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Mech Ageing Dev. 2013 May-Jun;134(5-6):275-83. doi: 10.1016/j.mad.2013.02.007. Epub 2013 Feb 19.

Mitochondrial deficiency in Cockayne syndrome.

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1
Laboratory of Molecular Gerontology, National Institute on Aging, NIH, USA.

Abstract

Cockayne syndrome is a rare inherited disorder characterized by accelerated aging, cachectic dwarfism and many other features. Recent work has implicated mitochondrial dysfunction in the pathogenesis of this disease. This is particularly interesting since mitochondrial deficiencies are believed to be important in the aging process. In this review, we discuss recent findings of mitochondrial pathology in Cockayne syndrome and suggest possible mechanisms for the mitochondrial dysfunction.

PMID:
23435289
PMCID:
PMC3663877
DOI:
10.1016/j.mad.2013.02.007
[Indexed for MEDLINE]
Free PMC Article
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