Figure 1

Complex I-supported mitochondrial function is impaired in XP-C cells. (A) O2 consumption for MRC-5 (WT), XP4PA (unXP-C) and XP4PA expressing XPC (corrXP-C) was measured as described, using 4 × 106 cells in standard reaction medium containing 5 mM CI substrate, with the subsequent additions of 30 µM Digitonin, 100 µM ADP, 4 µM CAT and 1 µM CCCP. The figure shows a representative trace OCR experiment. (B) The rates of O2 consumption and respiratory control (RC, ratio between CCCP/CAT) were calculated from data obtained in the experiments represented in (A). (C) CI + CIII activity was measured using 5 µg of crude mitochondrial protein. Reduction of cyt c was monitored at 550 nm at 37 °C for 5 min. For the calculations, only the region where the slopes of the reactions were linear were considered. The data represent mean ± SD of 4 independent experiments. *p < 0.05, **p < 0.01 and ***p < 0.001.