{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,22]],"date-time":"2026-10-22T11:06:43Z","timestamp":1792667203409,"version":"4.5.0"},"reference-count":73,"publisher":"Elsevier BV","license":[{"start":{"date-parts":[[2020,12,1]],"date-time":"2020-12-01T00:00:00Z","timestamp":1606780800000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/www.elsevier.com\/tdm\/userlicense\/1.0\/"},{"start":{"date-parts":[[2020,12,1]],"date-time":"2020-12-01T00:00:00Z","timestamp":1606780800000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/www.elsevier.com\/legal\/tdmrep-license"}],"funder":[{"DOI":"10.13039\/501100002945","name":"Direcci\u00f3n de Investigaci\u00f3n, Universidad Nacional de Colombia","doi-asserted-by":"publisher","id":[{"id":"10.13039\/501100002945","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":["elsevier.com","sciencedirect.com"],"crossmark-restriction":true},"short-container-title":["Mechanisms of Ageing and Development"],"published-print":{"date-parts":[[2020,12]]},"DOI":"10.1016\/j.mad.2020.111360","type":"journal-article","created":{"date-parts":[[2020,9,22]],"date-time":"2020-09-22T16:55:42Z","timestamp":1600793742000},"page":"111360","update-policy":"https:\/\/doi.org\/10.1016\/elsevier_cm_policy","source":"Crossref","is-referenced-by-count":24,"special_numbering":"C","title":["Nucleolar disruption, activation of P53 and premature senescence in POLR3A-mutated Wiedemann-Rautenstrauch syndrome fibroblasts"],"prefix":"10.1016","volume":"192","author":[{"given":"Cindy Tatiana","family":"B\u00e1ez-Becerra","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Estefania","family":"Valencia-Rinc\u00f3n","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Karen","family":"Vel\u00e1squez-M\u00e9ndez","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Nelson J.","family":"Ram\u00edrez-Su\u00e1rez","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Claudia","family":"Guevara","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Adrian","family":"Sandoval-Hernandez","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Carlos E.","family":"Arboleda-Bustos","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Leonora","family":"Olivos-Cisneros","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Gabriel","family":"Guti\u00e9rrez-Ospina","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Humberto","family":"Arboleda","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Gonzalo","family":"Arboleda","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"78","reference":[{"key":"10.1016\/j.mad.2020.111360_bib0005","first-page":"4417","article-title":"Hutchinson-gilford progeria syndrome: a premature aging disease","volume":"55","author":"Ahmed","year":"2018","journal-title":"Mol. Neurobiol."},{"key":"10.1016\/j.mad.2020.111360_bib0010","doi-asserted-by":"crossref","first-page":"562","DOI":"10.1002\/bdra.20166","article-title":"Follow-up study of Wiedemann-Rautenstrauch syndrome: long-term survival and comparison with Rautenstrauch\u2019s patient \u201cG\u201d","volume":"73","author":"Arboleda","year":"2005","journal-title":"Birth Defects Res. A Clin. Mol. Teratol."},{"key":"10.1016\/j.mad.2020.111360_bib0015","doi-asserted-by":"crossref","first-page":"1712","DOI":"10.1002\/ajmg.a.34019","article-title":"Neonatal progeroid syndrome (Wiedemann-Rautenstrauch syndrome): report of three affected sibs","volume":"155A","author":"Arboleda","year":"2011","journal-title":"Am. J. Med. Genet. A"},{"key":"10.1016\/j.mad.2020.111360_bib0020","doi-asserted-by":"crossref","first-page":"546","DOI":"10.1016\/j.tibs.2016.03.003","article-title":"RNA polymerase III advances: structural and tRNA functional views","volume":"41","author":"Arimbasseri","year":"2016","journal-title":"Trends Biochem. Sci."