Table 1. Enzymatic activities of mitochondrial respiratory chain complexes in patient tissues.
| Assaya | Muscle mitochondria | Lymphocyte homogenate | ||
|---|---|---|---|---|
| Patient | Controls, n=50 (range) | Patient | Controls, n=10 (range) | |
| Complex I (NADH-CoQ reductase) | 61 (136%) | 237±81 (56–393) | ND | ND |
| Complex II (succinate dehydrogenase) | 50 (111%) | 206±72 (57–291) | 19 (79%) | 15 (13–26) |
| Complex II+III (succinate cyt. c reductase) | 64 (105%) | 292±105 (65–453) | ND | ND |
| Complex IV (cytochrome c oxidase) | Undetectable | 1413±518 (374–3284) | 26 (17%) | 99 (72–135) |
| Citrate synthase (CS) | 440 | 2050 (560–3300) | 109 | 71 (64–118) |
Abbreviation: ND, not determined.
a
nmol/min/mg. ( %)—residual activity normalized to CS.