Figure 6: Relative protein expression in dKO muscle compared to mdx.
From: Evidence for ACTN3 as a genetic modifier of Duchenne muscular dystrophy

(a,b) dKO muscle at 12-months of age show a compensatory upregulation of α-actinin-2 for the loss of α-actinin-3 and a ∼2.4-fold upregulation in RCAN1.4, but no change in the level of utrophin. (c,d) No significant differences in the expression of total and phosphorylated ACC, and total and phosphorylated AMPK were observed, but dKO mice did show a trend for increased expression of each. (e,f) dKO muscle shows an increase in oxidative metabolism as evidenced by significant upregulation in ATP synthase (ATPase), succinate-Q oxidoreductase (Complex II), cytochrome-c oxidoreductase (Complex III) and cytochrome c oxidase (Complex IV). Graphs b,d and f represent relative OD levels normalized to actin and mdx. Data shown as mean±SEM, One-way ANOVA, *P< 0.05. mdx n=4, dKO n=4. Full-length blots are presented in Supplementary Fig. 6.