Document Detail


Partial deficiency of subunits in complex I or IV of patients with mitochondrial myopathies.
MedLine Citation:
PMID:  2884999     Owner:  NLM     Status:  MEDLINE    
Abstract/OtherAbstract:
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.
Authors:
M Tanaka; M Nishikimi; H Suzuki; T Ozawa; Y Koga; I Nonaka
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Publication Detail:
Type:  Journal Article; Research Support, Non-U.S. Gov't    
Journal Detail:
Title:  Biochemistry international     Volume:  14     ISSN:  0158-5231     ISO Abbreviation:  Biochem. Int.     Publication Date:  1987 Mar 
Date Detail:
Created Date:  1987-07-08     Completed Date:  1987-07-08     Revised Date:  2007-11-15    
Medline Journal Info:
Nlm Unique ID:  8100311     Medline TA:  Biochem Int     Country:  AUSTRALIA    
Other Details:
Languages:  eng     Pagination:  525-30     Citation Subset:  IM    
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MeSH Terms
Descriptor/Qualifier:
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:
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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