{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,22]],"date-time":"2026-09-22T10:15:02Z","timestamp":1790072102115,"version":"4.0.1"},"reference-count":88,"publisher":"Portland Press Ltd.","issue":"3","license":[{"start":{"date-parts":[[2020,6,15]],"date-time":"2020-06-15T00:00:00Z","timestamp":1592179200000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":["portlandpress.com"],"crossmark-restriction":true},"short-container-title":[],"published-print":{"date-parts":[[2020,6,30]]},"abstract":"<jats:p>The metazoan nucleus is equipped with a meshwork of intermediate filament proteins called the A- and B-type lamins. Lamins lie beneath the inner nuclear membrane and serve as a nexus to maintain the architectural integrity of the nucleus, chromatin organization, DNA repair and replication and to regulate nucleocytoplasmic transport. Perturbations or mutations in various components of the nuclear lamina result in a large spectrum of human diseases collectively called laminopathies. One of the most well-characterized laminopathies is Hutchinson\u2013Gilford progeria (HGPS), a rare segmental premature aging syndrome that resembles many features of normal human aging. HGPS patients exhibit alopecia, skin abnormalities, osteoporosis and succumb to cardiovascular complications in their teens. HGPS is caused by a mutation in LMNA, resulting in a mutated form of lamin A, termed progerin. Progerin expression results in a myriad of cellular phenotypes including abnormal nuclear morphology, loss of peripheral heterochromatin, transcriptional changes, DNA replication defects, DNA damage and premature cellular senescence. A key challenge is to elucidate how these different phenotypes are causally and mechanistically linked. In this mini-review, we highlight some key findings and present a model on how progerin-induced phenotypes may be temporally and mechanistically linked.<\/jats:p>","DOI":"10.1042\/bst20190882","type":"journal-article","created":{"date-parts":[[2020,6,15]],"date-time":"2020-06-15T10:09:25Z","timestamp":1592215765000},"page":"981-991","update-policy":"https:\/\/doi.org\/10.1042\/crossmark_policy","source":"Crossref","is-referenced-by-count":23,"title":["Towards delineating the chain of events that cause premature senescence in the accelerated aging syndrome Hutchinson\u2013Gilford progeria (HGPS)"],"prefix":"10.1042","volume":"48","author":[{"ORCID":"https:\/\/orcid.org\/0000-0003-1148-3557","authenticated-orcid":false,"given":"Oliver","family":"Dreesen","sequence":"first","affiliation":[{"name":"Cell Ageing Laboratory, Skin Research Institute of Singapore, 8A Biomedical Grove, #06-06 Immunos, 138648 Singapore"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"288","published-online":{"date-parts":[[2020,6,15]]},"reference":[{"key":"2021111916013700700_BST-2019-0882CC1","doi-asserted-by":"publisher","first-page":"1194","DOI":"10.1016\/j.cell.2013.05.039","article-title":"The hallmarks of aging","volume":"153","year":"2013","journal-title":"Cell"},{"key":"2021111916013700700_BST-2019-0882CC2","doi-asserted-by":"publisher","first-page":"17","DOI":"10.1016\/j.ceb.2016.12.005","article-title":"A-type lamins and cardiovascular disease in premature aging syndromes","volume":"46","year":"2017","journal-title":"Curr. Opin. Cell Biol."},{"key":"2021111916013700700_BST-2019-0882CC3","doi-asserted-by":"publisher","first-page":"401","DOI":"10.1007\/s00418-016-1411-1","article-title":"Molecular insights into the premature aging disease progeria","volume":"145","year":"2016","journal-title":"Histochem. Cell Biol."},{"key":"2021111916013700700_BST-2019-0882CC4","doi-asserted-by":"publisher","first-page":"595","DOI":"10.1038\/nrm.2017.68","article-title":"Shared molecular and cellular mechanisms of premature ageing and ageing-associated diseases","volume":"18","year":"2017","journal-title":"Nat. Rev. Mol. Cell Biol."