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Figure 3: Blue native (BN) gel electrophoresis of muscle and fibroblasts. (a) BN-PAGE of the muscle with lauryl maltoside solubilization. (b, c) Densitometric analysis of BN-PAGE from the muscle. (d) BN-PAGE of fibroblasts. (e, f) Densitometric analysis of BN-PAGE from fibroblasts. (g) BN-PAGE of the muscle with a digitonin solubilization. The membrane was incubated with antibodies targeted against NDUFS4, Core 2, COX2, ATP5A1, and SDHA. C1–C10 are normal controls. In addition, muscle homogenates from patients with loss of function mutations in either SURF1 (NM_003172.3) c.11_30del20 (p.Val4Alafs49) or NDUFS4 (NM_002495.2) c.466_469dupAAGT (p.Ser157) were used as disease controls.