Table 2.
Oxygen consumption of fibroblasts from proband 2 in a SUIT protocol.
| Parameter | Patient | Median | 5th and 95th percentile |
|---|---|---|---|
| Pyruvate + ADP | 12.84 (z = − 2.07) ↓ | 28.6 | 14.2–63.1 |
| Glutamate | 12.98 (z = − 2.28) ↓ | 30.7 | 15.1–65.4 |
| Succinate | 22.93 (z = − 1.85) ↓ | 39.3 | 23.0–80.3 |
| CCCP | 50.21 (z = − 1.07) | 66.1 | 45.9–139.5 |
| Acceptor control ratio | 2.33 (z = − 0.13) | 2.37 | 1.22–4.42 |
| Glutamate increase | 0.01 (z = − 1.85) ↓ | 0.08 | 0.01–0.13 |
| Qpoint | 1.77 ↑ | 1.28 | 1.13–1.67 |
| Coupling ratio | 0.46 (z = − 1.19) | 0.61 | 0.34–0.83 |
| Complex 1 | 26.19 ↓ | 48.4 | 31.6–123.2 |
| Complex 4 | 61.50 | 52.3 | 30.8–108.2 |
The oxygen consumption by fibroblasts from proband 2 was evaluated in a SUIT protocol as described. Results are expressed in nmol oxygen consumed per minute and per 106 cells. Following the median value, the calculated Z-factor is given for the variable without log transformation (glutamate increase and coupling ratio) and after log transformation (ADP + pyruvate, Glutamate, succinate, CCCP, acceptor control ratio) using mean and standard deviation derived from 53 runs of 29 control fibroblasts, which showed normality of distribution for that parameter.