},{"key":"2021111916013700700_BST-2019-0882CC5","doi-asserted-by":"publisher","first-page":"889","DOI":"10.18632\/aging.100383","article-title":"Accelerated aging syndromes, are they relevant to normal human aging?","volume":"3","year":"2011","journal-title":"Aging (Albany NY)"},{"key":"2021111916013700700_BST-2019-0882CC6","doi-asserted-by":"publisher","first-page":"719","DOI":"10.1242\/dmm.024711","article-title":"Hallmarks of progeroid syndromes: lessons from mice and reprogrammed cells","volume":"9","year":"2016","journal-title":"Dis. Model Mech."},{"key":"2021111916013700700_BST-2019-0882CC7","doi-asserted-by":"publisher","first-page":"58","DOI":"10.1016\/j.jdermsci.2019.10.003","article-title":"Premature aging syndromes: from patients to mechanism","volume":"96","year":"2019","journal-title":"J. Dermatol. Sci."},{"key":"2021111916013700700_BST-2019-0882CC8","doi-asserted-by":"publisher","first-page":"592","DOI":"10.1056\/NEJMoa0706898","article-title":"Phenotype and course of Hutchinson\u2013Gilford progeria syndrome","volume":"358","year":"2008","journal-title":"N. Engl. J. Med."},{"key":"2021111916013700700_BST-2019-0882CC9","doi-asserted-by":"publisher","first-page":"13","DOI":"10.1038\/nrm3488","article-title":"The nuclear lamins: flexibility in function","volume":"14","year":"2013","journal-title":"Nat. Rev. Mol. Cell Biol."},{"key":"2021111916013700700_BST-2019-0882CC10","doi-asserted-by":"publisher","first-page":"24","DOI":"10.1016\/j.jsb.2011.11.007","article-title":"Nuclear lamina at the crossroads of the cytoplasm and nucleus","volume":"177","year":"2012","journal-title":"J. Struct. Biol."},{"key":"2021111916013700700_BST-2019-0882CC11","doi-asserted-by":"publisher","first-page":"261","DOI":"10.1038\/nature21382","article-title":"The molecular architecture of lamins in somatic cells","volume":"543","year":"2017","journal-title":"Nature"},{"key":"2021111916013700700_BST-2019-0882CC12","doi-asserted-by":"publisher","first-page":"560","DOI":"10.1038\/323560a0","article-title":"The nuclear lamina is a meshwork of intermediate-type filaments","volume":"323","year":"1986","journal-title":"Nature"},{"key":"2021111916013700700_BST-2019-0882CC13","doi-asserted-by":"publisher","first-page":"1","DOI":"10.1016\/j.ceb.2015.03.005","article-title":"Networking in the nucleus: a spotlight on LEM-domain proteins","volume":"34","year":"2015","journal-title":"Curr. Opin. Cell Biol."},{"key":"2021111916013700700_BST-2019-0882CC14","doi-asserted-by":"publisher","first-page":"6450","DOI":"10.1073\/pnas.83.17.6450","article-title":"cDNA sequencing of nuclear lamins A and C reveals primary and secondary structural homology to intermediate filament proteins","volume":"83","year":"1986","journal-title":"Proc. Natl. Acad. Sci. U.S.A."},{"key":"2021111916013700700_BST-2019-0882CC15","doi-asserted-by":"publisher","first-page":"463","DOI":"10.1038\/319463a0","article-title":"Homologies in both primary and secondary structure between nuclear envelope and intermediate filament proteins","volume":"319","year":"1986","journal-title":"Nature"},{"key":"2021111916013700700_BST-2019-0882CC16","doi-asserted-by":"publisher","first-page":"505","DOI":"10.1007\/s004180050138","article-title":"A- and B-type lamins are differentially expressed in normal human tissues","volume":"107","year":"1997","journal-title":"Histochem. Cell Biol."},{"key":"2021111916013700700_BST-2019-0882CC17","doi-asserted-by":"publisher","first-page":"1307","DOI":"10.1161\/CIRCRESAHA.108.173989","article-title":"Nuclear shape, mechanics, and mechanotransduction","volume":"102","year":"2008","journal-title":"Circ. Res."