},{"key":"10.1016\/j.mad.2020.111360_bib0025","doi-asserted-by":"crossref","first-page":"287","DOI":"10.1093\/emph\/eoy033","article-title":"Is antagonistic pleiotropy ubiquitous in aging biology?","author":"Austad","year":"2018","journal-title":"Evol. Med. Public Health"},{"key":"10.1016\/j.mad.2020.111360_bib0030","first-page":"600","article-title":"Recent advances on nucleolar functions in health and disease","volume":"21","author":"Bahadori","year":"2018","journal-title":"Arch. Iran. Med."},{"key":"10.1016\/j.mad.2020.111360_bib0035","doi-asserted-by":"crossref","first-page":"305","DOI":"10.1002\/mrd.1080400306","article-title":"Localization of fibrillarin and nucleolin in nucleoli of mouse preimplantation embryos","volume":"40","author":"Baran","year":"1995","journal-title":"Mol. Reprod. Dev."},{"key":"10.1016\/j.mad.2020.111360_bib0040","doi-asserted-by":"crossref","first-page":"415","DOI":"10.1016\/j.ajhg.2011.07.014","article-title":"Mutations of POLR3A encoding a catalytic subunit of RNA polymerase Pol III cause a recessive hypomyelinating leukodystrophy","volume":"89","author":"Bernard","year":"2011","journal-title":"Am. J. Hum. Genet."},{"key":"10.1016\/j.mad.2020.111360_bib0045","doi-asserted-by":"crossref","first-page":"2873","DOI":"10.1016\/j.ejca.2005.08.025","article-title":"Telomerase inhibition, telomere shortening, cell growth suppression and induction of apoptosis by telomestatin in childhood neuroblastoma cells","volume":"41","author":"Binz","year":"2005","journal-title":"Eur. J. Cancer"},{"key":"10.1016\/j.mad.2020.111360_bib0050","doi-asserted-by":"crossref","first-page":"216","DOI":"10.1016\/j.molcel.2010.09.024","article-title":"The nucleolus under stress","volume":"40","author":"Boulon","year":"2010","journal-title":"Mol. Cell"},{"key":"10.1016\/j.mad.2020.111360_bib0055","doi-asserted-by":"crossref","first-page":"967","DOI":"10.1152\/physrev.00047.2005","article-title":"Nuclear lamins: laminopathies and their role in premature ageing","volume":"86","author":"Broers","year":"2006","journal-title":"Physiol. Rev."},{"key":"10.1016\/j.mad.2020.111360_bib0060","doi-asserted-by":"crossref","first-page":"328","DOI":"10.1038\/s41467-017-00322-z","article-title":"Nucleolar expansion and elevated protein translation in premature aging","volume":"8","author":"Buchwalter","year":"2017","journal-title":"Nat. Commun."},{"key":"10.1016\/j.mad.2020.111360_bib0065","doi-asserted-by":"crossref","first-page":"187","DOI":"10.1080\/19491034.2016.1179409","article-title":"Mammalian telomeres and their partnership with lamins","volume":"7","author":"Burla","year":"2016","journal-title":"Nucleus"},{"key":"10.1016\/j.mad.2020.111360_bib0070","doi-asserted-by":"crossref","first-page":"368","DOI":"10.1080\/19491034.2018.1476793","article-title":"Genomic instability and DNA replication defects in progeroid syndromes","volume":"9","author":"Burla","year":"2018","journal-title":"Nucleus"},{"key":"10.1016\/j.mad.2020.111360_bib0075","doi-asserted-by":"crossref","first-page":"710","DOI":"10.1101\/gr.101337.109","article-title":"Defining the RNA polymerase III transcriptome: genome-wide localization of the RNA polymerase III transcription machinery in human cells","volume":"20","author":"Canella","year":"2010","journal-title":"Genome Res."},{"key":"10.1016\/j.mad.2020.111360_bib0080","doi-asserted-by":"crossref","first-page":"271","DOI":"10.1007\/s10038-003-0025-3","article-title":"LMNA is mutated in Hutchinson-Gilford progeria (MIM 176670) but not in Wiedemann-Rautenstrauch progeroid syndrome (MIM 264090)","volume":"48","author":"Cao","year":"2003","journal-title":"J. Hum. Genet."