Impact of the Mitochondrial Genetic Background in Complex III Deficiency
Figure 2
BN-PAGE analysis of mitochondrial respiratory chain complexes in control and mutant fibroblasts and cybrids.
Mitochondrial particles were isolated as described in Methods and 40 µg of protein were analyzed on a 5–15% BN-polyacrylamide gel for the separation of multisubunit complexes. Western-blot analysis was performed using antibodies against the indicated OXPHOS subunits. CI, fully-assembled complex I. CIII2, complex III dimer. CIV, complex IV. CIII2+IV indicates the presence of the supercomplex containing complexes III and IV. C1 and C2, control fibroblasts. P, patient's fibroblasts. C, control cybrid. Two independent mutant cybrids are indicated as #1 and #2.