Table 1.
Enzymatic activity of the OXPHOS complexes in muscle and fibroblasts.
| Muscle | Fibroblasts | |||||
|---|---|---|---|---|---|---|
| Mean | M1 | M2 | Control range | Patient | Control range | |
| Citrate synthase | 140 | 140 | 139 | (166–311) | 210 | (225–459) |
| Complex I | 9 | 10 | 8 | (30–84) | 20 | (18–53) |
| Complex I + III | 30 | 30 | 30 | (27–58) | 152 | (73–220) |
| Complex II | 38 | 40 | 36 | (53–102) | 74 | (64–124) |
| Complex II + III | 34 | 31 | 36 | (41–84) | 125 | (79–219) |
| Complex III | 111 | 108 | 115 | (230–486) | 555 | (208–648) |
| Complex IV | 233 | 229 | 237 | (205–739) | 380 | (175–403) |
| Complex V | 79 | 72 | 85 | (78–178) | 92 | (43–190) |
Values are given in mUnits/mg protein. M1 and M2: values of two measurements.