Figure 3. Succinate prodrug treatment of mitochondrial complex I-deficient Leigh syndrome patient fibroblasts.

(a,b) Oxygen consumption rate (OCR) in three control fibroblast cell lines and a mitochondrial complex I-deficient cell line (recessive NDUFS2 mutation) treated with NV189 or vehicle. (c–f) Quantification of OCR in control and patient fibroblasts for each respiratory state. (g,h) Relative contribution of complex I- and complex II-linked respiration to maximum uncoupled respiration in patient cells and control cell lines. (i) Spare respiratory capacity, defined as per cent increase from endogenous baseline to maximum uncoupled respiration. Data presented as mean and s.e. of n=3 experiments from separate cell culture flasks performed with eight technical replicates each time for each cell lines. Data from the three control cell lines are pooled. *P<0.05 (two-tailed unpaired Student's t-test, difference between Leigh and control cell lines).