Skip to main content
. 2014 Apr 30;23(2):159–164. doi: 10.1038/ejhg.2014.85

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)
a

nmol/min/mg. (%)—residual activity normalized to CS.