Homozygous LmnaL530P/L530P mutation (Lmna∆9 or Lamin A∆Exon9) or loss of prelamin A-processing metalloproteinase Zmpste24 in mice phenocopies many of the progeroid features observed in Hutchinson-Gilford progeria syndrome (HGPS), one of the most severe forms of laminopathy that are predominantly caused by a 50-amino-acid deletion in prelamin A (lamin A∆50 or progerin). Here, ZMPSTE24 is linked to Hutchinson-Gilford progeria syndrome.