The generation of mouse models that recapitulate many of the clinical manifestations of HGPS, by mimicking the point mutation found in most affected patients (LmnaG609G/G609G mice) (Osorio et al., 2011) or by knocking‐out Zmpste24 gene (Zmpste24−/− mice) (Bergo et al., 2002; Pendás et al., 2002), has become an invaluable tool to understand the mechanisms that drive the aging process and to test potential therapeutic interventions aimed at improving progeroid features and age‐related diseases. Here, ZMPSTE24 is linked to Hutchinson-Gilford progeria syndrome.