The cause of Hutchinson-Gilford progeria syndrome (HGPS or progeria) is a dominant-negative C•G-to-T•A mutation (c.1824 C>T; p.G608G) in the LMNA gene, which encodes nuclear lamin A. This mutation results in RNA mis-splicing that produces progerin, a toxic protein that induces rapid aging and shortens the lifespan of children with progeria to approximately 14 years.
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