In fact, since the identification of the first laminopathy, the autosomal dominant Emery-Dreifuss muscular dystrophy (EDMD) [7], a range of muscular dystrophies of various clinical severity related to LMNA mutations has been described, from the most severe and early onset congenital form, the LMNA-related Congenital Muscular Dystrophy (L-CMD [8]) to less severe and almost adult onset form, the autosomal dominant Limb-Girdle Muscular Dystrophy type 1B (LGMD1B [9]). This evidence concerns the gene LMNA and Emery-Dreifuss muscular dystrophy.