Table 1.

Muscle mitochondrial respiratory chain enzyme activity analyses.

Electron transport chain activities	ETC complexes	Percent of mean (before correction for decreased citrate synthase) (%)	Percent of mean (after correction for decreased citrate synthase) (%)	Patient activity (nmol/min/mg protein)	Control ± standard deviation (nmol/min/mg protein)
NADH:cytochrome c reductase (rotenone sensitive)	Complex I+III	0	0	0	9.1 ± 2.5
Succinate dehydrogenase	Complex II	6	40	0.51	8.11 ± 2.44
Succinate:cytochrome c reductase	Complex II+III	18	115	0.87	4.9 ± 1.1
Cytochrome c oxidase	Complex IV	6	39	1.8	29.2 ± 9.1
Citrate synthase		16	100	44	280 ± 95