{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,29]],"date-time":"2026-09-29T19:22:07Z","timestamp":1790709727645,"version":"4.1.0"},"reference-count":85,"publisher":"Public Library of Science (PLoS)","issue":"12","license":[{"start":{"date-parts":[[2011,12,8]],"date-time":"2011-12-08T00:00:00Z","timestamp":1323302400000},"content-version":"unspecified","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":["www.plosgenetics.org"],"crossmark-restriction":false},"short-container-title":["PLoS Genet"],"DOI":"10.1371\/journal.pgen.1002405","type":"journal-article","created":{"date-parts":[[2011,12,8]],"date-time":"2011-12-08T21:51:50Z","timestamp":1323381110000},"page":"e1002405","update-policy":"https:\/\/doi.org\/10.1371\/journal.pgen.corrections_policy","source":"Crossref","is-referenced-by-count":72,"title":["Age-Related Neuronal Degeneration: Complementary Roles of Nucleotide Excision Repair and Transcription-Coupled Repair in Preventing Neuropathology"],"prefix":"10.1371","volume":"7","author":[{"given":"Dick","family":"Jaarsma","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ingrid","family":"van der Pluijm","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Monique C.","family":"de Waard","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Elize D.","family":"Haasdijk","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Renata","family":"Brandt","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Marcel","family":"Vermeij","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Yvonne","family":"Rijksen","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Alex","family":"Maas","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Harry","family":"van Steeg","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jan H. J.","family":"Hoeijmakers","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Gijsbertus T. J.","family":"van der Horst","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"340","published-online":{"date-parts":[[2011,12,8]]},"reference":[{"key":"ref1","doi-asserted-by":"crossref","first-page":"1475","DOI":"10.1056\/NEJMra0804615","article-title":"DNA damage, aging, and cancer.","volume":"361","author":"JH Hoeijmakers","year":"2009","journal-title":"N Engl J Med"},{"key":"ref2","doi-asserted-by":"crossref","first-page":"1213","DOI":"10.1016\/j.neuroscience.2006.07.006","article-title":"DNA repair in differentiated cells: some new answers to old questions.","volume":"145","author":"T Nouspikel","year":"2007","journal-title":"Neuroscience"},{"key":"ref3","doi-asserted-by":"crossref","first-page":"991","DOI":"10.1016\/j.cell.2007.08.043","article-title":"Defective DNA repair and neurodegenerative disease.","volume":"130","author":"U Rass","year":"2007","journal-title":"Cell"},{"key":"ref4","doi-asserted-by":"crossref","first-page":"986","DOI":"10.1016\/j.dnarep.2006.05.005","article-title":"DNA repair: from molecular mechanism to human disease.","volume":"5","author":"EC Friedberg","year":"2006","journal-title":"DNA Repair (Amst)"},{"key":"ref5","doi-asserted-by":"crossref","first-page":"100","DOI":"10.1038\/nrn2559","article-title":"DNA repair deficiency and neurological disease.","volume":"10","author":"PJ McKinnon","year":"2009","journal-title":"Nat Rev Neurosci"},{"key":"ref6","doi-asserted-by":"crossref","first-page":"366","DOI":"10.1038\/35077232","article-title":"Genome maintenance mechanisms for preventing cancer.","volume":"411","author":"JH Hoeijmakers","year":"2001","journal-title":"Nature"},{"key":"ref7","doi-asserted-by":"crossref","first-page":"958","DOI":"10.1038\/nrm2549","article-title":"Transcription-coupled DNA repair: two decades of progress and surprises.","volume":"9","author":"PC Hanawalt","year":"2008","journal-title":"Nat Rev Mol Cell