{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,26]],"date-time":"2026-09-26T03:51:11Z","timestamp":1790394671816,"version":"4.1.0"},"reference-count":42,"publisher":"Elsevier BV","issue":"4","license":[{"start":{"date-parts":[[2019,10,1]],"date-time":"2019-10-01T00:00:00Z","timestamp":1569888000000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/www.elsevier.com\/tdm\/userlicense\/1.0\/"},{"start":{"date-parts":[[2019,10,1]],"date-time":"2019-10-01T00:00:00Z","timestamp":1569888000000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/www.elsevier.com\/legal\/tdmrep-license"},{"start":{"date-parts":[[2019,9,12]],"date-time":"2019-09-12T00:00:00Z","timestamp":1568246400000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"funder":[{"DOI":"10.13039\/100009659","name":"Guangzhou Medical University","doi-asserted-by":"publisher","award":["B195002002044"],"award-info":[{"award-number":["B195002002044"]}],"id":[{"id":"10.13039\/100009659","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/501100001809","name":"National Natural Science Foundation of China","doi-asserted-by":"publisher","award":["81571092"],"award-info":[{"award-number":["81571092"]}],"id":[{"id":"10.13039\/501100001809","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":["cell.com","elsevier.com","sciencedirect.com"],"crossmark-restriction":true},"short-container-title":["Cell Reports"],"published-print":{"date-parts":[[2019,10]]},"DOI":"10.1016\/j.celrep.2019.09.028","type":"journal-article","created":{"date-parts":[[2019,10,22]],"date-time":"2019-10-22T10:41:27Z","timestamp":1571740887000},"page":"800-809.e5","update-policy":"https:\/\/doi.org\/10.1016\/elsevier_cm_policy","source":"Crossref","is-referenced-by-count":24,"title":["Rat Model of Cockayne Syndrome Neurological Disease"],"prefix":"10.1016","volume":"29","author":[{"given":"Yingying","family":"Xu","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Zhenzhen","family":"Wu","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Lingyun","family":"Liu","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jiena","family":"Liu","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Yuming","family":"Wang","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"78","reference":[{"key":"10.1016\/j.celrep.2019.09.028_bib1","doi-asserted-by":"crossref","first-page":"464","DOI":"10.1038\/428464a","article-title":"Laboratory animals: the Renaissance rat","volume":"428","author":"Abbott","year":"2004","journal-title":"Nature"},{"key":"10.1016\/j.celrep.2019.09.028_bib2","doi-asserted-by":"crossref","first-page":"1979","DOI":"10.1093\/brain\/awn126","article-title":"Neurological symptoms and natural course of xeroderma pigmentosum","volume":"131","author":"Anttinen","year":"2008","journal-title":"Brain"},{"key":"10.1016\/j.celrep.2019.09.028_bib3","doi-asserted-by":"crossref","first-page":"488","DOI":"10.1016\/j.dnarep.2012.02.004","article-title":"The conserved Cockayne syndrome B-piggyBac fusion protein (CSB-PGBD3) affects DNA repair and induces both interferon-like and innate antiviral responses in CSB-null cells","volume":"11","author":"Bailey","year":"2012","journal-title":"DNA Repair (Amst.)"},{"key":"10.1016\/j.celrep.2019.09.028_bib4","doi-asserted-by":"crossref","first-page":"8392","DOI":"10.1093\/nar\/gks565","article-title":"Human Cockayne syndrome B protein reciprocally communicates with mitochondrial proteins and promotes transcriptional elongation","volume":"40","author":"Berquist","year":"2012","journal-title":"Nucleic Acids Res."