In fact, mutations in the LMNA gene encoding lamin A -a fundamental constituent of the nuclear envelope- or in that encoding the metalloproteinase ZMPSTE24 involved in the proteolytic processing of lamin A- cause dramatic human progeroid syndromes, such as Hutchinson-Gilford progeria syndrome (HGPS), atypical Werner syndrome, mandibuloacral dysplasia and restrictive dermopathy [20,22,23]. Here, ZMPSTE24 is linked to Hutchinson-Gilford progeria syndrome.