title: Mitochondrial deficiency in Cockayne syndrome.
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.
authors: Laboratory of Molecular Gerontology, National Institute on Aging, NIH, USA.
authors: Morten
authors: M
authors: Scheibye-Knudsen
authors: Deborah L
authors: DL
authors: Croteau
authors: Vilhelm A
authors: VA
authors: Bohr
mesh_terms: D000818:Animals; D003057:Cockayne Syndrome / Q000235:genetics / Q000378:metabolism / Q000473:pathology / Q000503:physiopathology; D006801:Humans; D008928:Mitochondria / Q000235:genetics / Q000378:metabolism / Q000473:pathology; D028361:Mitochondrial Diseases / Q000235:genetics / Q000378:metabolism / Q000473:pathology / Q000503:physiopathology