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Article

N-terminal mutant huntingtin associates with mitochondria and impairs mitochondrial trafficking.

The Journal of neuroscience : the official journal of the Society for Neuroscience - 12 Mar 2008

Orr Adam L, Li Shihua, Wang Chuan-En, Li He, Wang Jianjun, Rong Juan, Xu Xingshun, Mastroberardino Pier Giorgio, Greenamyre J Timothy, Li Xiao-Jiang

Abstract excerpt

Huntington's disease (HD) is caused by polyglutamine (polyQ) expansion in huntingtin (htt), a large (350 kDa) protein that localizes predominantly to the cytoplasm. Proteolytic cleavage of mutant htt yields polyQ-containing N-terminal fragments that are prone to misfolding and aggregation. Disease progression in HD transgenic models correlates with age-related accumulation of soluble and aggregated forms of...

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Identifiers

PMID: 18337408
DOI: 10.1523/JNEUROSCI.0106-08.2008
PMCID: PMC2652473

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