{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,8,17]],"date-time":"2026-08-17T17:14:28Z","timestamp":1786986868364,"version":"build-2736575974"},"reference-count":25,"publisher":"Elsevier BV","issue":"7","license":[{"start":{"date-parts":[[2015,7,1]],"date-time":"2015-07-01T00:00:00Z","timestamp":1435708800000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/www.elsevier.com\/tdm\/userlicense\/1.0\/"},{"start":{"date-parts":[[2015,7,1]],"date-time":"2015-07-01T00:00:00Z","timestamp":1435708800000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/www.elsevier.com\/legal\/tdmrep-license"}],"content-domain":{"domain":["clinicalkey.com","clinicalkey.com.au","clinicalkey.es","clinicalkey.fr","clinicalkey.jp","elsevier.com","sciencedirect.com"],"crossmark-restriction":true},"short-container-title":["Clinical Neurophysiology"],"published-print":{"date-parts":[[2015,7]]},"DOI":"10.1016\/j.clinph.2014.10.014","type":"journal-article","created":{"date-parts":[[2014,10,19]],"date-time":"2014-10-19T22:47:52Z","timestamp":1413758872000},"page":"1435-1439","update-policy":"https:\/\/doi.org\/10.1016\/elsevier_cm_policy","source":"Crossref","is-referenced-by-count":16,"title":["Progressive demyelinating neuropathy correlates with clinical severity in Cockayne syndrome"],"prefix":"10.1016","volume":"126","author":[{"given":"Cyril","family":"Gitiaux","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Nathalie","family":"Blin-Rochemaure","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Marie","family":"Hully","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Andoni","family":"Echaniz-Laguna","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Nad\u00e8ge","family":"Calmels","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Nadia","family":"Bahi-Buisson","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Isabelle","family":"Desguerre","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ivana","family":"Dabaj","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Samer","family":"Wehbi","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Susana","family":"Quijano-Roy","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Vincent","family":"Laugel","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"78","reference":[{"key":"10.1016\/j.clinph.2014.10.014_b0005","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"},{"key":"10.1016\/j.clinph.2014.10.014_b0010","series-title":"Pediatric clinical electromyography","author":"Bolton","year":"1995"},{"key":"10.1016\/j.clinph.2014.10.014_b0015","doi-asserted-by":"crossref","first-page":"531","DOI":"10.1002\/mus.10569","article-title":"Multifocal slowing of nerve conduction in metachromatic leukodystrophy","volume":"29","author":"Cameron","year":"2004","journal-title":"Muscle Nerve"},{"key":"10.1016\/j.clinph.2014.10.014_b0020","doi-asserted-by":"crossref","first-page":"284","DOI":"10.1016\/j.mad.2013.02.005","article-title":"Conceptual developments in the causes of Cockayne syndrome","volume":"134","author":"Cleaver","year":"2013","journal-title":"Mech Ageing Dev"},{"key":"10.1016\/j.clinph.2014.10.014_b0025","doi-asserted-by":"crossref","first-page":"555","DOI":"10.1016\/0092-8674(95)90028-4","article-title":"The Cockayne syndrome group A gene encodes a WD repeat protein that interacts with CSB protein and a subunit of RNA polymerase II TFIIH","volume":"82","author":"Henning","year":"1995","journal-title":"Cell"},{"key":"10.1016\/j.clinph.2014.10.014_b0030","doi-asserted-by":"crossref","first-page":"180","DOI":"10.1016\/j.mad.2013.04.003","article-title":"Cockayne syndrome pathogenesis: lessons from mouse models","volume":"134","author":"Jaarsma","year":"2013","journal-title":"Mech Ageing Dev"},{"key":"10.1016\/j.clinph.2014.10.014_b0035","doi-asserted-by":"crossref","first-page":"744","DOI":"10.1016\/j.dnarep.2008.01.014","article-title":"Incidence of DNA repair deficiency disorders in western Europe: xeroderma pigmentosum, Cockayne syndrome and trichothiodystrophy","volume":"7","author":"Kleijer","year":"2008","journal-title":"DNA Repair"},{"key":"10.1016\/j.clinph.2014.10.014_b0040","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.clinph.2014.10.014_b0045","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.clinph.2014.10.014_b0050","doi-asserted-by":"crossref","first-page":"320","DOI":"10.1038\/sj.ejhg.5201991","article-title":"Deletion of 5\u2032 sequences of the CSB gene provides insight into the pathophysiology of Cockayne syndrome","volume":"16","author":"Laugel","year":"2008","journal-title":"Eur J Hum Genet"},{"key":"10.1016\/j.clinph.2014.10.014_b0055","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":"Leech","year":"1985","journal-title":"J Neuropathol Exp Neurol"},{"key":"10.1016\/j.clinph.2014.10.014_b0060","doi-asserted-by":"crossref","first-page":"225","DOI":"10.1053\/ejpn.2001.0523","article-title":"Xeroderma pigmentosum\/Cockayne syndrome complex: first neuropathological study and review of eight other cases","volume":"5","author":"Lindenbaum","year":"2001","journal-title":"Eur J Paediatr Neurol"},{"key":"10.1016\/j.clinph.2014.10.014_b0065","first-page":"1473","article-title":"Failure of RNA synthesis to recover after UV irradiation: an early defect in cells from individuals with