Figure 2: Immunoblotting and blue native polyacrylamide gel electrophoresis (BN-PAGE) in skeletal muscle and fibroblasts. | Genetics in Medicine

Figure 2: Immunoblotting and blue native polyacrylamide gel electrophoresis (BN-PAGE) in skeletal muscle and fibroblasts.

From: Mitochondrial oxodicarboxylate carrier deficiency is associated with mitochondrial DNA depletion and spinal muscular atrophy–like disease

Figure 2

(a,b) In skeletal muscle immunoblotting detected decreased steady-state levels of mitochondrial oxidative phosphorylation complex subunits for complexes I, III, IV, and V. (c,d) BN-PAGE detected reduced complexes I, IV, and V. (e) Immunoblotting detected slightly reduced SLC25A21 in the patient’s fibroblasts, while (f) mitochondrial oxidative phosphorylation proteins were normal. (g) BN-PAGE detected normal respiratory chain complexes in the patient’s cell line. Nuclear-encoded GAPDH, VDAC, SDHA, or SDHB proteins were used as loading controls. ATP5A, F1FO adenosine triphosphate synthase subunit; CI, complex I (etc.); CV, complex V/F1FO adenosine triphosphate synthase; COX1, cytochrome c oxidase 1; COX2, cytochrome c oxidase 2; GAPDH, glyceraldehyde 3-phosphate dehydrogenase; NDUFB8, nicotinamide adenine dinucleotide plus hydrogen (NAD) dehydrogenase ubiquinone 1 beta subcomplex subunit 8; SDHA, succinate dehydrogenase subunit A; SDHB, succinate dehydrogenase subunit B; UQCRC2, ubiquinol-cytochrome c reductase core protein II; VDAC, voltage-dependent anion-selective channel.

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