Fig. 4.

Biochemical and western blot analyses of patient fibroblasts. a Activity of mitochondrial respiratory complexes in control (red) and patient (blue) fibroblast samples. Mean enzyme activities normalised to citrate synthase (CS) of control fibroblasts (n = 8) are set to 100% and error bars represent standard deviation. b Western blots of protein lysate from patient fibroblasts (P) and two age-matched controls (C1 and C2) immunodecorated with antibodies against IARS2, NDUFB8 (CI), UQCRC2 (CIII), COXI (CIV), COXII (CIV) and ATP5A (CV). SDHA and SDHB (CII) were used as loading controls