},{"key":"2021111916013700700_BST-2019-0882CC18","doi-asserted-by":"publisher","first-page":"699","DOI":"10.1016\/j.devcel.2020.02.011","article-title":"Phosphorylated lamin A\/C in the nuclear interior binds active enhancers associated with abnormal transcription in progeria","volume":"52","year":"2020","journal-title":"Dev. Cell"},{"key":"2021111916013700700_BST-2019-0882CC19","doi-asserted-by":"crossref","first-page":"365","DOI":"10.1242\/dev.105.2.365","article-title":"Differential timing of nuclear lamin A\/C expression in the various organs of the mouse embryo and the young animal: a developmental study","volume":"105","year":"1989","journal-title":"Development"},{"key":"2021111916013700700_BST-2019-0882CC20","doi-asserted-by":"publisher","first-page":"577","DOI":"10.1083\/jcb.105.1.577","article-title":"Differential expression of nuclear lamin proteins during chicken development","volume":"105","year":"1987","journal-title":"J. Cell Biol."},{"key":"2021111916013700700_BST-2019-0882CC21","doi-asserted-by":"publisher","first-page":"1240104","DOI":"10.1126\/science.1240104","article-title":"Nuclear lamin-A scales with tissue stiffness and enhances matrix-directed differentiation","volume":"341","year":"2013","journal-title":"Science"},{"key":"2021111916013700700_BST-2019-0882CC22","doi-asserted-by":"publisher","first-page":"31","DOI":"10.1016\/j.stem.2010.12.002","article-title":"A human iPSC model of hutchinson Gilford progeria reveals vascular smooth muscle and mesenchymal stem cell defects","volume":"8","year":"2011","journal-title":"Cell Stem Cell"},{"key":"2021111916013700700_BST-2019-0882CC23","doi-asserted-by":"publisher","first-page":"e07759","DOI":"10.7554\/eLife.07759","article-title":"Progerin reduces LAP2a-telomere association in Hutchinson\u2013Gilford progeria","volume":"4","year":"2015","journal-title":"eLife"},{"key":"2021111916013700700_BST-2019-0882CC24","doi-asserted-by":"publisher","first-page":"2531","DOI":"10.1194\/jlr.R500011-JLR200","article-title":"Zmpste24, misshapen cell nuclei, and progeria\u2013new evidence suggesting that protein farnesylation could be important for disease pathogenesis","volume":"46","year":"2005","journal-title":"J. Lipid Res."},{"key":"2021111916013700700_BST-2019-0882CC25","doi-asserted-by":"publisher","first-page":"3265","DOI":"10.1242\/jcs.03156","article-title":"Farnesylated lamins, progeroid syndromes and farnesyl transferase inhibitors","volume":"119","year":"2006","journal-title":"J. Cell Sci."},{"key":"2021111916013700700_BST-2019-0882CC26","doi-asserted-by":"publisher","first-page":"969","DOI":"10.1016\/0092-8674(89)90753-8","article-title":"The CaaX motif of lamin A functions in conjunction with the nuclear localization signal to target assembly to the nuclear envelope","volume":"59","year":"1989","journal-title":"Cell"},{"key":"2021111916013700700_BST-2019-0882CC27","doi-asserted-by":"publisher","first-page":"533","DOI":"10.1101\/sqb.2010.75.018","article-title":"Lamina-independent lamins in the nuclear interior serve important functions","volume":"75","year":"2010","journal-title":"Cold Spring. Harb. Symp. Quant. Biol."},{"key":"2021111916013700700_BST-2019-0882CC28","doi-asserted-by":"publisher","first-page":"66","DOI":"10.4161\/nucl.28068","article-title":"Nuclear localization signal deletion mutants of lamin A and progerin reveal insights into lamin A processing and emerin targeting","volume":"5","year":"2014","journal-title":"Nucleus"},{"key":"2021111916013700700_BST-2019-0882CC29","doi-asserted-by":"publisher","first-page":"1365","DOI":"10.1016\/j.cell.2013.02.015","article-title":"When lamins go bad: nuclear structure and disease","volume":"152","year":"2013","journal-title":"Cell"},{"key":"2021111916013700700_BST-2019-0882CC30","doi-asserted-by":"publisher","first-page":"27","DOI":"10.1146\/annurev-physiol-021317-121454","article-title":"Aging in the cardiovascular system: lessons from Hutchinson\u2013Gilford progeria syndrome","volume":"80","year":"2018","journal-title":"Annu. Rev. Physiol."