Table 2 Mitochondrial Respiratory Chain Activities Subject cI/CS a cII/CS a cIII/CS a cIV/CS a CS b Muscle biopsy S1 142 44∗ 91 20∗ 90 S2 47∗ 11∗ 32∗ 3∗ 374∗ S3 140 69 93 5∗ 119 S4 58 100 42∗ 8∗ 29∗ S6 127 81 132 36∗ 100 Skin biopsy S1 128 58 105 61 101 S2 56 54∗ 82 50∗ 147 S4 58 98 104 25∗ 150∗ The analyses were performed in different laboratories, and the reference values are diverse (usually ranging between 60% and 150% of the mean control value). The values out of the control range (specific for each enzymatic activity and for each laboratory) are indicated with an asterisk (∗). a Percent of mean control value of MRC complexes/citrate synthase (CS) activities. b Percent of mean control value.