Prelamin A undergoes a series of posttranslational modifications at its C terminal region to give rise to mature lamin A. Most of progeroid laminopathies are due to mutations that are within this C-terminus, encompassing the cleavage sites for ZMPSTE24 protein, leading to the toxic accumulation at the nuclear envelope of the lamin A precursor, prelamin A, or progerin, a truncated form of lamin A. In fact, the accumulation of progerin at the nuclear envelope is the molecular hallmark of Hutchinson–Gilford Progeria Syndrome (HGPS), an extreme progeroid laminopathy. This evidence concerns the gene ZMPSTE24 and laminopathy.