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. 2017 Sep 21;101(4):525–538. doi: 10.1016/j.ajhg.2017.08.015

Figure 3.

Figure 3

Steady-State Levels of OXPHOS Complex Subunits

(A) Western blot analysis of OXPHOS subunits in skeletal-muscle lysates from control individuals (C1 and C2) and probands S1, S3, and S4.

(B) Western blot analysis of OXPHOS subunits in skin fibroblasts from probands S1–S3 and age-matched control individuals (C1–C3).

OXPHOS-subunit-specific antibodies were used against NDUFB8 or NDUFA9 (CI); SDHA or SDHB (CII); UQCRC2 (CIII); COXI, COXII, or COXIV (CIV); and ATP5A (CV). Cytosolic β-actin and mitochondrial markers porin (VDAC) and SDHA were used as loading controls. All experiments were repeated at least twice, and representative western blots are shown. Number of repeats: (A) n = 3 (S1) and n = 2 (S3 and S4); (B) n = 4 (S1) and n = 2 (S2 and S3).