},{"key":"10.1016\/j.celrep.2019.09.028_bib5","doi-asserted-by":"crossref","first-page":"819","DOI":"10.1016\/S1097-2765(02)00678-0","article-title":"CSB is a component of RNA pol I transcription","volume":"10","author":"Bradsher","year":"2002","journal-title":"Mol. Cell"},{"key":"10.1016\/j.celrep.2019.09.028_bib6","doi-asserted-by":"crossref","first-page":"656","DOI":"10.1016\/j.dnarep.2013.04.018","article-title":"Blinded by the UV light: how the focus on transcription-coupled NER has distracted from understanding the mechanisms of Cockayne syndrome neurologic disease","volume":"12","author":"Brooks","year":"2013","journal-title":"DNA Repair (Amst.)"},{"key":"10.1016\/j.celrep.2019.09.028_bib7","doi-asserted-by":"crossref","first-page":"834","DOI":"10.1016\/j.dnarep.2008.01.017","article-title":"Do all of the neurologic diseases in patients with DNA repair gene mutations result from the accumulation of DNA damage?","volume":"7","author":"Brooks","year":"2008","journal-title":"DNA Repair (Amst.)"},{"key":"10.1016\/j.celrep.2019.09.028_bib8","doi-asserted-by":"crossref","first-page":"329","DOI":"10.1136\/jmedgenet-2017-104877","article-title":"Functional and clinical relevance of novel mutations in a large cohort of patients with Cockayne syndrome","volume":"55","author":"Calmels","year":"2018","journal-title":"J.\u00a0Med. Genet."},{"key":"10.1016\/j.celrep.2019.09.028_bib9","doi-asserted-by":"crossref","first-page":"E2910","DOI":"10.1073\/pnas.1422264112","article-title":"Reversal of mitochondrial defects with CSB-dependent serine protease inhibitors in patient cells of the progeroid Cockayne syndrome","volume":"112","author":"Chatre","year":"2015","journal-title":"Proc. Natl. Acad. Sci. USA"},{"key":"10.1016\/j.celrep.2019.09.028_bib10","doi-asserted-by":"crossref","first-page":"10698","DOI":"10.1523\/JNEUROSCI.5772-12.2013","article-title":"A novel activator of CBP\/p300 acetyltransferases promotes neurogenesis and extends memory duration in adult mice","volume":"33","author":"Chatterjee","year":"2013","journal-title":"J.\u00a0Neurosci."},{"key":"10.1016\/j.celrep.2019.09.028_bib11","doi-asserted-by":"crossref","first-page":"35","DOI":"10.1385\/MN:30:1:035","article-title":"The role of BETA2\/NeuroD1 in the development of the nervous system","volume":"30","author":"Cho","year":"2004","journal-title":"Mol. Neurobiol."},{"key":"10.1016\/j.celrep.2019.09.028_bib12","doi-asserted-by":"crossref","first-page":"195","DOI":"10.1038\/35018098","article-title":"A one-hit model of cell death in inherited neuronal degenerations","volume":"406","author":"Clarke","year":"2000","journal-title":"Nature"},{"key":"10.1016\/j.celrep.2019.09.028_bib13","doi-asserted-by":"crossref","first-page":"343","DOI":"10.1038\/nrm3350","article-title":"TFIIH: when transcription met DNA repair","volume":"13","author":"Compe","year":"2012","journal-title":"Nat. Rev. Mol. Cell Biol."},{"key":"10.1016\/j.celrep.2019.09.028_bib14","doi-asserted-by":"crossref","first-page":"13","DOI":"10.1016\/S1568-7864(02)00188-X","article-title":"Cell type-specific hypersensitivity to oxidative damage in CSB and XPA mice","volume":"2","author":"de Waard","year":"2003","journal-title":"DNA Repair (Amst.)"