Research Paper Volume 8, Issue 5 pp 945—957

Study of mitochondrial respiratory defects on reprogramming to human induced pluripotent stem cells

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Figure 2. OXPHOS analysis of control and LHON fibroblasts. (A) Oxygen consumption of control and LHON fibroblasts, showing the endogenous respiration rate (Endog), ADP-stimulated cell respiration with glutamate + malate (CxI-ADP) or with succinate (CxI+II-ADP), the uncoupled maximal respiration by addition of CCCP (CxI+II-UC) and the complex II respiration with uncoupled feedback by addition of rotenone (CxII-U). n = 3 for each patient fibroblast. Error bars represent SEM. (B) Complex I respiration of individual fibroblast samples and pooled data, normalized to uncoupled maximal respiration (CxI-ADP/CxI+II-UC). (C) Uncoupled complex II respiration (CxII-U/CxI+II-UC) in control (CERA007, MRU11780, BJ) and LHON fibroblasts (LHON V31-1, LHON T1-20, LHON Q1-4). **** = p<0.0001, *** = p<0.001, ** = p<0.01, * = p<0.05, ns = not significant.