Fig. 2

Functional rescue of mitochondrial dysfunction. Bioenergetic profiles of SUCLA2 in patient P23 fibroblasts transduced with a lentiviral plasmid bearing wild-type SUCLA2 cDNA (Lv Wt) or a null construct (Lv Co), and control-derived fibroblasts. Experiments were performed using a Seahorse XF device. A Oxygen consumption rates of control and patient fibroblasts were measured in DMEM with galactose (1 g/L) instead of glucose, and upon the subsequent addition of a 6 μM oligomycin, b 50 μM FCCP, c 1 μM rotenone or, d 1 μM antimycin. B Basal and maximum respiration (Rmax) were calculated for each situation. C Oligomycin-sensitive respiration (OSR) was calculated as the difference between the basal respiration and the oxygen consumption rate measured as described in (C), after the addition of 6 μM oligomycin. The results reflect the mean of three biological repetitions. Control values are the mean for two different control cell lines. (*p < 0.05; **p < 0.01; ***p < 0.001 [Student t test])