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. Author manuscript; available in PMC: 2017 Sep 1.
Published in final edited form as: Mol Genet Metab. 2016 Jul 25;119(1-2):68–74. doi: 10.1016/j.ymgme.2016.07.007

Figure 2. Cellular respiration defect in SUCLG1 patient fibroblasts is dependent on SUCLG1 deficiency.

Figure 2

Cellular respiration analysis demonstrates reduced respiratory capacity in patient fibroblasts (P) compared to controls (Co) (n = 5) that is completely restored with ectopic expression of wild type SUCLG1 in patient cells (“P+R”). Experiment was done in triplicate. Error bars indicate standard deviation. “OCR” = oxygen consumption rate. Oligomycin = complex V (ATP synthase) inhibitor; FCCP = proton ionophore uncoupler; Antimycin & Rotenone (A&R) = inhibitors of ETC complex III and complex I, respectively.