Genotype-phenotype studies have established that complete absence of laminin α2 chain leads to a very severe muscular dystrophy (ambulation is typically not achieved) whereas partial deficiency causes a milder limb-girdle-type muscular dystrophy [2; 3] There are several relevant mouse models for LAMA2-CMD recapitulating grave and milder forms of LAMA2-CMD, including the dy3K/dy3K and dy2J/dy2J mouse models. This evidence concerns the gene LAMA2 and congenital muscular dystrophy.