Fig. 2

Fig. 2 Mitochondrial complex formation is disrupted in fibroblasts derived from the patient carrying the NDUFB7 mutations.

Using blue native polyacrylamide gel electrophoresis (BN-PAGE), mitochondrial extracts from control (C) and patient (P) skin fibroblasts were separated using Triton A X-100 or B digitonin. These extracts were probed for the indicated protein in parentheses to identify Complexes I–IV. Complex II served as a loading control, as shown at the bottom of each well, except in the Complex II lane. C The samples were further separated by 2D-SDS PAGE and probed for the indicated mitochondrial accessory proteins.