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Biol"},{"key":"ref81","doi-asserted-by":"crossref","first-page":"217","DOI":"10.1038\/ng1193-217","article-title":"Mice with DNA repair gene (ERCC-1) deficiency have elevated levels of p53, liver nuclear abnormalities and die before weaning","volume":"5","author":"J McWhir","year":"1993","journal-title":"Nat Genet"},{"key":"ref82","doi-asserted-by":"crossref","first-page":"609","DOI":"10.1002\/hep.24713","article-title":"A mouse model of accelerated liver aging caused by a defect in DNA repair","volume":"55","author":"SQ Gregg","year":"2012","journal-title":"Hepatology"},{"key":"ref83","doi-asserted-by":"crossref","first-page":"4541","DOI":"10.1093\/nar\/29.22.4541","article-title":"Correction of liver dysfunction in DNA repair-deficient mice with an ERCC1 transgene","volume":"29","author":"J Selfridge","year":"2001","journal-title":"Nucleic Acids Res"},{"key":"ref84","doi-asserted-by":"crossref","first-page":"604","DOI":"10.1038\/ncb1866","article-title":"Persistent transcription-blocking DNA lesions trigger somatic growth attenuation associated with longevity","volume":"11","author":"GA Garinis","year":"2009","journal-title":"Nat Cell Biol"},{"key":"ref85","doi-asserted-by":"crossref","first-page":"e192","DOI":"10.1371\/journal.pgen.0020192","article-title":"Adaptive stress response in segmental progeria resembles long-lived dwarfism and calorie restriction in mice","volume":"2","author":"M van de Ven","year":"2006","journal-title":"PLoS Genet"},{"key":"ref86","doi-asserted-by":"crossref","first-page":"89","DOI":"10.1146\/annurev.pharmtox.46.120604.141046","article-title":"Cell survival responses to environmental stresses via the Keap1-Nrf2-ARE pathway","volume":"47","author":"TW Kensler","year":"2007","journal-title":"Annu Rev Pharmacol Toxicol"},{"key":"ref87","doi-asserted-by":"crossref","first-page":"345","DOI":"10.1006\/scbi.1998.0097","article-title":"Regulation of p53 downstream genes","volume":"8","author":"WS el-Deiry","year":"1998","journal-title":"Semin Cancer Biol"},{"key":"ref88","doi-asserted-by":"crossref","first-page":"958","DOI":"10.1002\/hep.1840380422","article-title":"Characterization of premature liver polyploidy in DNA repair (Ercc1)-deficient mice","volume":"38","author":"MD Chipchase","year":"2003","journal-title":"Hepatology"},{"key":"ref89","doi-asserted-by":"crossref","first-page":"12543","DOI":"10.1523\/JNEUROSCI.1589-11.2011","article-title":"Accelerated age-related cognitive decline and neurodegeneration, caused by deficient DNA repair","volume":"31","author":"NZ Borgesius","year":"2011","journal-title":"J Neurosci"},{"key":"ref90","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":"ref91","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":"D Jaarsma","year":"2011","journal-title":"PLoS Genet"},{"key":"ref92","doi-asserted-by":"crossref","first-page":"1350","DOI":"10.1074\/mcp.M112.024950","article-title":"Spatio-temporal analysis of molecular determinants of neuronal degeneration in the aging mouse cerebellum","volume":"12","author":"EL de Graaf","year":"2013","journal-title":"Mol Cell Proteomics"},{"key":"ref93","doi-asserted-by":"crossref","first-page":"90","DOI":"10.1111\/j.1365-2990.2012.01308.x","article-title":"Review: Axon pathology in age-related neurodegenerative disorders","volume":"39","author":"R Adalbert","year":"2013","journal-title":"Neuropathol Appl Neurobiol"},{"key":"ref94","doi-asserted-by":"crossref","first-page":"619","DOI":"10.1016\/j.mad.2009.07.006","article-title":"Neuropathology of Cockayne syndrome: Evidence for impaired development, premature aging, and neurodegeneration","volume":"130","author":"KM Weidenheim","year":"2009","journal-title":"Mech Ageing Dev"},{"key":"ref95","doi-asserted-by":"crossref","first-page":"305","DOI":"10.1074\/jbc.274.1.305","article-title":"Dual roles for glucokinase in glucose homeostasis as determined by liver and pancreatic beta cell-specific gene knock-outs using Cre recombinase","volume":"274","author":"C Postic","year":"1999","journal-title":"J Biol Chem"},{"key":"ref96","first-page":"212","article-title":"Impaired liver regeneration in Nrf2 knockout mice: role of ROS-mediated insulin\/IGF-1 resistance","volume":"9","author":"XW Beyer TA","year":"2008","journal-title":"EMBO J"},{"key":"ref97","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":"ref98","doi-asserted-by":"crossref","first-page":"1623","DOI":"10.3174\/ajnr.A2135","article-title":"Neuroimaging in Cockayne syndrome","volume":"31","author":"M Koob","year":"2010","journal-title":"AJNR Am J Neuroradiol"},{"key":"ref99","doi-asserted-by":"crossref","first-page":"113","DOI":"10.1111\/j.1440-1789.2011.01241.x","article-title":"Brain vascular changes in Cockayne syndrome","volume":"32","author":"M