LMNA (Lamin A/C gene; HGPS)

LMNA is a gene that makes two nuclear-skeleton proteins, Lamin A and Lamin C (via alternative splicing). These lamins form a mesh just under the inner nuclear membrane of your cells. It provides mechanical support and organizes the chromatin at the nucleus's edge, influencing gene activity, DNA repair, and nuclear shape. A single-letter change at position 1824 (c.1824C>T; G608G) in exon 11 switches on a hidden splice site. That produces a shortened, permanently 'farnesylated' Lamin A called progerin. Progerin causes Hutchinson-Gilford Progeria Syndrome (HGPS), a devastating childhood aging disease with early heart disease and a median survival of about 14 years. Here is the wider relevance: small amounts of that same faulty splice product also build up in normal aging cells, even without the HGPS mutation, and nuclear lamina integrity declines broadly with age. So LMNA biology matters beyond the rare syndrome. A drug called lonafarnib (Zokinvy), which blocks progerin's farnesylation, won FDA approval in November 2020 for HGPS and extends median survival by about 2.5 years. Other progerin-targeting approaches are in early study.

Last reviewed: June 22, 2026

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