Fig. 4

Respiration, of cell lines. Oxygen consumption rates measured using Oroboros O2k per million cells in response to treatment with glutamate-malate (G/M), rotenone, succinate, ADP, and FCCP (A) are shown. The mean oxygen consumption rates were compared between the parent HEK293 and the mutant HEK293ΔSDHD. The p values for the differences between the mean values were 0.0019, 0.0101, 0.0002, 0.0002 and 0.0017, respectively for Complex I respiration, rotenone treatment, Complex II respiration, OXPHOS capacity, and maximal respiration. Oxygen consumption rate (OCR) was measured using Agilent Seahorse XFe96 of parent cells treated DMSO (Parent + DMSO), mutant cells treated DMSO (Mutant + DMSO), and mutant cells treated 1μM idebenone (Mutant + Ideb). OCR per 20,000 permeabilized cells (B), and the calculated basal respiration (C), reserve respiratory capacity (D) and maximal respiratory capacity (E) are shown. The mean OCR values were compared between the parent versus the mutant or DMSO treatment versus idebenone treatment. Error bars represent the SE of the mean. Representative Oroboros oxygraphs depicting oxygen consumption of cell lines (F) are shown. The top and bottom panels represent parent and mutant cell lines, respectively. The oxygen consumption rate is illustrated by the red line