Fig. 3
VCPR191Q/wt causes increased mitochondrial oxygen consumption rate and electron transport chain activity. A Oxygen consumption rate (OCR) in WT and VCPR191Q/wt cell lines: state 2 respiration following digitonin permeabilization, state 3 respiration following ADP addition, uncoupled respiration following mitochondrial uncoupling by FCCP and proton leak following oligomycin A treatment; dots represent individual measurements across at least three independent experiments. B Respiratory control ratio, calculated as state 3/state 2 respiration; dots represent individual measurements across at least three independent experiments. C Electron transport chain activity in WT and VCPR191Q/wt cell lines exposed to varying substrates: succinic acid, α-ketoglutaric acid, fumaric acid or L-malic acid; dots represent individual measurements across seven (VCPR191Q/wt) or eight (WT) independent experiments. A–C Means ± SEM are shown. Statistical significance was evaluated by Two-way ANOVA with Bonferroni correction for multiple comparisons (A, C) or unpaired Student t-test (B); ns p > 0.05, *p < 0.05, **p < 0.01, ***p < 0.001, ****p < 0.0001