Table 1. Respiratory function assays in cybrid cell lines.
Results are expressed as means±S.D.; each individual clone was analysed twice. *P<0.001 and **P<0.01 indicate statistical significance with respect to wild-type (wt) and 100% A8296G cell lines respectively, and ***P<0.05 indicates statistical significance with respect to 100% A8296G cell lines, by the two-tailed, Mann–Whitney U test for unpaired samples. Oxygen consumption is expressed in terms of fmol·min−1·cell−1 and mean specific enzyme activity is expressed in terms of (nmol of substrate)·min−1·(mg of protein)−1.
| Mean specific enzyme activity | ||||||
|---|---|---|---|---|---|---|
| Cell line | Oxygen consumption | Complex I | Complex II | Complex III | Complex IV | Citrate synthase |
| Wt (n=6) | 4.6±0.9 | 14.3±1.2 | 10.8±3.5 | 104.0±25 | 54±8 | 200±26 |
| 8363 (n=2) | 0 | 5.6±0.8*,*** | 10.2±1.6 | 15.0±4.5** | 5.1±2.8* | 188±22 |
| 8296 (n=9) | 4.5±0.8 | 11.8±3.4 | 9.1±4.0 | 93.1±24 | 57±3 | 183±22 |
| 8296/8363 (n=6) | 0.1±0.1* | 3.9±1.0* | 11.1±3.0 | 5.5±4.5* | 6.4±2.6* | 188±22 |
| 143B (n=3) | 4.4±0.5 | 18.1±3.5 | 14.1±3.7 | 102±23 | 70±20 | 189±30 |
| 143B/206 (ρ0) (n=3) | 0 | 1.5±0.4 | 11.1±0.8 | 2±1.8 | 1±0.8 | 167±15 |