},{"key":"2021111916013700700_BST-2019-0882CC31","doi-asserted-by":"publisher","first-page":"293","DOI":"10.1038\/nature01629","article-title":"Recurrent de novo point mutations in lamin A cause Hutchinson\u2013Gilford progeria syndrome","volume":"423","year":"2003","journal-title":"Nature"},{"key":"2021111916013700700_BST-2019-0882CC32","doi-asserted-by":"publisher","first-page":"2055","DOI":"10.1126\/science.1084125","article-title":"Lamin A truncation in Hutchinson\u2013Gilford progeria","volume":"300","year":"2003","journal-title":"Science"},{"key":"2021111916013700700_BST-2019-0882CC33","doi-asserted-by":"publisher","first-page":"1503","DOI":"10.1093\/hmg\/ddi159","article-title":"Loss of ZMPSTE24 (FACE-1) causes autosomal recessive restrictive dermopathy and accumulation of lamin A precursors","volume":"14","year":"2005","journal-title":"Hum. Mol. Genet."},{"key":"2021111916013700700_BST-2019-0882CC34","doi-asserted-by":"publisher","first-page":"1","DOI":"10.1016\/j.arr.2017.12.001","article-title":"Mandibuloacral dysplasia: a premature ageing disease with aspects of physiological ageing","volume":"42","year":"2018","journal-title":"Ageing Res. Rev."},{"key":"2021111916013700700_BST-2019-0882CC35","doi-asserted-by":"publisher","first-page":"8963","DOI":"10.1073\/pnas.0402943101","article-title":"Accumulation of mutant lamin A causes progressive changes in nuclear architecture in Hutchinson\u2013Gilford progeria syndrome","volume":"101","year":"2004","journal-title":"Proc. Natl. Acad. Sci. U.S.A."},{"key":"2021111916013700700_BST-2019-0882CC36","doi-asserted-by":"publisher","first-page":"8703","DOI":"10.1073\/pnas.0602569103","article-title":"Mutant nuclear lamin A leads to progressive alterations of epigenetic control in premature aging","volume":"103","year":"2006","journal-title":"Proc. Natl. Acad. Sci. U.S.A."},{"key":"2021111916013700700_BST-2019-0882CC37","doi-asserted-by":"publisher","first-page":"1059","DOI":"10.1126\/science.1127168","article-title":"Lamin A-dependent nuclear defects in human aging","volume":"312","year":"2006","journal-title":"Science"},{"key":"2021111916013700700_BST-2019-0882CC38","doi-asserted-by":"publisher","first-page":"780","DOI":"10.1038\/nm1266","article-title":"Genomic instability in laminopathy-based premature aging","volume":"11","year":"2005","journal-title":"Nat. Med."},{"key":"2021111916013700700_BST-2019-0882CC39","doi-asserted-by":"publisher","first-page":"4644","DOI":"10.1242\/jcs.03263","article-title":"DNA damage responses in progeroid syndromes arise from defective maturation of prelamin A","volume":"119","year":"2006","journal-title":"J. Cell Sci."},{"key":"2021111916013700700_BST-2019-0882CC40","doi-asserted-by":"publisher","first-page":"717","DOI":"10.1016\/j.exger.2004.02.002","article-title":"Aging of Hutchinson\u2013Gilford progeria syndrome fibroblasts is characterised by hyperproliferation and increased apoptosis","volume":"39","year":"2004","journal-title":"Exp. Gerontol."},{"key":"2021111916013700700_BST-2019-0882CC41","doi-asserted-by":"publisher","first-page":"10114","DOI":"10.1073\/pnas.89.21.10114","article-title":"Telomere length predicts replicative capacity of human fibroblasts","volume":"89","year":"1992","journal-title":"Proc. Natl. Acad. Sci. U.S.A."},{"key":"2021111916013700700_BST-2019-0882CC42","doi-asserted-by":"publisher","first-page":"377","DOI":"10.1016\/j.mad.2009.03.001","article-title":"Telomere length in Hutchinson\u2013Gilford progeria syndrome","volume":"130","year":"2009","journal-title":"Mech. Ageing Dev."