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EMBO J. 2012 Apr 18;31(8):1853-64. doi: 10.1038/emboj.2012.65. Epub 2012 Mar 23.

Shaping the role of mitochondria in the pathogenesis of Huntington's disease.

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1
Department of Cell Physiology and Medicine, University of Geneva, Genève, Switzerland.

Abstract

Intense research on the pathogenesis of Huntington's disease (HD), a genetic neurodegenerative disease caused by a polyglutamine expansion in the Huntingtin (Htt) protein, revealed multiple potential mechanisms, among which mitochondrial alterations had emerged as key determinants of the natural history of the disease. Pharmacological and genetic animal models of mitochondrial dysfunction in the striatum, which is mostly affected in HD corroborated a key role for these organelles in the pathogenesis of the disease. Here, we will give an account of the recent evidence indicating that the mitochondria-shaping machinery is altered in HD models and patients. Since its correction can counteract HD mitochondrial dysfunction and cellular damage, drugs impacting on mitochondrial shape are emerging as a new possibility of treatment for this devastating condition.

PMID:
22446390
PMCID:
PMC3343341
DOI:
10.1038/emboj.2012.65
[Indexed for MEDLINE]
Free PMC Article
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