High levels of CCN2/CTGF has been reported in skeletal muscle biopsies from Duchenne muscular dystrophy (DMD), Becker muscular dystrophy, and Fukuyama-type congenital muscular dystrophy patients [31,46], and in samples from murine models for DMD and Limb-Girdle muscular dystrophy (LGMD) [30,32,47]. This evidence concerns the gene CCN2 and limb-girdle muscular dystrophy.