},{"key":"2021111916013700700_BST-2019-0882CC43","doi-asserted-by":"publisher","first-page":"10271","DOI":"10.1073\/pnas.0601058103","article-title":"Distinct structural and mechanical properties of the nuclear lamina in Hutchinson\u2013Gilford progeria syndrome","volume":"103","year":"2006","journal-title":"Proc. Natl. Acad. Sci. U.S.A."},{"key":"2021111916013700700_BST-2019-0882CC44","doi-asserted-by":"publisher","first-page":"25768","DOI":"10.1074\/jbc.M513511200","article-title":"Lamins A and C but not lamin B1 regulate nuclear mechanics","volume":"281","year":"2006","journal-title":"J. Biol. Chem."},{"key":"2021111916013700700_BST-2019-0882CC45","doi-asserted-by":"publisher","first-page":"383","DOI":"10.1111\/j.1474-9726.2008.00382.x","article-title":"Increased mechanosensitivity and nuclear stiffness in Hutchinson\u2013Gilford progeria cells: effects of farnesyltransferase inhibitors","volume":"7","year":"2008","journal-title":"Aging Cell"},{"key":"2021111916013700700_BST-2019-0882CC46","doi-asserted-by":"publisher","first-page":"6412","DOI":"10.1039\/C5SM00521C","article-title":"Nuclear stiffening and chromatin softening with progerin expression leads to an attenuated nuclear response to force","volume":"11","year":"2015","journal-title":"Soft Matter"},{"key":"2021111916013700700_BST-2019-0882CC47","doi-asserted-by":"publisher","first-page":"260","DOI":"10.1101\/gr.138032.112","article-title":"Correlated alterations in genome organization, histone methylation, and DNA-lamin A\/C interactions in Hutchinson\u2013Gilford progeria syndrome","volume":"23","year":"2013","journal-title":"Genome Res."},{"key":"2021111916013700700_BST-2019-0882CC48","doi-asserted-by":"publisher","first-page":"e13108","DOI":"10.1111\/acel.13108","article-title":"Heterochromatin loss as a determinant of progerin-induced DNA damage in Hutchinson\u2013Gilford progeria","volume":"19","year":"2020","journal-title":"Aging Cell"},{"key":"2021111916013700700_BST-2019-0882CC49","doi-asserted-by":"publisher","first-page":"1261","DOI":"10.1038\/ncb1971","article-title":"Ageing-related chromatin defects through loss of the NURD complex","volume":"11","year":"2009","journal-title":"Nat. Cell Biol."},{"key":"2021111916013700700_BST-2019-0882CC50","doi-asserted-by":"publisher","first-page":"292","DOI":"10.1038\/ncb2170","article-title":"Interplay between oncogene-induced DNA damage response and heterochromatin in senescence and cancer","volume":"13","year":"2011","journal-title":"Nat. Cell Biol."},{"key":"2021111916013700700_BST-2019-0882CC51","doi-asserted-by":"publisher","first-page":"975","DOI":"10.1101\/gad.1742509","article-title":"Polycomb protein Ezh2 regulates pancreatic \u03b2-cell Ink4a\/Arf expression and proliferation in aging","volume":"23","year":"2009","journal-title":"Genes Dev."},{"key":"2021111916013700700_BST-2019-0882CC52","doi-asserted-by":"publisher","first-page":"2272","DOI":"10.1016\/j.jid.2019.05.005","article-title":"Accumulation of progerin affects the symmetry of cell division and is associated with impaired Wnt signaling and the mislocalization of nuclear envelope proteins","volume":"139","year":"2019","journal-title":"J. Invest. Dermatol."},{"key":"2021111916013700700_BST-2019-0882CC53","doi-asserted-by":"publisher","first-page":"440","DOI":"10.1038\/nm1204","article-title":"Reversal of the cellular phenotype in the premature aging disease Hutchinson\u2013Gilford progeria syndrome","volume":"11","year":"2005","journal-title":"Nat. Med."},{"key":"2021111916013700700_BST-2019-0882CC54","doi-asserted-by":"publisher","first-page":"150","DOI":"10.1152\/physiolgenomics.00060.2005","article-title":"Alterations of nuclear envelope and chromatin organization in mandibuloacral dysplasia, a rare form of laminopathy","volume":"23","year":"2005","journal-title":"Physiol. Genom."