Mutations in LMNA, which encodes lamin A and C, are associated to type A pattern of lipodystrophy (MADA; OMIM #248370) and mutations on the zinc metalloproteinase ZMPSTE24, encoding a protease involved in post-translational proteolytic processing of prelamin A to form mature lamin A, are linked to type B lipodystrophy (MADB; OMIM #608612).4,5. The gene discussed is LMNA; the disease is mandibuloacral dysplasia with type B lipodystrophy.