},{"key":"10.1016\/j.mad.2020.111360_bib0085","doi-asserted-by":"crossref","first-page":"e47","DOI":"10.1093\/nar\/30.10.e47","article-title":"Telomere measurement by quantitative PCR","volume":"30","author":"Cawthon","year":"2002","journal-title":"Nucleic Acids Res."},{"key":"10.1016\/j.mad.2020.111360_bib0090","doi-asserted-by":"crossref","first-page":"31","DOI":"10.1007\/978-1-4939-8931-7_4","article-title":"Cellular identification and quantification of senescence-associated beta-galactosidase activity in vivo","volume":"1896","author":"Childs","year":"2019","journal-title":"Methods Mol. Biol."},{"key":"10.1016\/j.mad.2020.111360_bib0095","doi-asserted-by":"crossref","first-page":"7445","DOI":"10.1074\/jbc.RA118.006271","article-title":"Leukodystrophy-associated POLR3A mutations down-regulate the RNA polymerase III transcript and important regulatory RNA BC200","volume":"294","author":"Choquet","year":"2019","journal-title":"J. Biol. Chem."},{"key":"10.1016\/j.mad.2020.111360_bib0100","doi-asserted-by":"crossref","first-page":"304","DOI":"10.1136\/jmg.2003.015651","article-title":"Novel lamin A\/C gene (LMNA) mutations in atypical progeroid syndromes","volume":"41","author":"Csoka","year":"2004","journal-title":"J. Med. Genet."},{"key":"10.1016\/j.mad.2020.111360_bib0105","doi-asserted-by":"crossref","first-page":"2055","DOI":"10.1126\/science.1084125","article-title":"Lamin a truncation in Hutchinson-Gilford progeria","volume":"300","author":"De Sandre-Giovannoli","year":"2003","journal-title":"Science"},{"key":"10.1016\/j.mad.2020.111360_bib0110","doi-asserted-by":"crossref","first-page":"377","DOI":"10.1016\/j.mad.2009.03.001","article-title":"Telomere length in Hutchinson-Gilford progeria syndrome","volume":"130","author":"Decker","year":"2009","journal-title":"Mech. Ageing Dev."},{"key":"10.1016\/j.mad.2020.111360_bib0115","doi-asserted-by":"crossref","first-page":"9363","DOI":"10.1073\/pnas.92.20.9363","article-title":"A biomarker that identifies senescent human cells in culture and in aging skin in vivo","volume":"92","author":"Dimri","year":"1995","journal-title":"Proc. Natl. Acad. Sci. U. S. A."},{"key":"10.1016\/j.mad.2020.111360_bib0120","doi-asserted-by":"crossref","first-page":"293","DOI":"10.1038\/nature01629","article-title":"Recurrent de novo point mutations in lamin A cause Hutchinson-Gilford progeria syndrome","volume":"423","author":"Eriksson","year":"2003","journal-title":"Nature"},{"key":"10.1016\/j.mad.2020.111360_bib0125","doi-asserted-by":"crossref","first-page":"263","DOI":"10.1038\/nature25007","article-title":"RNA polymerase III limits longevity downstream of TORC1","volume":"552","author":"Filer","year":"2017","journal-title":"Nature"},{"key":"10.1016\/j.mad.2020.111360_bib0130","doi-asserted-by":"crossref","first-page":"4934","DOI":"10.1158\/0008-5472.CAN-16-3541","article-title":"Breast Cancer suppression by progesterone receptors is mediated by their modulation of estrogen receptors and RNA polymerase III","volume":"77","author":"Finlay-Schultz","year":"2017","journal-title":"Cancer Res."},{"issue":"55\u201370","key":"10.1016\/j.mad.2020.111360_bib0135","first-page":"e54","article-title":"Autogenous Control of 5\u2019TOP mRNA Stability by 40S Ribosomes","volume":"67","author":"Gentilella","year":"2017","journal-title":"Mol. Cell"},{"key":"10.1016\/j.mad.2020.111360_bib0140","doi-asserted-by":"crossref","first-page":"169","DOI":"10.1385\/MB:27:2:169","article-title":"Real-time quantitative PCR of telomere length","volume":"27","author":"Gil","year":"2004","journal-title":"Mol. Biotechnol."