},{"key":"2021111916013700700_BST-2019-0882CC55","doi-asserted-by":"publisher","first-page":"1149","DOI":"10.1002\/jcb.21467","article-title":"Pre-lamin A processing is linked to heterochromatin organization","volume":"102","year":"2007","journal-title":"J. Cell Biochem."},{"key":"2021111916013700700_BST-2019-0882CC56","doi-asserted-by":"publisher","first-page":"2414","DOI":"10.1038\/emboj.2009.196","article-title":"Novel roles for A-type lamins in telomere biology and the DNA damage response pathway","volume":"28","year":"2009","journal-title":"EMBO J."},{"key":"2021111916013700700_BST-2019-0882CC57","doi-asserted-by":"publisher","first-page":"289","DOI":"10.1080\/19491034.2018.1454168","article-title":"Causes and consequences of genomic instability in laminopathies: Replication stress and interferon response","volume":"9","year":"2018","journal-title":"Nucleus"},{"key":"2021111916013700700_BST-2019-0882CC58","doi-asserted-by":"publisher","first-page":"2605","DOI":"10.1242\/jcs.067306","article-title":"Role of progerin-induced telomere dysfunction in HGPS premature cellular senescence","volume":"123","year":"2010","journal-title":"J. Cell Sci."},{"key":"2021111916013700700_BST-2019-0882CC59","doi-asserted-by":"publisher","first-page":"1039","DOI":"10.1111\/acel.12506","article-title":"Prelamin A impairs 53BP1 nuclear entry by mislocalizing NUP153 and disrupting the Ran gradient","volume":"15","year":"2016","journal-title":"Aging Cell"},{"key":"2021111916013700700_BST-2019-0882CC60","doi-asserted-by":"publisher","first-page":"1210","DOI":"10.1128\/MCB.01676-12","article-title":"Lamin A\/C depletion enhances DNA damage-induced stalled replication fork arrest","volume":"33","year":"2013","journal-title":"Mol. Cell. Biol."},{"key":"2021111916013700700_BST-2019-0882CC61","doi-asserted-by":"publisher","first-page":"1","DOI":"10.1128\/MCB.00659-16","article-title":"Progerin-induced replication stress facilitates premature senescence in Hutchinson\u2013Gilford progeria syndrome","volume":"37","year":"2017","journal-title":"Mol. Cell. Biol."},{"key":"2021111916013700700_BST-2019-0882CC62","doi-asserted-by":"publisher","first-page":"498","DOI":"10.1080\/19491034.2016.1239685","article-title":"Disruption of PCNA-lamins A\/C interactions by prelamin A induces DNA replication fork stalling","volume":"7","year":"2016","journal-title":"Nucleus"},{"key":"2021111916013700700_BST-2019-0882CC63","doi-asserted-by":"publisher","first-page":"3882","DOI":"10.1096\/fj.201700014R","article-title":"Progerin sequestration of PCNA promotes replication fork collapse and mislocalization of XPA in laminopathy-related progeroid syndromes","volume":"31","year":"2017","journal-title":"FASEB J."},{"key":"2021111916013700700_BST-2019-0882CC64","doi-asserted-by":"publisher","first-page":"1764","DOI":"10.1042\/BST20110687","article-title":"DNA-damage accumulation and replicative arrest in Hutchinson\u2013Gilford progeria syndrome","volume":"39","year":"2011","journal-title":"Biochem. Soc. Trans."},{"key":"2021111916013700700_BST-2019-0882CC65","doi-asserted-by":"publisher","first-page":"2006","DOI":"10.1016\/j.celrep.2018.01.090","article-title":"A cell-intrinsic interferon-like response links replication stress to cellular aging caused by progerin","volume":"22","year":"2018","journal-title":"Cell Rep."},{"key":"2021111916013700700_BST-2019-0882CC66","doi-asserted-by":"publisher","first-page":"a010132","DOI":"10.1101\/cshperspect.a010132","article-title":"DNA replication timing","volume":"5","year":"2013","journal-title":"Cold Spring. Harb. Perspect. Med."