In agreement with this pathogenetic hypothesis, Erk 1/2 activation has been demonstrated in the presence of pathogenetic LMNA and SYNE1 mutations [7,13], and altered positioning of muscle nuclei has been demonstrated in LMNA, SYNE1 and SUN1-linked Emery-Dreifuss muscular dystrophy with cardiomyopathy [12–14,18]. The gene discussed is SYNE1; the disease is Emery-Dreifuss muscular dystrophy.