Figure 1.

Western blot analysis of SDHA and CII in patient fibroblasts. (a) Lysates of fibroblasts of patients with SDHA mutations (P1–4) and a control cell line (C) were separated by SDS-PAGE and subsequently blotted to PVDF membrane. Membranes were probed with anti-SDHA and anti-actin as a loading control. (b) Blue native analysis of respiratory chain complexes in mitochondrial fractions from SDHA patient fibroblasts (P1–4) compared with control (C) and CI-deficient (P5 and P6) fibroblasts. Blots were incubated with the indicated antisera.