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. 2017 Sep 8;3(5):e184. doi: 10.1212/NXG.0000000000000184

Figure 3. Blue native polyacrylamide gel electrophoresis of respiratory chain complexes in the 3 patients (P1, P2, and P3) and controls (C1 and C2).

Figure 3

(A) Blue native polyacrylamide gel electrophoresis (PAGE) was performed using mitochondrial fractions prepared from myoblasts. No patient showed severely reduced assembly of respiratory chain complexes as compared to the control. (B) In-gel activity staining and Western blotting of complexes I and II. In-gel activity staining of complex II after separation by high-resolution clear native (hrCN)-PAGE using mitochondrial fractions prepared from the myoblasts. In parallel, complexes I, II, and V were detected by Western blotting after their separation by hrCN-PAGE. All patients showed clearly reduced complex II activity when compared with the control, whereas no such decrease was detected by Western blotting. The activity and amount of assembled complex I were not significantly different from those of controls. (C, D) The experiments were performed in triplicate and normalized to complex V (n.s. = not significant, **p < 0.005, Student t test).