Figure 2.

(A) Oxygen consumption rate (OCR) in healthy fibroblasts and mitochondrial disease (MD) fibroblasts. Mitochondrial respiration, reflected by OCR levels, was detected following the addition of oligomycin, carbonyl cyanide-p-trifluoromethoxyphenyl-hydrazon (FCCP), the uncoupler, or the electron transport inhibitor rotenone and antimycin A. Actual measurement values were normalized with basal respiration. The left panel shows a schematic of the experiment on OCR. (B) Opposite to the decrease in adenosine triphosphate (ATP)-linked OCR, proton leak increased in MD fibroblasts. Rates of ATP production, maximal respiration, spare capacity, and proton leak were quantified by normalization of basal OCR. The above data were graphed for each item. Error bars represent ± SD (n = 4 independent experiments). *P < 0.05, **P < 0.01 (Student’s t-test).