Biol"},{"key":"ref8","doi-asserted-by":"crossref","first-page":"756","DOI":"10.1038\/nrg2663","article-title":"Disorders of nucleotide excision repair: the genetic and molecular basis of heterogeneity.","volume":"10","author":"JE Cleaver","year":"2009","journal-title":"Nat Rev Genet"},{"key":"ref9","doi-asserted-by":"crossref","first-page":"441","DOI":"10.1016\/j.mad.2008.04.009","article-title":"The role of Cockayne Syndrome group B (CSB) protein in base excision repair and aging.","volume":"129","author":"T Stevnsner","year":"2008","journal-title":"Mech Ageing Dev"},{"key":"ref10","doi-asserted-by":"crossref","first-page":"9270","DOI":"10.1074\/jbc.M807006200","article-title":"Cockayne Syndrome Group B Protein Stimulates Repair of Formamidopyrimidines by NEIL1 DNA Glycosylase.","volume":"284","author":"M Muftuoglu","year":"2009","journal-title":"J Biol Chem"},{"key":"ref11","doi-asserted-by":"crossref","first-page":"1537","DOI":"10.1038\/jid.2009.409","article-title":"A prevalent mutation with founder effect in xeroderma pigmentosum group C from north Africa.","volume":"130","author":"N Soufir","year":"2010","journal-title":"J Invest Dermatol"},{"key":"ref12","doi-asserted-by":"crossref","first-page":"114","DOI":"10.1016\/j.dnarep.2008.09.007","article-title":"XPC initiation codon mutation in xeroderma pigmentosum patients with and without neurological symptoms.","volume":"8","author":"SG Khan","year":"2009","journal-title":"DNA Repair (Amst)"},{"key":"ref13","doi-asserted-by":"crossref","first-page":"471","DOI":"10.1016\/j.molcel.2006.06.029","article-title":"Cockayne syndrome A and B proteins differentially regulate recruitment of chromatin remodeling and repair factors to stalled RNA polymerase II in vivo.","volume":"23","author":"M Fousteri","year":"2006","journal-title":"Mol Cell"},{"key":"ref14","doi-asserted-by":"crossref","first-page":"1388","DOI":"10.1016\/j.neuroscience.2006.12.020","article-title":"Xeroderma pigmentosum, trichothiodystrophy and Cockayne syndrome: a complex genotype-phenotype relationship.","volume":"145","author":"KH Kraemer","year":"2007","journal-title":"Neuroscience"},{"key":"ref15","doi-asserted-by":"crossref","first-page":"68","DOI":"10.1002\/ajmg.1320420115","article-title":"Cockayne syndrome: review of 140 cases.","volume":"42","author":"MA Nance","year":"1992","journal-title":"Am J Med Genet"},{"key":"ref16","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":"V Laugel","year":"2010","journal-title":"Hum Mutat"},{"key":"ref17","doi-asserted-by":"crossref","first-page":"70","DOI":"10.1002\/ana.410200112","article-title":"Neurological manifestations in xeroderma pigmentosum.","volume":"20","author":"T Mimaki","year":"1986","journal-title":"Ann Neurol"},{"key":"ref18","doi-asserted-by":"crossref","first-page":"241","DOI":"10.1001\/archderm.1987.01660260111026","article-title":"Xeroderma pigmentosum. Cutaneous, ocular, and neurologic abnormalities in 830 published cases.","volume":"123","author":"KH Kraemer","year":"1987","journal-title":"Arch Dermatol"},{"key":"ref19","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":"A Anttinen","year":"2008","journal-title":"Brain"},{"key":"ref20","doi-asserted-by":"crossref","first-page":"7941","DOI":"10.1128\/MCB.24.18.7941-7948.2004","article-title":"Different effects of CSA and CSB deficiency on sensitivity to oxidative DNA damage.","volume":"24","author":"H de Waard","year":"2004","journal-title":"Mol Cell Biol"},{"key":"ref21","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.","author":"T Nardo","year":"2009","journal-title":"Proc Natl Acad Sci U S A"},{"key":"ref22","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":"PJ Brooks","year":"2008","journal-title":"DNA Repair (Amst)"},{"key":"ref23","doi-asserted-by":"crossref","first-page":"235","DOI":"10.1016\/j.molcel.2009.10.027","article-title":"UV-induced