},{"key":"10.1016\/j.celrep.2019.09.028_bib15","doi-asserted-by":"crossref","first-page":"1433","DOI":"10.1128\/MCB.01037-06","article-title":"Retinal degeneration and ionizing radiation hypersensitivity in a mouse model for Cockayne syndrome","volume":"27","author":"Gorgels","year":"2007","journal-title":"Mol. Cell. Biol."},{"key":"10.1016\/j.celrep.2019.09.028_bib16","doi-asserted-by":"crossref","first-page":"26","DOI":"10.1016\/S0959-437X(96)90006-4","article-title":"TFIIH: a key component in multiple DNA transactions","volume":"6","author":"Hoeijmakers","year":"1996","journal-title":"Curr. Opin. Genet. Dev."},{"key":"10.1016\/j.celrep.2019.09.028_bib17","doi-asserted-by":"crossref","first-page":"e1002405","DOI":"10.1371\/journal.pgen.1002405","article-title":"Age-related neuronal degeneration: complementary roles of nucleotide excision repair and transcription-coupled repair in preventing neuropathology","volume":"7","author":"Jaarsma","year":"2011","journal-title":"PLoS Genet."},{"key":"10.1016\/j.celrep.2019.09.028_bib18","doi-asserted-by":"crossref","first-page":"270","DOI":"10.1016\/j.mad.2013.03.005","article-title":"Mitochondrial CSA and CSB: protein interactions and protection from ageing associated DNA mutations","volume":"134","author":"Kamenisch","year":"2013","journal-title":"Mech. Ageing Dev."},{"key":"10.1016\/j.celrep.2019.09.028_bib19","doi-asserted-by":"crossref","first-page":"357","DOI":"10.1038\/nmeth.3317","article-title":"HISAT: a fast spliced aligner with low memory requirements","volume":"12","author":"Kim","year":"2015","journal-title":"Nat. Methods"},{"key":"10.1016\/j.celrep.2019.09.028_bib20","doi-asserted-by":"crossref","first-page":"1623","DOI":"10.3174\/ajnr.A2135","article-title":"Neuroimaging in Cockayne syndrome","volume":"31","author":"Koob","year":"2010","journal-title":"AJNR Am. J. Neuroradiol."},{"key":"10.1016\/j.celrep.2019.09.028_bib21","doi-asserted-by":"crossref","first-page":"1389","DOI":"10.1073\/pnas.0610619104","article-title":"Increased apoptosis, p53 up-regulation, and cerebellar neuronal degeneration in repair-deficient Cockayne syndrome mice","volume":"104","author":"Laposa","year":"2007","journal-title":"Proc. Natl. Acad. Sci. USA"},{"key":"10.1016\/j.celrep.2019.09.028_bib22","doi-asserted-by":"crossref","first-page":"161","DOI":"10.1016\/j.mad.2013.02.006","article-title":"Cockayne syndrome: the expanding clinical and mutational spectrum","volume":"134","author":"Laugel","year":"2013","journal-title":"Mech. Ageing Dev."},{"key":"10.1016\/j.celrep.2019.09.028_bib23","doi-asserted-by":"crossref","first-page":"113","DOI":"10.1002\/humu.21154","article-title":"Mutation update for the CSB\/ERCC6 and CSA\/ERCC8 genes involved in Cockayne syndrome","volume":"31","author":"Laugel","year":"2010","journal-title":"Hum. Mutat."},{"key":"10.1016\/j.celrep.2019.09.028_bib24","doi-asserted-by":"crossref","first-page":"859","DOI":"10.1002\/ana.23701","article-title":"Mutations in KCND3 cause spinocerebellar ataxia type 22","volume":"72","author":"Lee","year":"2012","journal-title":"Ann. Neurol."