Lamin A is a major component of the nuclear lamina, a dynamic meshwork located just under the nuclear envelope and it is encoded by lamin A/C gene (LMNA). Lamin A is synthesized as a longer precursor, prelamin A (code REP0038), that in vivo undergoes to serial post-translational modifications that lead to mature lamin A. Different mutations in the LMNA gene have been associated with different diseases that are collectively called laminophaties, including Emery-Dreifuss muscular dystrophy, familiar partial lipodystrophy, limb girdle muscular dystrophy, dilated cardiomyopathy, Charcot-Marie-Tooth disease, and Hutchinson-Gilford progeria syndrome.