Residual complex I activities in skeletal muscle homogenates and isolated fibroblast mitochondria
| Complex | % Activity (observed range for controls) | |||||
|---|---|---|---|---|---|---|
| Patient 1 | Patient 2 | Patient 3 | ||||
| Muscle | Fibroblasts | Muscle | Fibroblasts | Muscle | Fibroblasts | |
| All assays were performed in duplicate. | ||||||
| Complex I (CoI) activity was expressed as percentage of the mean of controls (for skeletal muscle homogenates, n = 16, mean activity 44 nmol/min/mg, observed range 19–90; for fibroblast mitochondria, n = 35, mean activity 74 nmol/min/mg, observed range 34–141) and relative to the activity of citrate synthase (CoI/CS) and complex II (CoI/CoII).2 | ||||||
| CoI | 68 (43–204) | 18 (46–191) | 2 (43–204) | 27 (46–191) | 27 (43–204) | 28 (46–191) |
| CoI/CS | 60 (36–167) | 35 (50–145) | 2 (36–167) | 34 (50–145) | 19 (36–167) | 24 (50–145) |
| CoI/CoII | 23 (51–153) | 19 (46–169) | 2 (51–153) | 14 (46–169) | 11 (51–153) | 13 (46–169) |