Figure 4

Peroxisome proliferator-activated receptor gamma coactivator (PGC)-1α ameliorates muscle damage in dystrophin/utrophin-deficient mice. (A) Five-week-old dystrophin-deficient male mice (mdx), PGC-1α muscle-specific transgenic in an mdx background (mdx/MCK-PGC-1α), dystrophin/utrophin-deficient mice (mdx/utrn^-/-), and PGC-1α muscle-specific transgenic in dystrophin/utrophin-deficient mice (mdx/utrn^-/-/MCK-PGC-1α) were sacrificed and quantitative PCR performed on gastrocnemius (n = 5 per group). Bars depict relative mRNA expression, error bars represent SEM. (B) Quadriceps from 5-week-old mdx, mdx/utrn^-/- and mdx/utrn^-/-/MCK-PGC-1α were harvested and subjected to immunoblot analysis with anti-utrophin antibody and anti-pan-actin antibody as loading control. Results are representative of three independent experiments. (C) Five-week-old male mdx, mdx/MCK-PGC-1α, mdx/utrn^-/- and mdx/utrn^-/-/MCK-PGC-1α mice were sacked and blood was drawn. Serum creatine kinase was measured. Bars represent average level of serum creatine kinase. Error bars represent SEM (n = 5). (D) Evans Blue was injected intraperitoneally 16 hours before sacking into 5-week-old male mdx, mdx/MCK-PGC-1α, mdx/utrn^-/- and mdx/utrn^-/-/MCK-PGC-1α. Histological sections from gastrocnemius and diaphragm were analyzed by fluorescence microscopy. *P < 0.05. AChR, acetylcholine receptor.