(A) Immunoblot analysis of one-dimension BNGE in immortalized fibroblasts from two controls (Ct1, Ct2) and mutant subjects S1 and S2. Samples were prepared using 10% w/v dodecylmaltoside. We used an antibody against NDUFA9 to detect complex I (I), an antibody against the subunit α of ATP synthase to detect complex V (V), an antibody against SDHA for complex II (II), an antibody against core I for complex III (III), and an antibody against subunit COX4 for complex IV (IV). Note that the amounts of cIV holocomplex and cIV+cIII2 supercomplex are clearly decreased in mutant samples compared to controls.