, Models, Biological, Protein Transport
Abstract

Impaired mitochondrial function has been well documented in Huntington's disease. Mutant huntingtin is found to affect mitochondria via various mechanisms including the dysregulation of gene transcription and impairment of mitochondrial function or trafficking. The lengthy and highly branched neuronal processes constitute complex neural networks in which there is a large demand for mitochondria-generated energy. Thus, the impaired mitochondrial trafficking in neuronal cells may play an important role in the selective neuropathology of Huntington's disease. Here we discuss the evidence for the effect of the Huntington's disease protein huntingtin on the intracellular trafficking of mitochondria and the involvement of this defective trafficking in the pathogenesis of Huntington's disease.

DOI10.1016/j.bbadis.2009.06.008
Alternate JournalBiochim. Biophys. Acta
PubMed ID19591925
PubMed Central IDPMC2790549
Grant ListR01 AG019206-08 / AG / NIA NIH HHS / United States
R01 NS036232-07 / NS / NINDS NIH HHS / United States
R01 NS041669 / NS / NINDS NIH HHS / United States
NS045016 / NS / NINDS NIH HHS / United States
NS041669 / NS / NINDS NIH HHS / United States
R01 AG019206 / AG / NIA NIH HHS / United States
AG019206 / AG / NIA NIH HHS / United States
R01 NS045016 / NS / NINDS NIH HHS / United States
R01 AG031153 / AG / NIA NIH HHS / United States
R01 NS036232 / NS / NINDS NIH HHS / United States
NS036232 / NS / NINDS NIH HHS / United States
R01 NS041669-08 / NS / NINDS NIH HHS / United States