association of the CSB remodeling protein with chromatin requires ATP-dependent relief of N-terminal autorepression.","volume":"37","author":"RJ Lake","year":"2010","journal-title":"Mol Cell"},{"key":"ref24","doi-asserted-by":"crossref","first-page":"339","DOI":"10.1016\/j.dnarep.2007.10.007","article-title":"Hot topics in DNA repair: the molecular basis for different disease states caused by mutations in TFIIH and XPG.","volume":"7","author":"OD Scharer","year":"2008","journal-title":"DNA Repair (Amst)"},{"key":"ref25","doi-asserted-by":"crossref","first-page":"231","DOI":"10.1016\/j.molcel.2007.03.013","article-title":"XPG stabilizes TFIIH, allowing transactivation of nuclear receptors: implications for Cockayne syndrome in XP-G\/CS patients.","volume":"26","author":"S Ito","year":"2007","journal-title":"Mol Cell"},{"key":"ref26","doi-asserted-by":"crossref","first-page":"351","DOI":"10.1016\/j.dnarep.2004.10.009","article-title":"Severe growth retardation and short life span of double-mutant mice lacking Xpa and exon 15 of Xpg.","volume":"4","author":"N Shiomi","year":"2005","journal-title":"DNA Repair (Amst)"},{"key":"ref27","doi-asserted-by":"crossref","first-page":"1912","DOI":"10.1016\/j.dnarep.2008.07.008","article-title":"XPD structure reveals its secrets.","volume":"7","author":"AR Lehmann","year":"2008","journal-title":"DNA Repair (Amst)"},{"key":"ref28","doi-asserted-by":"crossref","first-page":"e322","DOI":"10.1371\/journal.pbio.0040322","article-title":"Rescue of progeria in trichothiodystrophy by homozygous lethal Xpd alleles.","volume":"4","author":"JO Andressoo","year":"2006","journal-title":"PLoS Biol"},{"key":"ref29","doi-asserted-by":"crossref","first-page":"1276","DOI":"10.1128\/MCB.01229-08","article-title":"An Xpb mouse model for combined xeroderma pigmentosum and cockayne syndrome reveals progeroid features upon further attenuation of DNA repair.","volume":"29","author":"JO Andressoo","year":"2009","journal-title":"Mol Cell Biol"},{"key":"ref30","doi-asserted-by":"crossref","first-page":"245","DOI":"10.1016\/j.molcel.2007.03.009","article-title":"Distinct roles for the XPB\/p52 and XPD\/p44 subcomplexes of TFIIH in damaged DNA opening during nucleotide excision repair.","volume":"26","author":"F Coin","year":"2007","journal-title":"Mol Cell"},{"key":"ref31","doi-asserted-by":"crossref","first-page":"2","DOI":"10.1016\/j.dnarep.2009.10.005","article-title":"Trichothiodystrophy: from basic mechanisms to clinical implications.","volume":"9","author":"M Stefanini","year":"2010","journal-title":"DNA Repair (Amst)"},{"key":"ref32","doi-asserted-by":"crossref","first-page":"83","DOI":"10.1007\/978-0-387-09599-8_9","article-title":"XPG: its products and biological roles.","volume":"637","author":"OD Scharer","year":"2008","journal-title":"Adv Exp Med Biol"},{"key":"ref33","doi-asserted-by":"crossref","first-page":"1318","DOI":"10.1016\/S0140-6736(76)92652-0","article-title":"Relation of D.N.A. repair processes to pathological ageing of the nervous system in xeroderma pigmentosum.","volume":"1","author":"AD Andrews","year":"1976","journal-title":"Lancet"},{"key":"ref34","doi-asserted-by":"crossref","first-page":"225","DOI":"10.1111\/j.1399-0004.1995.tb04094.x","article-title":"Chronological difference in walking impairment among Japanese group A xeroderma pigmentosum (XP-A) patients with various combinations of mutation sites.","volume":"48","author":"T Maeda","year":"1995","journal-title":"Clin Genet"},{"key":"ref35","doi-asserted-by":"crossref","first-page":"326","DOI":"10.1016\/S0387-7604(99)00033-9","article-title":"Neurodegeneration in hereditary nucleotide repair disorders.","volume":"21","author":"M Itoh","year":"1999","journal-title":"Brain