Figure 5

Peroxisome proliferator-activated receptor gamma coactivator (PGC)-1α reverses poor growth and kyphosis in dystrophin/utrophin-deficient mice. (A) Photograph of representative 5-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) indicating difference in size and pronounced kyphosis in mdx/utrn^-/- that is largely reversed in mdx/utrn^-/-/MCK-PGC-1α animals. (B) Representative radiograph of 13-week-old male mdx, mdx/MCK-PGC-1α, mdx/utrn^-/- and mdx/utrn^-/-/MCK-PGC-1α mice showing small size and pronounced kyphosis in mdx/utrn^-/- mice is largely reversed in mdx/utrn^-/-/MCK-PGC-1α mice. (C) Body weight (left) and body length from nose to tail base (right) of mdx (n = 8), mdx/MCK-PGC-1α (n = 8), mdx/utrn^-/- (n = 5) and mdx/utrn^-/-/MCK-PGC-1α (n = 5) male mice were measured every 2 weeks from 5 to 15 weeks of age. Graph represents average body weight and average body length, error bars SEM. (D) Kaplan-Meier survival plot of mdx (n = 8), mdx/MCK-PGC-1α (n = 8), mdx/utrn^-/- (n = 5) and mdx/utrn^-/-/MCK-PGC-1α (n = 5) male mice with time. P < 0.02 by Log-rank (Mantel-Cox) test for mdx/utrn^-/- compared to mdx/utrn^-/-/MCK-PGC-1α cohorts.