Fibroblasts | Skeletal muscle homogenate | Liver homogenate | ||||
---|---|---|---|---|---|---|
Enzymes | Patient | Controls (n = 22) | Patient | Controls (n = 26) | Patient | Controls (n = 12) |
Complex I | 0.29 | 0.19 - 0.46 (0.29 +/- 0.07) | 0.16/0.13* | 0.12 - 0.28 (0.19 +/- 0.04) | 0.007/0.008* | 0.22 - 0.76 (0.43 +/- 0.2) |
Complex II | 0.25 | 0.17 - 0.52 (0.33 +/- 0.09) | 0.13/0.15* | 0.14 - 0.36 (0.21 +/- 0.05) | 0.68/0.75* | 0.59 - 2.11 (1.35 +/- 0.45) |
Complex III | 0.42 | 0.35 - 0.87 (0.6 +/- 0.15) | 0.74 | 0.55 - 1.11 (1.16 +/- 0.28) | 0.26/0.3* | 0.54 - 2.16 (1.47 +/- 0.49) |
Complex IV | 0.49 | 0.42 - 1.11 (0.75 +/- 0.18) | 0.27/0.17* | 0.57 - 1.77 (0.78 +/- 0.15) | 0.35/0.42* | 0.74 - 5.17 (2.1 +/- 1.2) |
Complex V | 0.16 | 0.14 - 0.42 (0.22 +/- 0.08) | 0.19 | 0.19 - 0.65 (0.39 +/- 0.13) | 0.42 | 0.25 - 1.14 (0.58 +/- 0.28) |
Citrate synthase | 172 | 106 - 317 (184 +/- 43) | 124 | 70 - 169 (105 +/- 25) | 68 | 21 - 40 (31 +/- 6.5) |