Table 1.
Mitochondrial respiratory chain enzyme activities in skeletal muscle and cultured skin fibroblasts.
| Skeletal Muscle | Fibroblasts | |||
|---|---|---|---|---|
| Patient (Controls) 1 | % 2 | Patient (Controls) 3 | % 4 | |
| CI (NADH–DB oxidoreductase) | 8.1 (11.3–24.7) | 72% | 7.1 (20.4 ± 2.3) | 35% |
| CII (Succinate dehydrogenase) | 4.7 (5.8–19.9) | 81% | 58.5 (48.4 ± 7.16) | 120% |
| CIII (DBH2–Cytochrome c oxidoreductase) | 52.3 (31–127) | 169% | 162 (160 ± 22) | 101% |
| CIV (Cytochrome c oxidase) | 21.1 (20–79.2) | 106% | 66.2 (55.4 ± 14.3) | 119% |
| CS (Citrate synthase) 5 | 212 (102–257) | - | 47.1 (77.0 ± 27.0) | - |
(1, 3) nmol min−1 mg−1 protein enzyme activity expressed with respect to citrate synthase (CS) activity. (1) Control range, 2.5th–97.5th percentile (n = 95). (2) % of complex activity relative to the 2.5th percentile of controls. (3) Control mean ± SD (n = 3 cultured skin fibroblasts assayed in parallel to patient´s fibroblasts). (4) % of complex activity relative to the mean control values. (5) CS activity expressed as nmol min−1 mg−1 protein.