Dev"},{"key":"ref36","doi-asserted-by":"crossref","first-page":"511","DOI":"10.1111\/j.1651-2227.1996.tb14074.x","article-title":"Truncated XPA protein detected in atypical group A xeroderma pigmentosum.","volume":"85","author":"T Mimaki","year":"1996","journal-title":"Acta Paediatr"},{"key":"ref37","doi-asserted-by":"crossref","first-page":"1597","DOI":"10.1038\/sj.jid.5701210","article-title":"Adult-onset neurological degeneration in a patient with Cockayne syndrome and a null mutation in the CSB gene.","volume":"128","author":"S Hashimoto","year":"2008","journal-title":"J Invest Dermatol"},{"key":"ref38","doi-asserted-by":"crossref","first-page":"991","DOI":"10.1177\/08830738060210110101","article-title":"Cockayne syndrome in adults: review with clinical and pathologic study of a new case.","volume":"21","author":"I Rapin","year":"2006","journal-title":"J Child Neurol"},{"key":"ref39","doi-asserted-by":"crossref","first-page":"13","DOI":"10.1016\/j.dnarep.2005.06.017","article-title":"Host cell reactivation of plasmids containing oxidative DNA lesions is defective in Cockayne syndrome but normal in UV-sensitive syndrome fibroblasts.","volume":"5","author":"G Spivak","year":"2006","journal-title":"DNA Repair (Amst)"},{"key":"ref40","doi-asserted-by":"crossref","first-page":"1180","DOI":"10.1016\/j.dnarep.2007.12.006","article-title":"Nucleotide excision repair deficient mouse models and neurological disease.","volume":"7","author":"LJ Niedernhofer","year":"2008","journal-title":"DNA Repair (Amst)"},{"key":"ref41","doi-asserted-by":"crossref","first-page":"169","DOI":"10.1038\/377169a0","article-title":"Increased susceptibility to ultraviolet-B and carcinogens of mice lacking the DNA excision repair gene XPA.","volume":"377","author":"A de Vries","year":"1995","journal-title":"Nature"},{"key":"ref42","doi-asserted-by":"crossref","first-page":"165","DOI":"10.1038\/377165a0","article-title":"High incidence of ultraviolet-B-or chemical-carcinogen-induced skin tumours in mice lacking the xeroderma pigmentosum group A gene.","volume":"377","author":"H Nakane","year":"1995","journal-title":"Nature"},{"key":"ref43","doi-asserted-by":"crossref","first-page":"1347","DOI":"10.1158\/0008-5472.CAN-07-6067","article-title":"Mouse models for xeroderma pigmentosum group A and group C show divergent cancer phenotypes.","volume":"68","author":"JP Melis","year":"2008","journal-title":"Cancer Res"},{"key":"ref44","doi-asserted-by":"crossref","first-page":"1938","DOI":"10.1016\/j.dnarep.2008.08.003","article-title":"Impaired spermatogenesis and elevated spontaneous tumorigenesis in xeroderma pigmentosum group A gene (Xpa)-deficient mice.","volume":"7","author":"H Nakane","year":"2008","journal-title":"DNA Repair (Amst)"},{"key":"ref45","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":"GT van der Horst","year":"1997","journal-title":"Cell"},{"key":"ref46","doi-asserted-by":"crossref","first-page":"143","DOI":"10.1016\/S1568-7864(01)00010-6","article-title":"UVB radiation-induced cancer predisposition in Cockayne syndrome group A (Csa) mutant mice.","volume":"1","author":"GT van der Horst","year":"2002","journal-title":"DNA Repair (Amst)"},{"key":"ref47","doi-asserted-by":"crossref","first-page":"1","DOI":"10.1016\/S0027-5107(97)00046-8","article-title":"Characterization of defective nucleotide excision repair in XPC mutant mice.","volume":"374","author":"DL Cheo","year":"1997","journal-title":"Mutat Res"},{"key":"ref48","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":"TG Gorgels","year":"2007","journal-title":"Mol Cell Biol"},{"key":"ref49","doi-asserted-by":"crossref","first-page":"121","DOI":"10.1016\/j.ccr.2006.05.027","article-title":"An Xpd mouse model for the combined xeroderma pigmentosum\/Cockayne syndrome