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Predicate | Object |
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rdf:type | |
lifeskim:mentions | |
pubmed:issue |
1
|
pubmed:dateCreated |
1991-2-4
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pubmed:abstractText |
Cytochrome oxidase deficiency was detected in the skeletal muscle of a newborn floppy child. There was a significant decrease in the quantity of subunit 5 and 6 of cytochrome oxidase as showed in Western blot with cytochrome oxidase antibody. By contrast, the NADH: cytochrome c oxidoreductase activity was normal. Electron microscopic studies revealed serious distortion in the myofibres with broken Z-bands and disorganized fibers. The relative molecular mass of actin in the myopathic muscle was smaller than in control. The diffuse actin band in Western blot suggested a proteolytic degradation of F-actin in the myopathic muscle. There was also a serious distortion in the mitochondrial structure. Cytochrome oxidase has a direct role in the formation of cristae and mutation in its components may be directly responsible for the abnormal structure.
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pubmed:language |
eng
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pubmed:journal | |
pubmed:citationSubset |
IM
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pubmed:status |
MEDLINE
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pubmed:month |
Nov
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pubmed:issn |
0009-8981
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pubmed:author | |
pubmed:issnType |
Print
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pubmed:day |
15
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pubmed:volume |
192
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pubmed:owner |
NLM
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pubmed:authorsComplete |
Y
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pubmed:pagination |
9-18
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pubmed:dateRevised |
2004-11-17
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pubmed:meshHeading |
pubmed-meshheading:2175684-Blotting, Western,
pubmed-meshheading:2175684-Cell Membrane,
pubmed-meshheading:2175684-Cytochrome-c Oxidase Deficiency,
pubmed-meshheading:2175684-Electrophoresis, Polyacrylamide Gel,
pubmed-meshheading:2175684-Female,
pubmed-meshheading:2175684-Humans,
pubmed-meshheading:2175684-Infant, Newborn,
pubmed-meshheading:2175684-Male,
pubmed-meshheading:2175684-Microscopy, Electron,
pubmed-meshheading:2175684-Mitochondria, Muscle,
pubmed-meshheading:2175684-Myofibrils,
pubmed-meshheading:2175684-Pregnancy
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pubmed:year |
1990
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pubmed:articleTitle |
Cytochrome oxidase deficiency affecting the structure of the myofibre and the shape of mitochondrial cristae membrane.
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pubmed:affiliation |
Department of Biochemistry, University Medical School Szigeti, Hungary.
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pubmed:publicationType |
Journal Article,
Case Reports
|