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Multiple defects of the respiratory chain including complex II in a family with myopathy and encephalopathy.
- Source :
-
Biochemical and biophysical research communications [Biochem Biophys Res Commun] 1989 Sep 15; Vol. 163 (2), pp. 695-700. - Publication Year :
- 1989
-
Abstract
- We report severe deficiency of complex II of the mitochondrial respiratory chain and low activities of complex I and II in skeletal muscle mitochondria from a young woman with progressive muscle weakness and encephalopathy. Defects of complex II have only very rarely been described and this is the first report of decreased immunoreactive subunits associated with severe deficiency of this enzyme.
- Subjects :
- Adolescent
Blotting, Western
Electron Transport
Electron Transport Complex II
Electron Transport Complex III metabolism
Electrophoresis, Polyacrylamide Gel
Female
Humans
Mitochondria, Muscle enzymology
NAD(P)H Dehydrogenase (Quinone)
Oligopeptides antagonists & inhibitors
Quinone Reductases metabolism
Brain Diseases metabolism
Indoles pharmacology
Multienzyme Complexes deficiency
Neuromuscular Diseases metabolism
Oxidoreductases deficiency
Oxygen Consumption
Succinate Dehydrogenase deficiency
Subjects
Details
- Language :
- English
- ISSN :
- 0006-291X
- Volume :
- 163
- Issue :
- 2
- Database :
- MEDLINE
- Journal :
- Biochemical and biophysical research communications
- Publication Type :
- Academic Journal
- Accession number :
- 2551272
- Full Text :
- https://doi.org/10.1016/0006-291x(89)92279-1