exhibiting both cancer predisposition and segmental progeria.","volume":"10","author":"JO Andressoo","year":"2006","journal-title":"Cancer Cell"},{"key":"ref50","doi-asserted-by":"crossref","first-page":"981","DOI":"10.1016\/S1097-2765(00)80098-2","article-title":"A mouse model for the basal transcription\/DNA repair syndrome trichothiodystrophy.","volume":"1","author":"J de Boer","year":"1998","journal-title":"Mol Cell"},{"key":"ref51","first-page":"3489","article-title":"Mouse model for the DNA repair\/basal transcription disorder trichothiodystrophy reveals cancer predisposition.","volume":"59","author":"J de Boer","year":"1999","journal-title":"Cancer Res"},{"key":"ref52","doi-asserted-by":"crossref","first-page":"1314","DOI":"10.1016\/j.dnarep.2005.07.002","article-title":"Accelerated aging pathology in ad libitum fed Xpd(TTD) mice is accompanied by features suggestive of caloric restriction.","volume":"4","author":"SW Wijnhoven","year":"2005","journal-title":"DNA Repair (Amst)"},{"key":"ref53","doi-asserted-by":"crossref","first-page":"22","DOI":"10.1016\/j.mrfmmm.2005.11.008","article-title":"Increased genomic instability is not a prerequisite for shortened lifespan in DNA repair deficient mice.","volume":"596","author":"ME Dolle","year":"2006","journal-title":"Mutat Res"},{"key":"ref54","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":"H de Waard","year":"2003","journal-title":"DNA Repair (Amst)"},{"key":"ref55","doi-asserted-by":"crossref","first-page":"1659","DOI":"10.1016\/j.dnarep.2008.06.009","article-title":"Cell-type-specific consequences of nucleotide excision repair deficiencies: Embryonic stem cells versus fibroblasts.","volume":"7","author":"H de Waard","year":"2008","journal-title":"DNA Repair (Amst)"},{"key":"ref56","doi-asserted-by":"crossref","first-page":"77","DOI":"10.1016\/j.mrfmmm.2005.12.018","article-title":"Tissue specific mutagenic and carcinogenic responses in NER defective mouse models.","volume":"614","author":"SW Wijnhoven","year":"2007","journal-title":"Mutat Res"},{"key":"ref57","doi-asserted-by":"crossref","first-page":"1027","DOI":"10.1038\/sj.cdd.4401910","article-title":"The P53 pathway: what questions remain to be explored?","volume":"13","author":"AJ Levine","year":"2006","journal-title":"Cell Death Differ"},{"key":"ref58","doi-asserted-by":"crossref","first-page":"1607","DOI":"10.1002\/glia.20713","article-title":"Galectin-3\/MAC-2, Ras and PI3K activate complement receptor-3 and scavenger receptor-AI\/II mediated myelin phagocytosis in microglia.","volume":"56","author":"S Rotshenker","year":"2008","journal-title":"Glia"},{"key":"ref59","doi-asserted-by":"crossref","first-page":"563","DOI":"10.1016\/S0301-0082(98)00069-0","article-title":"Reactive microgliosis.","volume":"57","author":"WJ Streit","year":"1999","journal-title":"Prog Neurobiol"},{"key":"ref60","first-page":"487","article-title":"Alpha B-crystallin and 27-kd heat shock protein are regulated by stress conditions in the central nervous system and accumulate in Rosenthal fibers.","volume":"143","author":"T Iwaki","year":"1993","journal-title":"Am J Pathol"},{"key":"ref61","doi-asserted-by":"crossref","first-page":"261","DOI":"10.1016\/S0955-0674(99)80035-0","article-title":"Deciphering the pathways of life and death.","volume":"11","author":"H Li","year":"1999","journal-title":"Curr Opin Cell Biol"},{"key":"ref62","doi-asserted-by":"crossref","first-page":"13379","DOI":"10.1073\/pnas.231329598","article-title":"Early postnatal ataxia and abnormal cerebellar development in mice lacking Xeroderma pigmentosum Group A and Cockayne syndrome Group B DNA repair genes.","volume":"98","author":"M Murai","year":"2001","journal-title":"Proc Natl Acad Sci U S A"},{"key":"ref63","doi-asserted-by":"crossref","first-page":"e2","DOI":"10.1371\/journal.pbio.0050002","article-title":"Impaired