| Partial deficiency of subunits in complex I or IV of patients with mitochondrial myopathies. | |
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MedLine Citation:
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PMID: 2884999 Owner: NLM Status: MEDLINE |
Abstract/OtherAbstract:
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Subunits of Complexes I, III, IV, and V from skeletal muscle mitochondria isolated from four patients with mitochondrial myopathies were analyzed by immunoblotting. A deficiency was observed in Complex I or IV, or in both. The patterns of deficiency of subunits of each complex were similar both in patients with deficiency of a single complex and in patients with combined deficiency of the two complexes. The deficiency of any of these subunits was only partial, and no abnormalities in the electrophoretic mobilities of the subunits were observed. These results suggest that the deficiency of Complex I or IV in these patients are based on similar molecular defects which lead to decreased levels of these subunits. |
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Authors:
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M Tanaka; M Nishikimi; H Suzuki; T Ozawa; Y Koga; I Nonaka |
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Publication Detail:
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Type: Journal Article; Research Support, Non-U.S. Gov't |
Journal Detail:
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Title: Biochemistry international Volume: 14 ISSN: 0158-5231 ISO Abbreviation: Biochem. Int. Publication Date: 1987 Mar |
Date Detail:
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Created Date: 1987-07-08 Completed Date: 1987-07-08 Revised Date: 2007-11-15 |
Medline Journal Info:
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Nlm Unique ID: 8100311 Medline TA: Biochem Int Country: AUSTRALIA |
Other Details:
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Languages: eng Pagination: 525-30 Citation Subset: IM |
Export Citation:
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APA/MLA Format Download EndNote Download BibTex |
| MeSH Terms | |
Descriptor/Qualifier:
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Adolescent Child Cytochrome-c Oxidase Deficiency* Electron Transport Complex II Electron Transport Complex IV / metabolism Female Humans Infant Macromolecular Substances Male Mitochondria, Muscle / enzymology* Multienzyme Complexes / metabolism Muscular Diseases / enzymology* NAD(P)H Dehydrogenase (Quinone) Oxidoreductases / metabolism Proton-Translocating ATPases / metabolism Quinone Reductases / deficiency*, metabolism Succinate Dehydrogenase / metabolism |
| Chemical | |
Reg. No./Substance:
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0/Macromolecular Substances; 0/Multienzyme Complexes; EC 1.-/Oxidoreductases; EC 1.3.5.1/Electron Transport Complex II; EC 1.3.99.1/Succinate Dehydrogenase; EC 1.6.5.2/NAD(P)H Dehydrogenase (Quinone); EC 1.6.99.-/Quinone Reductases; EC 1.9.3.1/Electron Transport Complex IV; EC 3.6.3.14/Proton-Translocating ATPases |
From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine
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