},{"key":"10.1016\/j.mad.2020.111360_bib0145","doi-asserted-by":"crossref","first-page":"969","DOI":"10.1016\/j.leukres.2004.01.002","article-title":"Real-time quantitative RT-PCR for human telomere elongation reverse transcriptase in chronic myeloid leukemia","volume":"28","author":"Gil","year":"2004","journal-title":"Leuk. Res."},{"key":"10.1016\/j.mad.2020.111360_bib0150","doi-asserted-by":"crossref","first-page":"686","DOI":"10.4161\/cc.5.7.2623","article-title":"Regulation of p14ARF through subnuclear compartmentalization","volume":"5","author":"Gjerset","year":"2006","journal-title":"Cell Cycle"},{"key":"10.1016\/j.mad.2020.111360_bib0155","doi-asserted-by":"crossref","first-page":"8963","DOI":"10.1073\/pnas.0402943101","article-title":"Accumulation of mutant lamin A causes progressive changes in nuclear architecture in Hutchinson-Gilford progeria syndrome","volume":"101","author":"Goldman","year":"2004","journal-title":"Proc. Natl. Acad. Sci. U. S. A."},{"key":"10.1016\/j.mad.2020.111360_bib0160","doi-asserted-by":"crossref","first-page":"1127","DOI":"10.1038\/nature07986","article-title":"Cytoplasmic functions of the tumour suppressor p53","volume":"458","author":"Green","year":"2009","journal-title":"Nature"},{"key":"10.1016\/j.mad.2020.111360_bib0165","doi-asserted-by":"crossref","first-page":"40","DOI":"10.1038\/s41421-018-0044-z","article-title":"Structural visualization of RNA polymerase III transcription machineries","volume":"4","author":"Han","year":"2018","journal-title":"Cell Discov."},{"key":"10.1016\/j.mad.2020.111360_bib0170","doi-asserted-by":"crossref","first-page":"415","DOI":"10.1002\/wrna.39","article-title":"The nucleolus: structure\/function relationship in RNA metabolism","volume":"1","author":"Hernandez-Verdun","year":"2010","journal-title":"Wiley Interdiscip. Rev. RNA"},{"key":"10.1016\/j.mad.2020.111360_bib0175","doi-asserted-by":"crossref","first-page":"219","DOI":"10.1038\/329219a0","article-title":"Functional inactivation of genes by dominant negative mutations","volume":"329","author":"Herskowitz","year":"1987","journal-title":"Nature"},{"key":"10.1016\/j.mad.2020.111360_bib0180","doi-asserted-by":"crossref","first-page":"231","DOI":"10.1038\/nature16143","article-title":"Molecular structures of unbound and transcribing RNA polymerase III","volume":"528","author":"Hoffmann","year":"2015","journal-title":"Nature"},{"key":"10.1016\/j.mad.2020.111360_bib0185","doi-asserted-by":"crossref","first-page":"197","DOI":"10.1002\/0470093765.ch13","article-title":"LMNA mutations in progeroid syndromes","volume":"264","author":"Huang","year":"2005","journal-title":"Novartis Found. Symp."},{"key":"10.1016\/j.mad.2020.111360_bib0190","doi-asserted-by":"crossref","first-page":"3343","DOI":"10.1002\/ajmg.a.37960","article-title":"Neonatal progeriod syndrome associated with biallelic truncating variants in POLR3A","volume":"170","author":"Jay","year":"2016","journal-title":"Am. J. Med. Genet. A"},{"key":"10.1016\/j.mad.2020.111360_bib0195","first-page":"882","article-title":"Loss of Peter Pan protein is associated with cell cycle defects and apoptotic events. Biochimica et biophysica acta","volume":"1866","author":"Keil","year":"2019","journal-title":"Mol. Cell Res."},{"key":"10.1016\/j.mad.2020.111360_bib0200","doi-asserted-by":"crossref","first-page":"88","DOI":"10.1016\/j.sbi.2017.05.008","article-title":"RNA polymerase I and III: similar yet unique","volume":"47","author":"Khatter","year":"2017","journal-title":"Curr. Opin. Struct. Biol."},{"key":"10.1016\/j.mad.2020.111360_bib0205","doi-asserted-by":"crossref","first-page":"393","DOI":"10.1038\/nrm4007","article-title":"p53 in survival, death and metabolic health: a lifeguard with a licence to kill","volume":"16","author":"Kruiswijk","year":"2015","journal-title":"Nat. Rev. Mol. Cell Biol."