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. 2019 Sep 2;9:12651. doi: 10.1038/s41598-019-49014-2

Figure 2.

Figure 2

Mitochondrial respiration impairment in ETHE1 deficient fibroblasts. Oxygen consumption rate (OCR) was measured in the resting state (basal respiration) followed by injection of oligomycin (an inhibitor of ATP synthase) that reduces OCR, representing ATP turnover. Subsequent injection of FCCP dissipates the proton gradient and allows maximum respiration. Finally, rotenone (Rot) and antimycin A (AA) are added to completely disable the electron transport chain, inhibiting the total mitochondrial respiration. The remaining OCR represents non-mitochondrial respiration (A). Basal respiration of ETHE1-1 (B), ETHE1-2 (C), ETHE1-3 (D) and ETHE1-4 fibroblasts (E), and maximal respiration of ETHE1-1 (F), ETHE1-2 (G), ETHE1-3 (H) and ETHE1-4 (I) fibroblasts exposed or not to 40 nM JP4-039 (JP4) for 24 h. Data are means ± SD; number of replicates: 7–8. *P < 0.05, **P < 0.01, ***P < 0.001, ****P < 0.0001, compared to control (wild type) cells; #P < 0.05, ##P < 0.01 compared to patient cells (Tukey multiple range test).