Table 2.
Enzyme Activities of Respiratory Chain Complexes and Citrate Synthase Normalized to Total Protein Content of Control and Affected Individual A:II-1 Muscle Tissue
| Activitya(Reference Range) | SDb | % of Control Mean | Activity/Citrate Synthasea(Reference Range) | SDb | |
|---|---|---|---|---|---|
| Complex I | 23.5 (23.6–74.8) | −1.7 | 52% | 120 (98–271) | −1.5 |
| Complex II | 25.8 (49.0–133.4) | −3.5 | 29% | 131 (251–573) | −3.6 |
| Complex III | 11.6 (5.7–31.4) | −0.3 | 67% | 59 (19–172) | −0.1 |
| Complex II + III | 30.7 (34.2–107.6) | −1.7 | 49% | 157 (173–472) | −1.8 |
| Complex IV | 1.2 (1.1–3.8) | −1.7 | 45% | 6 (4–23) | −1.7 |
| Citrate synthase | 196.1 (159.8–353.3) | −0.9 | 76% | NA | NA |
a
The activity in nmol·min−1·protein−1 and the ratio of the activity over citrate synthase is shown.
b
The distribution of the results from controls (n = 25) when log transformed is normal, and the affected individual value expressed as a Z-score of this control range. A SD of < −2 is considered significant. NA, not applicable.