},{"key":"10.1016\/j.mad.2020.111360_bib0210","doi-asserted-by":"crossref","first-page":"R652","DOI":"10.1016\/j.cub.2006.07.029","article-title":"Aging: progeria and the lamin connection","volume":"16","author":"Kudlow","year":"2006","journal-title":"Curr. Biol."},{"key":"10.1016\/j.mad.2020.111360_bib0215","doi-asserted-by":"crossref","first-page":"3550","DOI":"10.1016\/S0021-9258(17)34836-6","article-title":"beta-Galactosidase from rat mammary gland. Its purification, properties, and role in the biosynthesis of 6beta-O-D-galactopyranosyl myo-inositol","volume":"253","author":"Kuo","year":"1978","journal-title":"J. Biol. Chem."},{"key":"10.1016\/j.mad.2020.111360_bib0220","first-page":"7","article-title":"Novel layers of RNA polymerase III control affecting tRNA gene transcription in eukaryotes","author":"Lesniewska","year":"2017","journal-title":"Open Biol."},{"key":"10.1016\/j.mad.2020.111360_bib0225","doi-asserted-by":"crossref","first-page":"911","DOI":"10.1038\/nrc2539","article-title":"Non-coding RNA production by RNA polymerase III is implicated in cancer","volume":"8","author":"Marshall","year":"2008","journal-title":"Nat. Rev. Cancer"},{"key":"10.1016\/j.mad.2020.111360_bib0230","first-page":"241","article-title":"Syndromes of accelerated aging","volume":"60","author":"Martin","year":"1982","journal-title":"Cancer Inst. Monogr."},{"key":"10.1016\/j.mad.2020.111360_bib0235","doi-asserted-by":"crossref","first-page":"e1269","DOI":"10.1371\/journal.pone.0001269","article-title":"The mutant form of lamin A that causes Hutchinson-Gilford progeria is a biomarker of cellular aging in human skin","volume":"2","author":"McClintock","year":"2007","journal-title":"PLoS One"},{"key":"10.1016\/j.mad.2020.111360_bib0240","doi-asserted-by":"crossref","first-page":"386","DOI":"10.1107\/S0907444911007281","article-title":"Presenting your structures: the CCP4mg molecular-graphics software. Acta crystallographica. Section D","volume":"67","author":"McNicholas","year":"2011","journal-title":"Biol. Crystallogr."},{"key":"10.1016\/j.mad.2020.111360_bib0245","doi-asserted-by":"crossref","first-page":"2695","DOI":"10.1002\/ajmg.a.33090","article-title":"Absence of Lamin A\/C gene mutations in four Wiedemann-Rautenstrauch syndrome patients","volume":"149A","author":"Morales","year":"2009","journal-title":"Am. J. Med. Genet. A"},{"key":"10.1016\/j.mad.2020.111360_bib0250","doi-asserted-by":"crossref","first-page":"322","DOI":"10.1016\/j.ceb.2004.03.009","article-title":"Aging and nuclear organization: lamins and progeria","volume":"16","author":"Mounkes","year":"2004","journal-title":"Curr. Opin. Cell Biol."},{"key":"10.1016\/j.mad.2020.111360_bib0255","doi-asserted-by":"crossref","first-page":"167","DOI":"10.1080\/19491034.2016.1162933","article-title":"Recent advances in understanding nuclear size and shape","volume":"7","author":"Mukherjee","year":"2016","journal-title":"Nucleus"},{"key":"10.1016\/j.mad.2020.111360_bib0260","doi-asserted-by":"crossref","first-page":"105","DOI":"10.1016\/j.ceb.2018.02.013","article-title":"Dynamic regulation of nucleolar architecture","volume":"52","author":"Nemeth","year":"2018","journal-title":"Curr. Opin. Cell Biol."},{"key":"10.1016\/j.mad.2020.111360_bib0265","doi-asserted-by":"crossref","first-page":"593","DOI":"10.1083\/jcb.200708054","article-title":"Nuclear size control in fission yeast","volume":"179","author":"Neumann","year":"2007","journal-title":"J. Cell Biol."