Fig 1: Imbalances in OXPHOS complexes. (a) The steady-state levels of five OXPHOS complexes by Blue Native gel electrophoresis. Fifteen µg of mitochondrial proteins from mutant and control cell lines was electrophoresed through a Blue Native gel, electroblotted and hybridized with antibodies for NDUFA9, SDHA, UQCRC2, COX5A, ATP5C (complexes I, II, III, IV, and V), and VDAC as a loading control. (b) Quantification of levels of complexes I, II, III, IV, and V in two mutant cell lines and two control cybrid cell lines was determined. The error bars indicate two standard errors of the means. *P < 0.05 and **P < 0.01 indicate the significance, according to the t-test, of the differences between mutant and control cybrid cell lines.