In 2002, Pendás et al. and Bergo et al., developed the first HGPS mouse model – Zmpste24-null (Zmpste24−/−) mice – which showed growth retardation, cardiomyopathy, muscular dystrophy, lipodystrophy and premature death (Bergo et al., 2002; Pendas et al., 2002). Here, ZMPSTE24 is linked to muscular dystrophy.