Table 1.
Mitochondrial respiratory chain enzyme analysis
| Complex | Electron transport chain activity assay | Patient (nmol/min/mg) | Patient (normalized values) | Control (+/– SD) (nmol/min/mg)1 |
|---|---|---|---|---|
| I | NADH : ferricyanide dehydrogenase | 548 | 154% | 280 +/– 91 |
| I + III | NADH : cytochrome C reductase (total) | 34.2 | 86% | 28.2 +/– 4.3 |
| NADH : cytochrome C reductase (rotenone sensitive) | 5.3 | 46% | 9.1 +/– 2.5 | |
| II | Succinate dehydrogenase | 12.5 | 122% | 8.1 +/–2.44 |
| II + III | Succinate : cytochrome C reductase | 3.19 | 51% | 4.9 +/– 1.1 |
| IV | Sytochrome C oxidase | 45.2 | 122% | 29.2 +/– 9.1 |
| None | Citrate synthase (mitochondrial content marker) | 355 | 100% | 280 +/– 95 |
1Control values for quadriceps femoris (control values for paraspinal muscle are not available).