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Article

Absence of progeria-like disease phenotypes in knock-in mice expressing a non-farnesylated version of progerin.

Human molecular genetics - 1 Feb 2011

Yang Shao H, Chang Sandy Y, Ren Shuxun, Wang Yibin, Andres Douglas A, Spielmann H Peter, Fong Loren G, Young Stephen G

Abstract excerpt

Hutchinson-Gilford progeria syndrome (HGPS) is caused by a mutant prelamin A, progerin, that terminates with a farnesylcysteine. HGPS knock-in mice (Lmna(HG/+)) develop severe progeria-like disease phenotypes. These phenotypes can be ameliorated with a protein farnesyltransferase inhibitor (FTI), suggesting that progerin's farnesyl lipid is important for disease pathogenesis and raising the possibility that FTIs...

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Identifiers

PMID: 21088111
DOI: 10.1093/hmg/ddq490
PMCID: PMC3016906

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PubMed metadata and Literature Corpus relationships. Corpus snapshot updated 6 Sept 2026. Manifest publication-graph-cohort-2026-09-06.1.

Source receipt: pubmed, retrieved 6 Sept 2026.

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