Laminopathies are caused by mutations in the LMNA gene encoding the ubiquitous proteins lamins A/C that are components of the lamina, a fibrous meshwork located at the inner face of the nuclear envelope. Laminopathies may affect one or several tissues such as striated muscles, peripheral nerves and adipose tissue in isolate or combined fashion. This review focuses on laminopathies affecting the striated muscle tissue only, namely Emery-Dreifuss muscular dystrophy (EDMD), limb girdle muscular dystrophy type 1B (LGMD1B) and dilated cardiomyopathy with conduction defects (DCM-CD). The phenotype of animal models in which the same mutation as that identified in EDMD or DCM-CD patients has been reproduced is presented as well as the pathophysiological mechanisms known to date.