Fig. 3.

mdx^4cv/mTR^G2 animal model faithfully recapitulates the skeletal and the cardiac phenotype of DMD. Molecular and functional characterization of skeletal muscle and cardiac phenotypes in the mdx^4cv/mTR^G2 double knockout mouse. Overall, mdx^4cv/mTR^G2 exhibited decreased life span. In the skeletal muscle, mdx^4cv/mTR^G2 exhibited decreased exercise capacity, decreased muscle strength, histological evidence of muscular dystrophy, and kyphosis. In the heart, mdx^4cv/mTR^G2 displayed cardiac dysfunction as measured by MRI, histology, ECHO, ECG, and electron microscopy. Figure adapted from prior mdx^4cv/mTR^G2 studies^108,109