Oxygen consumption of mitochondria isolated from wild-type and KGDHc subunit heterozygote KO mice respiring on α-ketoglutarate (α-KG) in the absence (B) or presence (C,D) of ADP, and after addition of carboxyatractilozide (CAT), (D). (A) Trace are representatives of experiments with wild-type mice (WT; solid line), dihydrolipoyl succinyltransferase transgenic mice (DLST+/−; dotted line), and dihydrolipoyl dehydrogenase transgenic mice (DLD+/−; dashed line). Oxygen consumption was monitored by high-resolution respirometry. Mitochondria (0.1 mg/mL) were incubated in a standard medium, as described under Materials and Methods. Afterwards, α-KG (5 mM), ADP (2 mM); (B,C), and carboxyatractyloside (CAT; 2 µM); (D) were given. White bars: WT; bars with left diagonal stripes: DLST+/− mutation; bars with right diagonal stripes: DLD+/− mutation. The results are expressed as the means of oxygen consumption in nmol/min/mg protein ± SEM (N = 4–16). Statistically significant differences are indicated by asterisks; * p < 0.05.