{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,14]],"date-time":"2026-10-14T10:50:15Z","timestamp":1791975015972,"version":"4.4.0"},"reference-count":74,"publisher":"Portland Press Ltd.","issue":"6","content-domain":{"domain":["portlandpress.com"],"crossmark-restriction":true},"short-container-title":[],"published-print":{"date-parts":[[2011,12,1]]},"abstract":"<jats:p>A common feature of progeria syndromes is a premature aging phenotype and an enhanced accumulation of DNA damage arising from a compromised repair system. HGPS (Hutchinson\u2013Gilford progeria syndrome) is a severe form of progeria in which patients accumulate progerin, a mutant lamin A protein derived from a splicing variant of the lamin A\/C gene (LMNA). Progerin causes chromatin perturbations which result in the formation of DSBs (double-strand breaks) and abnormal DDR (DNA-damage response). In the present article, we review recent findings which resolve some mechanistic details of how progerin may disrupt DDR pathways in HGPS cells. We propose that progerin accumulation results in disruption of functions of some replication and repair factors, causing the mislocalization of XPA (xeroderma pigmentosum group A) protein to the replication forks, replication fork stalling and, subsequently, DNA DSBs. The binding of XPA to the stalled forks excludes normal binding by repair proteins, leading to DSB accumulation, which activates ATM (ataxia telangiectasia mutated) and ATR (ATM- and Rad3-related) checkpoints, and arresting cell-cycle progression.<\/jats:p>","DOI":"10.1042\/bst20110687","type":"journal-article","created":{"date-parts":[[2011,11,21]],"date-time":"2011-11-21T10:56:00Z","timestamp":1321872960000},"page":"1764-1769","update-policy":"https:\/\/doi.org\/10.1042\/crossmark_policy","source":"Crossref","is-referenced-by-count":69,"title":["DNA-damage accumulation and replicative arrest in Hutchinson\u2013Gilford progeria syndrome"],"prefix":"10.1042","volume":"39","author":[{"given":"Phillip R.","family":"Musich","sequence":"first","affiliation":[{"name":"Department of Biochemistry and Molecular Biology, Quillen College of Medicine, East Tennessee State University, Johnson City, TN 37614-0581, U.S.A."}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Yue","family":"Zou","sequence":"additional","affiliation":[{"name":"Department of Biochemistry and Molecular Biology, Quillen College of Medicine, East Tennessee State University, Johnson City, TN 37614-0581, U.S.A."}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"288","published-online":{"date-parts":[[2011,11,21]]},"reference":[{"key":"2021111720442553700_B1","doi-asserted-by":"crossref","first-page":"7466","DOI":"10.1093\/nar\/gkm756","article-title":"Changes in DNA repair during aging","volume":"35","author":"Gorbunova","year":"2007","journal-title":"Nucleic Acids Res."},{"key":"2021111720442553700_B2","doi-asserted-by":"crossref","first-page":"437","DOI":"10.1016\/j.cell.2005.01.027","article-title":"Understanding the odd science of aging","volume":"120","author":"Kirkwood","year":"2005","journal-title":"Cell"},{"key":"2021111720442553700_B3","doi-asserted-by":"crossref","first-page":"497","DOI":"10.1016\/j.cell.2005.01.028","article-title":"DNA repair, genome stability, and aging","volume":"120","author":"Lombard","year":"2005","journal-title":"Cell"},{"key":"2021111720442553700_B4","doi-asserted-by":"crossref","first-page":"168","DOI":"10.1038\/ncb1095","article-title":"Senescing human cells and ageing mice accumulate DNA lesions with unrepairable double-strand breaks","volume":"6","author":"Sedelnikova","year":"2004","journal-title":"Nat. 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