CLCN1 and muscular dystrophy: These models show that the molecular defects underlying the pathology not only results in AS defects but also in alterations of the transcriptome, that can be only partially ascribed to changes in muscle activity or to muscle damage, as evidenced by an only partial overlap with the transcriptome changes observed in Clcn1 KO mice, exhibiting myotomia, or in mdx mice, lacking dystrophin, a model of muscular dystrophy where muscle regeneration is continuously active.