Twenty human ZMPSTE24 mutations that reduce the enzyme activity have been identified to associate with three disease categories of increasing severity: mandibuloacral dysplasia type B, severe progeria (atypical ‘HGPS’) and restrictive dermopathy (Barrowman et al., 2012); the crucial involvement of ZMPSTE24 in laminopathies was further emphasised by a novel mouse model expressing non‐cleavable prelamin A (Wang, Shilagardi, et al., 2022). This evidence concerns the gene ZMPSTE24 and Hutchinson-Gilford progeria syndrome.