},{"key":"10.1016\/j.mad.2020.111360_bib0270","doi-asserted-by":"crossref","first-page":"488","DOI":"10.1182\/blood-2017-07-746396","article-title":"DNA damage responses and p53 in the aging process","volume":"131","author":"Ou","year":"2018","journal-title":"Blood"},{"key":"10.1016\/j.mad.2020.111360_bib0275","doi-asserted-by":"crossref","DOI":"10.1002\/ajmg.a.38246","article-title":"Wiedemann-Rautenstrauch syndrome: a phenotype analysis","author":"Paolacci","year":"2017","journal-title":"Am. J. Med. Genet. A"},{"key":"10.1016\/j.mad.2020.111360_bib0280","doi-asserted-by":"crossref","first-page":"837","DOI":"10.1136\/jmedgenet-2018-105528","article-title":"Specific combinations of biallelic POLR3A variants cause Wiedemann-Rautenstrauch syndrome","volume":"55","author":"Paolacci","year":"2018","journal-title":"J. Med. Genet."},{"key":"10.1016\/j.mad.2020.111360_bib0285","doi-asserted-by":"crossref","DOI":"10.3390\/cells8020088","article-title":"Hutchinson-gilford progeria syndrome-current status and prospects for gene therapy treatment","volume":"8","author":"Piekarowicz","year":"2019","journal-title":"Cells"},{"key":"10.1016\/j.mad.2020.111360_bib0290","doi-asserted-by":"crossref","DOI":"10.1128\/MCB.00099-19","article-title":"NOL12 repression induces nucleolar stress-driven cellular senescence and is associated with normative aging","author":"Pinho","year":"2019","journal-title":"Mol. Cell. Biol."},{"key":"10.1016\/j.mad.2020.111360_bib0295","doi-asserted-by":"crossref","first-page":"7475","DOI":"10.1093\/nar\/gkm744","article-title":"Two faces of p53: aging and tumor suppression","volume":"35","author":"Rodier","year":"2007","journal-title":"Nucleic Acids Res."},{"key":"10.1016\/j.mad.2020.111360_bib0300","doi-asserted-by":"crossref","first-page":"6068","DOI":"10.1093\/emboj\/cdg579","article-title":"Disruption of the nucleolus mediates stabilization of p53 in response to DNA damage and other stresses","volume":"22","author":"Rubbi","year":"2003","journal-title":"EMBO J."},{"key":"10.1016\/j.mad.2020.111360_bib0305","doi-asserted-by":"crossref","first-page":"440","DOI":"10.1038\/nm1204","article-title":"Reversal of the cellular phenotype in the premature aging disease Hutchinson-Gilford progeria syndrome","volume":"11","author":"Scaffidi","year":"2005","journal-title":"Nat. Med."},{"key":"10.1016\/j.mad.2020.111360_bib0310","doi-asserted-by":"crossref","first-page":"1059","DOI":"10.1126\/science.1127168","article-title":"Lamin A-dependent nuclear defects in human aging","volume":"312","author":"Scaffidi","year":"2006","journal-title":"Science"},{"key":"10.1016\/j.mad.2020.111360_bib0315","first-page":"667","article-title":"Progeria: a rare genetic premature ageing disorder","volume":"139","author":"Sinha","year":"2014","journal-title":"Indian J. Med. Res."},{"key":"10.1016\/j.mad.2020.111360_bib0320","doi-asserted-by":"crossref","first-page":"4015","DOI":"10.1007\/s00018-015-1984-1","article-title":"Emerging roles of nucleolar and ribosomal proteins in cancer, development, and aging","volume":"72","author":"Takada","year":"2015","journal-title":"Cell. Mol. Life Sci."},{"key":"10.1016\/j.mad.2020.111360_bib0325","doi-asserted-by":"crossref","first-page":"662","DOI":"10.1016\/j.tcb.2018.03.007","article-title":"Nucleolar function in lifespan regulation","volume":"28","author":"Tiku","year":"2018","journal-title":"Trends Cell Biol."},{"key":"10.1016\/j.mad.2020.111360_bib0330","doi-asserted-by":"crossref","first-page":"2512","DOI":"10.18632\/aging.101922","article-title":"Impaired ribosome biogenesis: mechanisms and relevance to cancer and aging","volume":"11","author":"Turi","year":"2019","journal-title":"Aging (Albany NY)"},{"key":"10.1016\/j.mad.2020.111360_bib0335","doi-asserted-by":"crossref","first-page":"1367","DOI":"10.1042\/BST20160062","article-title":"Transcription by RNA polymerase III: insights into mechanism and regulation","volume":"44","author":"Turowski","year":"2016","journal-title":"Biochem. Soc. Trans."