},{"key":"2021111916013700700_BST-2019-0882CC67","doi-asserted-by":"publisher","first-page":"e1000920","DOI":"10.1371\/journal.pgen.1000920","article-title":"Replication timing of human telomeres is chromosome arm-specific, influenced by subtelomeric structures and connected to nuclear localization","volume":"6","year":"2010","journal-title":"PLoS Genet."},{"key":"2021111916013700700_BST-2019-0882CC68","doi-asserted-by":"publisher","first-page":"240","DOI":"10.1038\/415240a","article-title":"Myocyte renewal and ventricular remodelling","volume":"415","year":"2002","journal-title":"Nature"},{"key":"2021111916013700700_BST-2019-0882CC69","doi-asserted-by":"publisher","first-page":"98","DOI":"10.1126\/science.1164680","article-title":"Evidence for cardiomyocyte renewal in humans","volume":"324","year":"2009","journal-title":"Science"},{"key":"2021111916013700700_BST-2019-0882CC70","doi-asserted-by":"publisher","first-page":"1566","DOI":"10.1016\/j.cell.2015.05.026","article-title":"Dynamics of cell generation and turnover in the human heart","volume":"161","year":"2015","journal-title":"Cell"},{"key":"2021111916013700700_BST-2019-0882CC71","doi-asserted-by":"publisher","first-page":"1446","DOI":"10.1073\/pnas.1214608110","article-title":"Cardiomyocyte proliferation contributes to heart growth in young humans","volume":"110","year":"2013","journal-title":"Proc. Natl. Acad. Sci. U.S.A."},{"key":"2021111916013700700_BST-2019-0882CC72","doi-asserted-by":"publisher","first-page":"2100","DOI":"10.1101\/gad.1346005","article-title":"Shelterin: the protein complex that shapes and safeguards human telomeres","volume":"19","year":"2005","journal-title":"Genes Dev."},{"key":"2021111916013700700_BST-2019-0882CC73","doi-asserted-by":"publisher","first-page":"45","DOI":"10.1146\/annurev-genom-082908-150046","article-title":"Syndromes of telomere shortening","volume":"10","year":"2009","journal-title":"Annu. Rev. Genom. Hum. Genet."},{"key":"2021111916013700700_BST-2019-0882CC74","doi-asserted-by":"publisher","first-page":"52","DOI":"10.1016\/j.arr.2016.05.009","article-title":"Telomere-associated aging disorders","volume":"33","year":"2017","journal-title":"Ageing Res. Rev."},{"key":"2021111916013700700_BST-2019-0882CC75","doi-asserted-by":"publisher","first-page":"1521","DOI":"10.1016\/j.celrep.2012.11.019","article-title":"Human telomeres are tethered to the nuclear envelope during postmitotic nuclear assembly","volume":"2","year":"2012","journal-title":"Cell Rep."},{"key":"2021111916013700700_BST-2019-0882CC76","doi-asserted-by":"publisher","first-page":"5467","DOI":"10.1038\/ncomms6467","article-title":"TRF2 and lamin A\/C interact to facilitate the functional organization of chromosome ends","volume":"5","year":"2014","journal-title":"Nat. Commun."},{"key":"2021111916013700700_BST-2019-0882CC77","doi-asserted-by":"publisher","first-page":"6117","DOI":"10.1242\/jcs.01529","article-title":"LAP2alpha and BAF transiently localize to telomeres and specific regions on chromatin during nuclear assembly","volume":"117","year":"2004","journal-title":"J. Cell Sci."},{"key":"2021111916013700700_BST-2019-0882CC78","doi-asserted-by":"publisher","first-page":"160103","DOI":"10.1098\/rsob.160103","article-title":"The telomeric protein AKTIP interacts with A- and B-type lamins and is involved in regulation of cellular senescence","volume":"6","year":"2016","journal-title":"Open Biol."