Patient 1 | Patient 2 | Patient 3 | Patient 4 | Patient 5 | Patient 6 | Patient 7 | |
Complex I(NADH-ubiquinone reductase) | 77 [15–135] | 27 [15–135] | – | – | – | 6.4[15–135] | 72 [15–135] |
Complex II(succinate ubiquinone DCPIP reductase) | 366 [34–218] | 87 [34–218] | 95 [70–147] | 51[70–147] | 12 [70–147] | 85 [34–218] | 133 [34–218] |
Complex III(ubiquinol cytochrome creductase) | 1558 [191–789] | 337 [191–789] | 358 [222–596] | 207[222–596] | 60 [222–596] | 252 [191–789] | 110 [191–789] |
Complex IV (cytochrome oxidase) | 2069[236–1315] | 478 [236–1315] | 452 [228–964] | 252 [228–964] | 50[228–964] | 61 [236–1315] | 822 [290–1340] |
Complexes II + III(succinate cytochrome c reductase) | 384 [74–456] | 105 [74–456] | 156 [82–304] | 74[82–304] | 18 [82–304] | 108 [74–456] | 55 [43–368] |
Complexes I + III (NADH cytochrome creductase) | 178 [30–421] | – | 78[149–300] | 79[149–300] | 7 [149–300] | 31 [30–421] | 72 [47–515] |
Complex I defect | Complex I defect | Complexes I ± IV defect | Multiple defect | Multiple defect | Complexes I ± IV defects | Complex III defect |
The values in square brackets are the range of the values of the controls. They are different because the measures were done at different moments for each patients with a set of controls in which the spectrophotometric dosage of the MRC complexes was performed during the same experiment. The unit for the absolute values is nmol/min/mg of proteins. The figures in bold represent abnormal values.