},{"key":"10.1016\/j.mad.2020.111360_bib0340","doi-asserted-by":"crossref","first-page":"249","DOI":"10.1016\/B978-0-444-62702-5.00018-4","article-title":"Hutchinson-Gilford progeria syndrome","volume":"132","author":"Ullrich","year":"2015","journal-title":"Handb. Clin. Neurol."},{"key":"10.1016\/j.mad.2020.111360_bib0345","doi-asserted-by":"crossref","first-page":"39","DOI":"10.1007\/978-1-4939-8931-7_5","article-title":"Relative human telomere length quantification by real-time PCR","volume":"1896","author":"Vasilishina","year":"2019","journal-title":"Methods Mol. Biol."},{"key":"10.1016\/j.mad.2020.111360_bib0350","doi-asserted-by":"crossref","first-page":"3843","DOI":"10.1105\/tpc.107.055053","article-title":"Exploring the molecular etiology of dominant-negative mutations","volume":"19","author":"Veitia","year":"2007","journal-title":"Plant Cell"},{"key":"10.1016\/j.mad.2020.111360_bib0355","doi-asserted-by":"crossref","first-page":"559","DOI":"10.1016\/j.yexcr.2003.11.025","article-title":"Long-term molecular and cellular stability of human neural stem cell lines","volume":"294","author":"Villa","year":"2004","journal-title":"Exp. Cell Res."},{"key":"10.1016\/j.mad.2020.111360_bib0360","first-page":"37","article-title":"Progerin-induced replication stress facilitates premature senescence in Hutchinson-Gilford progeria syndrome","author":"Wheaton","year":"2017","journal-title":"Mol. Cell. Biol."},{"key":"10.1016\/j.mad.2020.111360_bib0365","doi-asserted-by":"crossref","first-page":"75","DOI":"10.1146\/annurev-biochem-062917-012624","article-title":"Signaling to and from the RNA polymerase III transcription and processing machinery","volume":"87","author":"Willis","year":"2018","journal-title":"Annu. Rev. Biochem."}],"container-title":["Mechanisms of Ageing and Development"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/api.elsevier.com\/content\/article\/PII:S0047637420301561?httpAccept=text\/xml","content-type":"text\/xml","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/api.elsevier.com\/content\/article\/PII:S0047637420301561?httpAccept=text\/plain","content-type":"text\/plain","content-version":"vor","intended-application":"text-mining"}],"deposited":{"date-parts":[[2025,9,23]],"date-time":"2025-09-23T17:43:23Z","timestamp":1758649403000},"score":1,"resource":{"primary":{"URL":"https:\/\/linkinghub.elsevier.com\/retrieve\/pii\/S0047637420301561"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2020,12]]},"references-count":73,"alternative-id":["S0047637420301561"],"URL":"https:\/\/doi.org\/10.1016\/j.mad.2020.111360","relation":{"has-preprint":[{"id-type":"doi","id":"10.1101\/2020.01.29.925131","asserted-by":"object"}]},"ISSN":["0047-6374"],"issn-type":[{"value":"0047-6374","type":"print"}],"subject":[],"published":{"date-parts":[[2020,12]]},"assertion":[{"value":"Elsevier","name":"publisher","label":"This article is maintained by"},{"value":"Nucleolar disruption, activation of P53 and premature senescence in POLR3A-mutated Wiedemann-Rautenstrauch syndrome fibroblasts","name":"articletitle","label":"Article Title"},{"value":"Mechanisms of Ageing and Development","name":"journaltitle","label":"Journal Title"},{"value":"https:\/\/doi.org\/10.1016\/j.mad.2020.111360","name":"articlelink","label":"CrossRef DOI link to publisher maintained version"},{"value":"article","name":"content_type","label":"Content Type"},{"value":"\u00a9 2020 Elsevier B.V. All rights reserved.","name":"copyright","label":"Copyright"}],"article-number":"111360"}}