Studies of respiratory chain compounds and mitochondrial DNA
| Complex | Controls (n = 29) (mean (SD)) | Patient II-8 (case 4) | Patient III-5 (case 6) | Patient III-7 (case 7) | Patient III-10 (case 5) |
|---|---|---|---|---|---|
| Total I | 35 (14) | 13 | 19 | 16 | 12 |
| I (with rotenone) | 24 (13) | 7 | 5 | 10 | — |
| II + III | 22 (7) | 18 | 16 | 15 | — |
| III antim | 127 (33) | 71 | 57 | 57 | 87 |
| IV | 69 (24) | 54 | 39 | 57 | 85 |
| Citrate synthetase (CS) | 140 (71) | 105 | 196 | 144 | 97 |
| III / CS | 1.08 (0.61) | 0.53 | 0.32 | 0.40 | 0.90 |
| IV / CS | 0.68 (0.46) | 0.51 | 0.22 | 0.40 | 0.90 |
| I / CS | 0.21 (0.13) | 0.07 | 0.03 | 0.07 | — |
| IV / II+III | 3.35 (1.36) | 3.60 | 2.44 | 3.17 | — |
| III / II+III | 6.34 (2.56) | 3.94 | 3.56 | 3.17 | — |
| Mitochondrial DNA | — | Normal | Normal | Normal | Normal |
Values in bold were below the lowest control value. Rotenone = rotenone dependent activity; antim = antimycine dependent activity; — = not measured.