genome maintenance suppresses the growth hormone\u2013insulin-like growth factor 1 axis in mice with Cockayne syndrome.","volume":"5","author":"I van der Pluijm","year":"2007","journal-title":"PLoS Biol"},{"key":"ref64","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":"RR Laposa","year":"2007","journal-title":"Proc Natl Acad Sci U S A"},{"key":"ref65","doi-asserted-by":"crossref","first-page":"318","DOI":"10.1006\/bbrc.1997.7111","article-title":"A transgenic mouse line that retains Cre recombinase activity in mature oocytes irrespective of the cre transgene transmission.","volume":"237","author":"K Sakai","year":"1997","journal-title":"Biochem Biophys Res Commun"},{"key":"ref66","doi-asserted-by":"crossref","first-page":"133","DOI":"10.1002\/(SICI)1526-968X(200002)26:2<133::AID-GENE10>3.0.CO;2-V","article-title":"CaMKIIalpha-Cre transgene expression and recombination patterns in the mouse brain.","volume":"26","author":"I Dragatsis","year":"2000","journal-title":"Genesis"},{"key":"ref67","doi-asserted-by":"crossref","first-page":"14163","DOI":"10.1073\/pnas.0705738104","article-title":"Cytochrome c oxidase deficiency in neurons decreases both oxidative stress and amyloid formation in a mouse model of Alzheimer&apos;s disease.","volume":"104","author":"H Fukui","year":"2007","journal-title":"Proc Natl Acad Sci U S A"},{"key":"ref68","doi-asserted-by":"crossref","first-page":"151","DOI":"10.1385\/JMN:18:1-2:151","article-title":"Evaluation of an anxiety-related phenotype in galanin overexpressing transgenic mice.","volume":"18","author":"A Holmes","year":"2002","journal-title":"J Mol Neurosci"},{"key":"ref69","doi-asserted-by":"crossref","first-page":"7488","DOI":"10.1038\/sj.onc.1205896","article-title":"ATF3 induction following DNA damage is regulated by distinct signaling pathways and over-expression of ATF3 protein suppresses cells growth.","volume":"21","author":"F Fan","year":"2002","journal-title":"Oncogene"},{"key":"ref70","doi-asserted-by":"crossref","first-page":"728","DOI":"10.1038\/cdd.2009.2","article-title":"ATF3 and p15PAF are novel gatekeepers of genomic integrity upon UV stress.","volume":"16","author":"L Turchi","year":"2009","journal-title":"Cell Death Differ"},{"key":"ref71","doi-asserted-by":"crossref","first-page":"93","DOI":"10.1002\/1526-968X(200011\/12)28:3\/4<93::AID-GENE10>3.0.CO;2-W","article-title":"Cre recombinase expression in cerebellar Purkinje cells.","volume":"28","author":"JJ Barski","year":"2000","journal-title":"Genesis"},{"key":"ref72","doi-asserted-by":"crossref","first-page":"340","DOI":"10.1002\/ana.410060407","article-title":"Cockayne syndrome: unusual neuropathological findings and review of the literature.","volume":"6","author":"D Soffer","year":"1979","journal-title":"Ann Neurol"},{"key":"ref73","doi-asserted-by":"crossref","first-page":"507","DOI":"10.1097\/00005072-198509000-00006","article-title":"Cockayne syndrome: clinicopathologic and tissue culture studies of affected siblings.","volume":"44","author":"RW Leech","year":"1985","journal-title":"J Neuropathol Exp Neurol"},{"key":"ref74","doi-asserted-by":"crossref","first-page":"829","DOI":"10.1093\/jnen\/60.9.829","article-title":"Caspases, apoptosis, and Alzheimer disease: causation, correlation, and confusion.","volume":"60","author":"KA Roth","year":"2001","journal-title":"J Neuropathol Exp Neurol"},{"key":"ref75","doi-asserted-by":"crossref","first-page":"153","DOI":"10.1172\/JCI200317610","article-title":"Programmed cell death in amyotrophic lateral sclerosis.","volume":"111","author":"C Guegan","year":"2003","journal-title":"J Clin Invest"},{"key":"ref76","doi-asserted-by":"crossref","first-page":"133","DOI":"10.1016\/S0304-3940(98)00109-8","article-title":"Cerebellar