Cockayne\u2019s syndrome and xeroderma pigmentosum","volume":"42","author":"Mayne","year":"1982","journal-title":"Cancer Res"},{"key":"10.1016\/j.clinph.2014.10.014_b0070","doi-asserted-by":"crossref","first-page":"205","DOI":"10.1002\/mus.880080305","article-title":"Acquired versus familial demyelinative neuropathies in children","volume":"8","author":"Miller","year":"1985","journal-title":"Muscle Nerve"},{"key":"10.1016\/j.clinph.2014.10.014_b0075","doi-asserted-by":"crossref","first-page":"674","DOI":"10.1136\/adc.45.243.674","article-title":"Peripheral neuropathy in Cockayne\u2019s syndrome","volume":"45","author":"Moosa","year":"1970","journal-title":"Arch Dis Child"},{"key":"10.1016\/j.clinph.2014.10.014_b0080","doi-asserted-by":"crossref","first-page":"68","DOI":"10.1002\/ajmg.1320420115","article-title":"Cockayne syndrome: review of 140 cases","volume":"42","author":"Nance","year":"1992","journal-title":"Am J Med Genet"},{"key":"10.1016\/j.clinph.2014.10.014_b0085","doi-asserted-by":"crossref","first-page":"e1000031","DOI":"10.1371\/journal.pgen.1000031","article-title":"An abundant evolutionarily conserved CSB-PiggyBac fusion protein expressed in Cockayne syndrome","volume":"4","author":"Newman","year":"2008","journal-title":"PLoS Genet"},{"key":"10.1016\/j.clinph.2014.10.014_b0090","doi-asserted-by":"crossref","first-page":"163","DOI":"10.1002\/mus.880100210","article-title":"Primary segmental demyelination in the sural nerve in Cockayne\u2019s syndrome","volume":"10","author":"Ohnishi","year":"1987","journal-title":"Muscle Nerve"},{"key":"10.1016\/j.clinph.2014.10.014_b0095","doi-asserted-by":"crossref","first-page":"312","DOI":"10.1016\/S0887-8994(96)00229-9","article-title":"Cockayne syndrome: review of 25 cases","volume":"15","author":"\u00d6zdirim","year":"1996","journal-title":"Pediatr Neurol"},{"key":"10.1016\/j.clinph.2014.10.014_b0100","doi-asserted-by":"crossref","first-page":"114","DOI":"10.1016\/S0387-7604(12)80098-2","article-title":"Demyelinating peripheral neuropathy in Cockayne syndrome: a histopathologic and morphometric study","volume":"14","author":"Sasaki","year":"1992","journal-title":"Brain Dev"},{"key":"10.1016\/j.clinph.2014.10.014_b0105","doi-asserted-by":"crossref","first-page":"161","DOI":"10.1055\/s-2008-1059617","article-title":"Peripheral and central myelinopathy in Cockayne\u2019s syndrome. Report of 3 siblings","volume":"13","author":"Smits","year":"1982","journal-title":"Neuropediatrics"},{"key":"10.1016\/j.clinph.2014.10.014_b0110","doi-asserted-by":"crossref","first-page":"418","DOI":"10.1007\/BF02267059","article-title":"Genetic analysis of twenty-two patients with Cockayne syndrome","volume":"97","author":"Stefanini","year":"1996","journal-title":"Hum Genet"},{"key":"10.1016\/j.clinph.2014.10.014_b0115","doi-asserted-by":"crossref","first-page":"939","DOI":"10.1016\/0092-8674(92)90390-X","article-title":"ERCC6, a member of a subfamily of putative helicases, is involved in Cockayne\u2019s syndrome and preferential repair of active genes","volume":"71","author":"Troelstra","year":"1992","journal-title":"Cell"},{"key":"10.1016\/j.clinph.2014.10.014_b0120","doi-asserted-by":"crossref","first-page":"153","DOI":"10.1007\/BF00697396","article-title":"The neuropathy of Cockayne syndrome","volume":"61","author":"Vos","year":"1983","journal-title":"Acta Neuropathol"},{"key":"10.1016\/j.clinph.2014.10.014_b0125","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":"Weidenheim","year":"2009","journal-title":"Mech Ageing Dev"}],"container-title":["Clinical Neurophysiology"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/api.elsevier.com\/content\/article\/PII:S1388245714005549?httpAccept=text\/xml","content-type":"text\/xml","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/api.elsevier.com\/content\/article\/PII:S1388245714005549?httpAccept=text\/plain","content-type":"text\/plain","content-version":"vor","intended-application":"text-mining"}],"deposited":{"date-parts":[[2025,10,25]],"date-time":"2025-10-25T11:26:25Z","timestamp":1761391585000},"score":1,"resource":{"primary":{"URL":"https:\/\/linkinghub.elsevier.com\/retrieve\/pii\/S1388245714005549"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2015,7]]},"references-count":25,"journal-issue":{"issue":"7","published-print":{"date-parts":[[2015,7]]}},"alternative-id":["S1388245714005549"],"URL":"https:\/\/doi.org\/10.1016\/j.clinph.2014.10.014","relation":{},"ISSN":["1388-2457"],"issn-type":[{"value":"1388-2457","type":"print"}],"subject":[],"published":{"date-parts":[[2015,7]]},"assertion":[{"value":"Elsevier","name":"publisher","label":"This article is maintained by"},{"value":"Progressive demyelinating neuropathy correlates with clinical severity in Cockayne syndrome","name":"articletitle","label":"Article Title"},{"value":"Clinical Neurophysiology","name":"journaltitle","label":"Journal Title"},{"value":"https:\/\/doi.org\/10.1016\/j.clinph.2014.10.014","name":"articlelink","label":"CrossRef DOI link to publisher maintained version"},{"value":"article","name":"content_type","label":"Content Type"},{"value":"Copyright \u00a9 2014 International Federation of Clinical Neurophysiology. Published by Elsevier Ireland Ltd. All rights reserved.","name":"copyright","label":"Copyright"}]}}