학술논문

Mitochondrial cytochrome c release: a factor to consider in mitochondrial disease?
Document Type
Report
Source
Journal of Inherited Metabolic Disease. April, 2009, Vol. 32 Issue 2, p269, 5 p.
Subject
Enzymes -- Physiological aspects
Mitochondrial DNA -- Physiological aspects
Cytochrome c -- Physiological aspects
Language
English
ISSN
0141-8955
Abstract
The pathogenesis of mitochondrial disorders has largely focused on the impairment of cellular energy metabolism. However, mitochondrial dysfunction has also been implicated as a factor in the initiation of apoptosis due to the translocation of cytochrome c, from mitochondria to the cytosol, and the subsequent cleavage of pro-caspase 3. In this study, we determined the cytochrome c content of cytosols (skeletal muscle) prepared from 22 patients with evidence of compromised mitochondrial electron transport chain enzyme activity and 26 disease controls. The cytochrome c content of the mitochondrial electron transport chain-deficient group was found to be significantly (p