},{"key":"10.1016\/j.celrep.2019.09.028_bib25","doi-asserted-by":"crossref","first-page":"1754","DOI":"10.1093\/bioinformatics\/btp324","article-title":"Fast and accurate short read alignment with Burrows-Wheeler transform","volume":"25","author":"Li","year":"2009","journal-title":"Bioinformatics"},{"key":"10.1016\/j.celrep.2019.09.028_bib26","doi-asserted-by":"crossref","first-page":"2078","DOI":"10.1093\/bioinformatics\/btp352","article-title":"The Sequence Alignment\/Map format and SAMtools","volume":"25","author":"Li","year":"2009","journal-title":"Bioinformatics"},{"key":"10.1016\/j.celrep.2019.09.028_bib27","doi-asserted-by":"crossref","first-page":"e31","DOI":"10.1093\/nar\/gkp023","article-title":"A rapid non-radioactive technique for measurement of repair synthesis in primary human fibroblasts by incorporation of ethynyl deoxyuridine (EdU)","volume":"37","author":"Limsirichaikul","year":"2009","journal-title":"Nucleic Acids Res."},{"key":"10.1016\/j.celrep.2019.09.028_bib28","doi-asserted-by":"crossref","first-page":"475","DOI":"10.1038\/428475a","article-title":"Genome sequencing: three\u2019s company","volume":"428","author":"Lindblad-Toh","year":"2004","journal-title":"Nature"},{"key":"10.1016\/j.celrep.2019.09.028_bib29","doi-asserted-by":"crossref","first-page":"506","DOI":"10.1016\/j.dnarep.2010.01.015","article-title":"A semi-automated non-radioactive system for measuring recovery of RNA synthesis and unscheduled DNA synthesis using ethynyluracil derivatives","volume":"9","author":"Nakazawa","year":"2010","journal-title":"DNA Repair (Amst.)"},{"key":"10.1016\/j.celrep.2019.09.028_bib30","doi-asserted-by":"crossref","first-page":"6209","DOI":"10.1073\/pnas.0902113106","article-title":"A UV-sensitive syndrome patient with a specific CSA mutation reveals separable roles for CSA in response to UV and oxidative DNA damage","volume":"106","author":"Nardo","year":"2009","journal-title":"Proc. Natl. Acad. Sci. USA"},{"key":"10.1016\/j.celrep.2019.09.028_bib31","doi-asserted-by":"crossref","first-page":"9613","DOI":"10.1073\/pnas.0510909103","article-title":"Cockayne syndrome group B protein (CSB) plays a general role in chromatin maintenance and remodeling","volume":"103","author":"Newman","year":"2006","journal-title":"Proc. Natl. Acad. Sci. USA"},{"key":"10.1016\/j.celrep.2019.09.028_bib32","doi-asserted-by":"crossref","first-page":"2281","DOI":"10.1038\/nprot.2013.143","article-title":"Genome engineering using the CRISPR-Cas9 system","volume":"8","author":"Ran","year":"2013","journal-title":"Nat. Protoc."},{"key":"10.1016\/j.celrep.2019.09.028_bib33","doi-asserted-by":"crossref","first-page":"855","DOI":"10.1084\/jem.20111721","article-title":"Cockayne syndrome group B protein prevents the accumulation of damaged mitochondria by promoting mitochondrial autophagy","volume":"209","author":"Scheibye-Knudsen","year":"2012","journal-title":"J.\u00a0Exp. Med."},{"key":"10.1016\/j.celrep.2019.09.028_bib34","doi-asserted-by":"crossref","first-page":"671","DOI":"10.1038\/nmeth.2089","article-title":"NIH Image to ImageJ: 25 years of image analysis","volume":"9","author":"Schneider","year":"2012","journal-title":"Nat. Methods"},{"key":"10.1016\/j.celrep.2019.09.028_bib35","doi-asserted-by":"crossref","first-page":"74","DOI":"10.1016\/j.cell.2009.12.011","article-title":"Human TUBB3 mutations perturb microtubule dynamics, kinesin interactions, and axon guidance","volume":"140","author":"Tischfield","year":"2010","journal-title":"Cell"},{"key":"10.1016\/j.celrep.2019.09.028_bib36","doi-asserted-by":"crossref","first-page":"45772","DOI":"10.1074\/jbc.M107888200","article-title":"The Cockayne syndrome group B gene product is involved in general genome base excision repair of 8-hydroxyguanine in DNA","volume":"276","author":"Tuo","year":"2001","journal-title":"J.\u00a0Biol. Chem."