neurodegeneration in human hereditary DNA repair disorders.","volume":"243","author":"T Kohji","year":"1998","journal-title":"Neurosci Lett"},{"key":"ref77","doi-asserted-by":"crossref","first-page":"246","DOI":"10.1046\/j.1471-4159.1997.69010246.x","article-title":"Loss of the xeroderma pigmentosum group A gene (XPA) enhances apoptosis of cultured cerebellar neurons induced by UV but not by low-K+ medium.","volume":"69","author":"Y Enokido","year":"1997","journal-title":"J Neurochem"},{"key":"ref78","doi-asserted-by":"crossref","first-page":"9451","DOI":"10.1523\/JNEUROSCI.0523-07.2007","article-title":"Repair capacity for platinum-DNA adducts determines the severity of cisplatin-induced peripheral neuropathy.","volume":"27","author":"A Dzagnidze","year":"2007","journal-title":"J Neurosci"},{"key":"ref79","doi-asserted-by":"crossref","first-page":"1168","DOI":"10.1016\/j.dnarep.2008.03.016","article-title":"The 8,5\u2032-cyclopurine-2\u2032-deoxynucleosides: candidate neurodegenerative DNA lesions in xeroderma pigmentosum, and unique probes of transcription and nucleotide excision repair.","volume":"7","author":"PJ Brooks","year":"2008","journal-title":"DNA Repair (Amst)"},{"key":"ref80","doi-asserted-by":"crossref","first-page":"1155","DOI":"10.1016\/j.dnarep.2008.03.015","article-title":"Nucleotide excision repair and neurological diseases.","volume":"7","author":"T Nouspikel","year":"2008","journal-title":"DNA Repair (Amst)"},{"key":"ref81","doi-asserted-by":"crossref","first-page":"461","DOI":"10.1007\/s00401-010-0715-9","article-title":"Age-related motor neuron degeneration in DNA repair-deficient Ercc1 mice.","volume":"120","author":"MC de Waard","year":"2010","journal-title":"Acta Neuropathol"},{"key":"ref82","doi-asserted-by":"crossref","first-page":"343","DOI":"10.1093\/mutage\/gem024","article-title":"Transcription through 8-oxoguanine in DNA repair-proficient and Csb(\u2212)\/Ogg1(\u2212) DNA repair-deficient mouse embryonic fibroblasts is dependent upon promoter strength and sequence context.","volume":"22","author":"M Pastoriza-Gallego","year":"2007","journal-title":"Mutagenesis"},{"key":"ref83","doi-asserted-by":"crossref","first-page":"1233","DOI":"10.1128\/MCB.22.4.1233-1245.2002","article-title":"Developmental defects and male sterility in mice lacking the ubiquitin-like DNA repair gene mHR23B.","volume":"22","author":"JM Ng","year":"2002","journal-title":"Mol Cell Biol"},{"key":"ref84","doi-asserted-by":"crossref","first-page":"7859","DOI":"10.1523\/JNEUROSCI.16-24-07859.1996","article-title":"Motor discoordination results from combined gene disruption of the NMDA receptor NR2A and NR2C subunits, but not from single disruption of the NR2A or NR2C subunit.","volume":"16","author":"H Kadotani","year":"1996","journal-title":"J Neurosci"},{"key":"ref85","article-title":"The mouse brain in stereotaxic coordinates","author":"G Paxinos","year":"2001"}],"container-title":["PLoS Genetics"],"original-title":[],"language":"en","link":[{"URL":"http:\/\/dx.plos.org\/10.1371\/journal.pgen.1002405","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2019,6,20]],"date-time":"2019-06-20T16:43:02Z","timestamp":1561048982000},"score":1,"resource":{"primary":{"URL":"https:\/\/dx.plos.org\/10.1371\/journal.pgen.1002405"}},"subtitle":[],"editor":[{"given":"James M.","family":"Ford","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"editor"}]}],"short-title":[],"issued":{"date-parts":[[2011,12,8]]},"references-count":85,"journal-issue":{"issue":"12","published-online":{"date-parts":[[2011,12,8]]}},"URL":"https:\/\/doi.org\/10.1371\/journal.pgen.1002405","relation":{},"ISSN":["1553-7404"],"issn-type":[{"value":"1553-7404","type":"electronic"}],"subject":[],"published":{"date-parts":[[2011,12,8]]}}}