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Biochim Biophys Acta. 2010 Jan;1802(1):62-5. doi: 10.1016/j.bbadis.2009.06.008. Epub 2009 Jul 8.

Impaired mitochondrial trafficking in Huntington's disease.

Author information

1
Department of Human Genetics, Emory University School of Medicine, Atlanta, Georgia 30322, USA. xli2@emory.edu

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.

PMID:
19591925
PMCID:
PMC2790549
DOI:
10.1016/j.bbadis.2009.06.008
[Indexed for MEDLINE]
Free PMC Article

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