},{"key":"10.1016\/j.celrep.2019.09.028_bib37","doi-asserted-by":"crossref","first-page":"4155","DOI":"10.1093\/emboj\/16.14.4155","article-title":"Cockayne syndrome: defective repair of transcription?","volume":"16","author":"van Gool","year":"1997","journal-title":"EMBO J."},{"key":"10.1016\/j.celrep.2019.09.028_bib38","doi-asserted-by":"crossref","first-page":"425","DOI":"10.1016\/S0092-8674(00)80223-8","article-title":"Defective transcription-coupled repair in Cockayne syndrome B mice is associated with skin cancer predisposition","volume":"89","author":"van der Horst","year":"1997","journal-title":"Cell"},{"key":"10.1016\/j.celrep.2019.09.028_bib39","doi-asserted-by":"crossref","first-page":"14454","DOI":"10.1073\/pnas.1412569111","article-title":"Dysregulation of gene expression as a cause of Cockayne syndrome neurological disease","volume":"111","author":"Wang","year":"2014","journal-title":"Proc. Natl. Acad. Sci. USA"},{"key":"10.1016\/j.celrep.2019.09.028_bib40","doi-asserted-by":"crossref","first-page":"2554","DOI":"10.1016\/j.celrep.2016.02.051","article-title":"Pharmacological Bypass of Cockayne Syndrome B Function in Neuronal Differentiation","volume":"14","author":"Wang","year":"2016","journal-title":"Cell Rep."},{"key":"10.1016\/j.celrep.2019.09.028_bib41","doi-asserted-by":"crossref","first-page":"2090","DOI":"10.1523\/JNEUROSCI.19-06-02090.1999","article-title":"Specification of distinct dopaminergic neural pathways: roles of the Eph family receptor EphB1 and ligand ephrin-B2","volume":"19","author":"Yue","year":"1999","journal-title":"J.\u00a0Neurosci."},{"key":"10.1016\/j.celrep.2019.09.028_bib42","doi-asserted-by":"crossref","first-page":"885","DOI":"10.1093\/brain\/awz032","article-title":"Ossified blood vessels in primary familial brain calcification elicit a neurotoxic astrocyte response","volume":"142","author":"Zarb","year":"2019","journal-title":"Brain"}],"container-title":["Cell Reports"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/api.elsevier.com\/content\/article\/PII:S2211124719312057?httpAccept=text\/xml","content-type":"text\/xml","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/api.elsevier.com\/content\/article\/PII:S2211124719312057?httpAccept=text\/plain","content-type":"text\/plain","content-version":"vor","intended-application":"text-mining"}],"deposited":{"date-parts":[[2025,10,31]],"date-time":"2025-10-31T10:44:17Z","timestamp":1761907457000},"score":1,"resource":{"primary":{"URL":"https:\/\/linkinghub.elsevier.com\/retrieve\/pii\/S2211124719312057"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2019,10]]},"references-count":42,"journal-issue":{"issue":"4","published-print":{"date-parts":[[2019,10]]}},"alternative-id":["S2211124719312057"],"URL":"https:\/\/doi.org\/10.1016\/j.celrep.2019.09.028","relation":{},"ISSN":["2211-1247"],"issn-type":[{"value":"2211-1247","type":"print"}],"subject":[],"published":{"date-parts":[[2019,10]]},"assertion":[{"value":"Elsevier","name":"publisher","label":"This article is maintained by"},{"value":"Rat Model of Cockayne Syndrome Neurological Disease","name":"articletitle","label":"Article Title"},{"value":"Cell Reports","name":"journaltitle","label":"Journal Title"},{"value":"https:\/\/doi.org\/10.1016\/j.celrep.2019.09.028","name":"articlelink","label":"CrossRef DOI link to publisher maintained version"},{"value":"article","name":"content_type","label":"Content Type"},{"value":"\u00a9 2019 The Author(s).","name":"copyright","label":"Copyright"}]}}