The most commonly used DMD model is the C57BL/10ScSn (mdx) mouse, which carries a naturally occurring point mutation in exon 23 of the dystrophin gene, an absence of the full-length Dp427 protein isoform (3, 4) and to a lesser extent genetic variation of the mdx model (5–7). This evidence concerns the gene DMD and Duchenne muscular dystrophy.