Approximately 90% of HGPS patients have a de novo heterozygous synonymous mutation at codon 608 (c.1824C>T; p.G608G) in the LMNA gene that activates the use of an internal 5′ splicing site in exon 11 and results in the synthesis of progerin, an unprocessed, farnesylated form of prelamin A (Figure 1) [3,4]. The gene discussed is LMNA; the disease is Hutchinson-Gilford progeria syndrome.