Increased PGC-1α expression increases mitochondrial biogenesis and function in the skeletal muscle of Mut mice. (A) Western blot showing the levels of mitochondrial proteins in the quadriceps of mice and loading control actin. ATP synthase subunit 5α (ATP5A; subunit of complex V), ubiquinol-cytochrome c reductase core protein 2 (UQCRC2; subunit of complex III), mitochondrial cytochrome c oxidase subunit 1 (MTCO1; subunit of complex IV), succinate dehydrogenase subunit A and B, or SDHA and SDHB (subunits of complex II) and NADH dehydrogenase (ubiquinone) 1β subcomplex subunit 8 (NDUFB8; subunit of complex I; n = 4/group). (B) Quantification of western blot in (A) showing protein levels normalized to actin. (C) Histology of the quadriceps showing COX (complex IV) and SDH (complex II) activity staining (n = 3/group). (D) Complex I and III activity, (E) complex IV activity and (F) CS activity in total quadricep homogenate (n = 4/group). (G) MtDNA levels relative to WT in the quadriceps based on the ND1 copy number (subunit of complex I) normalized to GAPDH (n = 4/group). Analyzed 10-month-old male mice. Student's t-test: *P< 0.05, **P< 0.01 and ***P< 0.001. Error bars represent the SEM.