Fig. 4.

Effect of bezafibrate and riboflavin supplementation on respiratory chain activities in fibroblast cell lines. Maximal oxygen consumption rate (OCR) was measured in pmol/(s*Mill) of ACAD9 patient and control fibroblasts with and without (a) bezafibrate (400 μM for 72 h) and (b) riboflavin (530 nM for 72 h) treatment. Data are expressed as the average of > 10 technical replicates and normalized to control. ± SD. ***P < 0.001, **P < 0.01, *P < 0.05. (c) Whole cell lysate of control and different ACAD9 deficient fibroblasts +/− bezafibrate/riboflavin visualized with antibodies against ACAD9, ACADVL, ACADM, SDHA, ß-actin (loading control), NDUFS1 and NDUFA9 (d, e) Quantification of ACAD9 and ACADVL protein levels