Hayashi","year":"2012","journal-title":"Neuropathology"},{"key":"ref100","doi-asserted-by":"crossref","first-page":"130","DOI":"10.1002\/gene.20009","article-title":"Dorsal telencephalon-specific expression of Cre recombinase in PAC transgenic mice","volume":"38","author":"T Iwasato","year":"2004","journal-title":"Genesis"},{"key":"ref101","doi-asserted-by":"crossref","first-page":"6145","DOI":"10.1523\/JNEUROSCI.15-09-06145.1995","article-title":"Corticospinal control during reach, grasp, and precision lift in man","volume":"15","author":"RN Lemon","year":"1995","journal-title":"J Neurosci"},{"key":"ref102","doi-asserted-by":"crossref","first-page":"99","DOI":"10.1007\/s12031-009-9186-7","article-title":"The role of Galectin-3\/MAC-2 in the activation of the innate-immune function of phagocytosis in microglia in injury and disease","volume":"39","author":"S Rotshenker","year":"2009","journal-title":"J Mol Neurosci"},{"key":"ref103","doi-asserted-by":"crossref","first-page":"823","DOI":"10.1016\/S0092-8674(02)00795-X","article-title":"Requirement of yeast RAD2, a homolog of human XPG gene, for efficient RNA polymerase II transcription. implications for Cockayne syndrome","volume":"109","author":"SK Lee","year":"2002","journal-title":"Cell"},{"key":"ref104","doi-asserted-by":"crossref","first-page":"10670","DOI":"10.1128\/MCB.24.24.10670-10680.2004","article-title":"Definition of a short region of XPG necessary for TFIIH interaction and stable recruitment to sites of UV damage","volume":"24","author":"F Thorel","year":"2004","journal-title":"Mol Cell Biol"},{"key":"ref105","doi-asserted-by":"crossref","first-page":"1998","DOI":"10.4161\/cc.10.12.15878","article-title":"The DNA repair endonuclease XPG interacts directly and functionally with the WRN helicase defective in Werner syndrome","volume":"10","author":"KS Trego","year":"2011","journal-title":"Cell Cycle"},{"key":"ref106","doi-asserted-by":"crossref","first-page":"1144","DOI":"10.1111\/acel.12142","article-title":"Lifespan extension by dietary intervention in a mouse model of Cockayne syndrome uncouples early postnatal development from segmental progeria","volume":"12","author":"LE Brace","year":"2013","journal-title":"Aging Cell"},{"key":"ref107","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":"ref108","first-page":"185","article-title":"Xeroderma pigmentosum complementation group G associated with Cockayne syndrome","volume":"53","author":"W Vermeulen","year":"1993","journal-title":"Am J Hum Genet"},{"key":"ref109","doi-asserted-by":"crossref","first-page":"e1000161","DOI":"10.1371\/journal.pgen.1000161","article-title":"Delayed and accelerated aging share common longevity assurance mechanisms","volume":"4","author":"B Schumacher","year":"2008","journal-title":"PLoS Genet"},{"key":"ref110","article-title":"Aging: Not All DNA Damage is Equal","author":"WP Vermeij","year":"2014","journal-title":"Curr Opin Genet Dev"},{"key":"ref111","doi-asserted-by":"crossref","first-page":"348","DOI":"10.1002\/jnr.1085","article-title":"Purkinje cell degeneration in mice lacking the xeroderma pigmentosum group G gene","volume":"64","author":"XZ Sun","year":"2001","journal-title":"J Neurosci Res"},{"key":"ref112","doi-asserted-by":"crossref","first-page":"1855","DOI":"10.1021\/pr201203m","article-title":"Synaptic proteome changes in a DNA repair deficient ercc1 mouse model of accelerated aging","volume":"11","author":"MJ Vegh","year":"2012","journal-title":"J Proteome Res"},{"key":"ref113","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":"ref114","doi-asserted-by":"crossref","first-page":"446","DOI":"10.1093\/carcin\/bgi254","article-title":"An in vivo analysis of MMC-induced DNA damage and its repair","volume":"27","author":"YJ Lee","year":"2006","journal-title":"Carcinogenesis"},{"key":"ref115","doi-asserted-by":"crossref","first-page":"4336","DOI":"10.1038\/sj.onc.1210232","article-title":"The role of CSA in the response to oxidative DNA damage in human cells","volume":"26","author":"M D'Errico","year":"2007","journal-title":"Oncogene"},{"key":"ref116","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":"ref117","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":"ref118","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":"ref119","doi-asserted-by":"crossref","first-page":"3","DOI":"10.1016\/S0014-5793(03)00235-7","article-title":"Oxidative stress in cell culture: an under-appreciated problem?","volume":"540","author":"B Halliwell","year":"2003","journal-title":"FEBS Letters"},{"key":"ref120","doi-asserted-by":"crossref","first-page":"194","DOI":"10.1093\/brain\/aws317","article-title":"Auditory analysis of xeroderma pigmentosum 1971\u20132012: hearing function, sun sensitivity and DNA repair predict neurological degeneration","volume":"136","author":"MB 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