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Single muscle fibre analyses in 2 brothers with succinate dehydrogenase deficiency
1Department of Neurology, University of Würzburg, FRG.
European Neurology
|January 1, 1994
Abstract:
We report 2 brothers with hypertrophic cardiomyopathy, lipid storage and skeletal mitochondrial myopathy. Biochemical analyses detected complex II (succinate dehydrogenase) deficiency of the respiratory chain. Single fiber analyses using a microphotometer were performed and revealed reduced succinate dehydrogenase activity in all muscle fibers. This report shows the validity of kinetic microphotometric analysis in revealing complex II defects.