LMNA and laminopathy: 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.