Table 2. Restoration of complex IV activity by complementation with the wild-type gene.
Assaya, fibroblast homogenate | Patient+vector | Patient+COX6B1 | Controls, n=5 (range) |
---|---|---|---|
Complex II (succinate dehydrogenase) | 19.5 (66%) | 17.9 (81%) | 21.6 (13–36) |
Complex IV (cytochrome c oxidase) | Undetectable | 41.4 (46%) | 81.8 (43–139) |
Citrate synthase (CS) | 85.0 | 63.5 | 55.8±15 (39–84) |
nmol/min/mg. (%)—residual activity normalized to CS.