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

被引：131

作者：

Costa, Veronica ^{[1
]}

Scorrano, Luca ^{[1
]}

机构：

[1] Univ Geneva, Dept Cell Physiol & Med, CH-1206 Geneva, Switzerland

来源：

EMBO JOURNAL | 2012年 / 31卷 / 08期

关键词：

fusion-fission; Huntington's disease; mitochondria; ultrastructure; DYNAMIN-RELATED PROTEIN-1; TRANSCRIPTIONAL COACTIVATOR PGC-1; OUTER-MEMBRANE PERMEABILIZATION; TRANSGENIC MOUSE MODEL; DOMINANT OPTIC ATROPHY; CYTOCHROME-C RELEASE; MUTANT HUNTINGTIN; CELL-DEATH; ENERGY-METABOLISM; IN-VIVO;

D O I：

10.1038/emboj.2012.65

中图分类号：

Q5 [生物化学]; Q7 [分子生物学];

学科分类号：

070307 [化学生物学]; 071010 [生物化学与分子生物学];

摘要：

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. The EMBO Journal (2012) 31, 1853-1864. doi:10.1038/emboj.2012.65; Published online 23 March 2012 Subject Categories: neuroscience; molecular biology of disease

引用

页码：1853 / 1864

页数：12

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