Impaired mitochondrial trafficking in Huntington's disease.

Impaired mitochondrial trafficking in Huntington's disease.
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DOI:
10.1016/j.bbadis.2009.06.008
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发表时间:
2010-01
影响因子:
6.2
通讯作者:
Li, Shihua
Li, Shihua
中科院分区:
生物学2区
文献类型:
--
作者:
Li, Xiao-Jiang;Orr, Adam L.;Li, Shihua

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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 mitochondria 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.
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