},{"key":"2021111916013700700_BST-2019-0882CC79","doi-asserted-by":"publisher","first-page":"5238","DOI":"10.1091\/mbc.e08-05-0492","article-title":"Suppression of proliferative defects associated with processing-defective lamin A mutants by hTERT or inactivation of p53","volume":"19","year":"2008","journal-title":"Mol. Biol. Cell"},{"key":"2021111916013700700_BST-2019-0882CC80","doi-asserted-by":"publisher","first-page":"e12979","DOI":"10.1111\/acel.12979","article-title":"Transient introduction of human telomerase mRNA improves hallmarks of progeria cells","volume":"18","year":"2019","journal-title":"Aging Cell"},{"key":"2021111916013700700_BST-2019-0882CC81","doi-asserted-by":"publisher","first-page":"691","DOI":"10.1016\/j.stem.2013.11.006","article-title":"Human iPSC-based modeling of late-onset disease via progerin-induced aging","volume":"13","year":"2013","journal-title":"Cell Stem Cell"},{"key":"2021111916013700700_BST-2019-0882CC82","doi-asserted-by":"publisher","first-page":"173","DOI":"10.1002\/(SICI)1520-6408(1996)18:2&lt;173::AID-DVG10&gt;3.0.CO;2-3","article-title":"Telomerase activity in human germline and embryonic tissues and cells","volume":"18","year":"1996","journal-title":"Dev. Genet."},{"key":"2021111916013700700_BST-2019-0882CC83","doi-asserted-by":"publisher","first-page":"371","DOI":"10.1016\/j.bbadis.2009.01.010","article-title":"Dyskeratosis congenita, stem cells and telomeres","volume":"1792","year":"2009","journal-title":"Biochim. Biophys. Acta"},{"key":"2021111916013700700_BST-2019-0882CC84","doi-asserted-by":"publisher","first-page":"447","DOI":"10.1038\/ng1346","article-title":"Disease anticipation is associated with progressive telomere shortening in families with dyskeratosis congenita due to mutations in TERC","volume":"36","year":"2004","journal-title":"Nat. Genet."},{"key":"2021111916013700700_BST-2019-0882CC85","doi-asserted-by":"publisher","first-page":"4990","DOI":"10.1038\/s41467-019-13018-3","article-title":"Sequence-specific inhibition of DNA damage response at telomeres improves the detrimental phenotypes of Hutchinson\u2013Gilford progeria syndrome","volume":"10","year":"2019","journal-title":"Nat. Commun."},{"key":"2021111916013700700_BST-2019-0882CC86","doi-asserted-by":"publisher","first-page":"194","DOI":"10.1038\/nature02118","article-title":"A DNA damage checkpoint response in telomere-initiated senescence","volume":"426","year":"2003","journal-title":"Nature"},{"key":"2021111916013700700_BST-2019-0882CC87","doi-asserted-by":"publisher","first-page":"106ra107","DOI":"10.1126\/scitranslmed.3002847","article-title":"Splicing-directed therapy in a new mouse model of human accelerated aging","volume":"3","year":"2011","journal-title":"Sci. Transl. Med."},{"key":"2021111916013700700_BST-2019-0882CC88","doi-asserted-by":"publisher","first-page":"423","DOI":"10.1038\/s41591-018-0338-6","article-title":"Development of a CRISPR\/Cas9-based therapy for Hutchinson\u2013Gilford progeria syndrome","volume":"25","year":"2019","journal-title":"Nat. Med."}],"container-title":["Biochemical Society Transactions"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/portlandpress.com\/biochemsoctrans\/article-pdf\/48\/3\/981\/886887\/bst-2019-0882c.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"syndication"},{"URL":"https:\/\/portlandpress.com\/biochemsoctrans\/article-pdf\/48\/3\/981\/886887\/bst-2019-0882c.pdf","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2021,11,19]],"date-time":"2021-11-19T11:22:21Z","timestamp":1637320941000},"score":1,"resource":{"primary":{"URL":"https:\/\/portlandpress.com\/biochemsoctrans\/article\/48\/3\/981\/225298\/Towards-delineating-the-chain-of-events-that-cause"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2020,6,15]]},"references-count":88,"journal-issue":{"issue":"3","published-online":{"date-parts":[[2020,6,15]]},"published-print":{"date-parts":[[2020,6,30]]}},"URL":"https:\/\/doi.org\/10.1042\/bst20190882","relation":{},"ISSN":["0300-5127","1470-8752"],"issn-type":[{"value":"0300-5127","type":"print"},{"value":"1470-8752","type":"electronic"}],"subject":[],"published":{"date-parts":[[2020,6,15]]}}}