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Article

Progerin elicits disease phenotypes of progeria in mice whether or not it is farnesylated.

The Journal of clinical investigation - 1 Oct 2008

Yang Shao H, Andres Douglas A, Spielmann H Peter, Young Stephen G, Fong Loren G

Abstract excerpt

Hutchinson-Gilford progeria syndrome (HGPS), a rare disease that results in what appears to be premature aging, is caused by the production of a mutant form of prelamin A known as progerin. Progerin retains a farnesyl lipid anchor at its carboxyl terminus, a modification that is thought to be important in disease pathogenesis. Inhibition of protein farnesylation improves the hallmark nuclear shape abnormalities...

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Identifiers

PMID: 18769635
DOI: 10.1172/JCI35876
PMCID: PMC2525700

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PubMed metadata and Literature Corpus relationships. Corpus snapshot updated 7 Sept 2026. Manifest publication-graph-cohort-2026-09-07.1.

Source receipt: pubmed, retrieved 7 Sept 2026.

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Progerin elicits disease phenotypes of progeria in mice whether or not it is farnesylated.PMID 18769635Read ↗

Increasing the length of progerin's isoprenyl anchor does not worsen bone disease or survival in mice with Hutchinson-Gilford progeria syndrome.Semantic proximity Read ↗

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Inhibiting farnesylation of progerin prevents the characteristic nuclear blebbing of Hutchinson-Gilford progeria syndrome.Semantic proximity Read ↗

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A farnesyltransferase inhibitor improves disease phenotypes in mice with a Hutchinson-Gilford progeria syndrome mutation.Semantic proximity Read ↗

Inhibiting farnesylation reverses the nuclear morphology defect in a HeLa cell model for Hutchinson-Gilford progeria syndrome.Semantic proximity Read ↗

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