Multiple cytochrome deficiency and deteriorated mitochondrial polypeptide composition in fatal infantile mitochondrial myopathy and renal dysfunction |
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| Authors: | M Tanaka M Nishikimi H Suzuki T Ozawa E Okino H Takahashi |
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| Affiliation: | 1. Memorial University of Newfoundland, St. John''s, Newfoundland A1B 3X9, Canada;2. Fisheries, Oceans and the Canadian Coast Guard, Institute of Ocean Sciences, Sidney, British Columbia V8L 4B2, Canada;3. Environment Canada, Canada Centre for Inland Waters, 867 Lakeshore Road, Burlington, Ontario L7R 4A6, Canada;4. Environment and Climate Change Canada, National Wildlife Research Centre, Carleton University, Raven Road, Ottawa, Ontario K1A 0H3, Canada;5. Centre for Earth Observation Science, Department of Environment and Geography, University of Manitoba, Winnipeg R3T 2N2, Canada |
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| Abstract: | Mitochondria isolated from the skeletal muscle of an infant with mitochondrial myopathy and renal dysfunction were analyzed. Activities of NADH dehydrogenase, succinate dehydrogenase, ubiquinol-cytochrome c oxidoreductase, and cytochrome c oxidase were severely decreased. Cytochromes aa3 and b were not detected in patient mitochondria, and the cytochrome c+c1 content was 14% of control. Immunoblotting demonstrated that the amount of cytochrome c oxidase subunits were markedly decreased in patient mitochondria. The polypeptide profile of patient mitochondria was quite different from that of control mitochondria. These results suggest that deterioration of mitochondria in a severe case of mitochondrial myopathy involves not only cytochrome c oxidase but also other mitochondrial proteins. |
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