Nearly all HGPS patients harbor a dominant LMNA mutation that, through altered splicing, generates an internally deleted version of prelamin A called progerin, which retains its CAAX motif but lacks the ZMPSTE24 cleavage site and causes disease phenotypes (De Sandre-Giovannoli et al., 2003; Eriksson et al., 2003; Gordon et al., 2014; Merideth et al., 2008). The gene discussed is LMNA; the disease is Hutchinson-Gilford progeria syndrome.