Fig. 7.

Respiratory flux profiles of control and patient-derived human dermal fibroblasts and HeLa cell lines. A, B. Oxygen consumption rates (OCR) of control and patient fibroblasts and HeLa cell lines following injection of optimal concentrations of Oligomycin (4μM), FCCP (1μM) and Rotenone/antimycin A (1μM). Panel C shows decreased respiratory potential of FIB_65fs compared to FIB_WT with significant reductions in non-mitochondrial respiration, maximal respiration, ATP production and spare respiratory capacity. D. Oxygen consumption rates (OCR) of SGPL1-WT and SGPL1-KO cell lines show decreased respiratory potential of KO cells compared to control with significant reductions in non-mitochondrial and basal respiration. (* p < 0.05, ** p < 0.01); Data are presented as the mean of three experiments.