Table 3

Respiratory Chain Complex Activities in Skeletal Muscle Mitochondria and Cardiac (Left Ventricle) Mitochondria From the Index Case (Patient IV-03) of Family 1

ComplexSkeletal MuscleCardiac Muscle
Controls (n = 15)PatientsControls (n = 8)Patients
Complex I/CS0.240 ± 0.0600.3170.254 ± 0.0740.015
Complex II/CS0.320 ± 0.0880.4130.281 ± 0.0360.196
Complex IV/CS1.34 ± 0.391.482.34 ± 0.540.059
Complex I/II0.74 ± 0.120.770.88 ± 0.180.077
Complex IV/II4.31 ± 1.363.578.08 ± 1.900.302

Control data are presented as the mean value ± SD. Enzyme activities are expressed as nmol NADH oxidized min/U citrate synthase (CS) for complex I; nmol 2,6-dichlorophenol-indophenol reduced min/U citrate synthase for complex II (succinate:ubiquinone-1 reductase); and the apparent first-order rate constant.s/U citrate synthase for complex IV (×103).