The vast majority of HGPS is caused by a de novo autosomal dominant point mutation in LMNA that results in a splicing defect of pre-lamin A (preLaA) and the accumulation of a preLaA protein lacking 50 amino acids called progerin, or LaA Δ50 [43,44]. The gene discussed is LMNA; the disease is Hutchinson-Gilford progeria syndrome.