{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,8,20]],"date-time":"2026-08-20T14:52:35Z","timestamp":1787237555953,"version":"build-2736575974"},"reference-count":111,"publisher":"MDPI AG","issue":"4","license":[{"start":{"date-parts":[[2021,4,10]],"date-time":"2021-04-10T00:00:00Z","timestamp":1618012800000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"funder":[{"name":"European Research Council","award":["309612"],"award-info":[{"award-number":["309612"]}]},{"name":"Horizon 2020 Marie Sklodowska-Curie","award":["722729"],"award-info":[{"award-number":["722729"]}]},{"DOI":"10.13039\/501100003448","name":"General Secretariat for Research and Technology","doi-asserted-by":"publisher","award":["Flagship Initiative to address SARS-CoV-2 (2020\u03a3\u039501300001)"],"award-info":[{"award-number":["Flagship Initiative to address SARS-CoV-2 (2020\u03a3\u039501300001)"]}],"id":[{"id":"10.13039\/501100003448","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/501100013209","name":"Hellenic Foundation for Research and Innovation","doi-asserted-by":"publisher","award":["grants no. 775 and 3782"],"award-info":[{"award-number":["grants no. 775 and 3782"]}],"id":[{"id":"10.13039\/501100013209","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/501100005187","name":"National and Kapodistrian University of Athens","doi-asserted-by":"publisher","award":["SARG grant 70\/3\/8916"],"award-info":[{"award-number":["SARG grant 70\/3\/8916"]}],"id":[{"id":"10.13039\/501100005187","id-type":"DOI","asserted-by":"publisher"}]},{"name":"Welfare Foundation for Social &amp; Cultural Sciences","award":["KIKPE"],"award-info":[{"award-number":["KIKPE"]}]},{"name":"Pappas","award":["donation"],"award-info":[{"award-number":["donation"]}]}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Cells"],"abstract":"<jats:p>Cockayne syndrome (CS) is a DNA repair syndrome characterized by a broad spectrum of clinical manifestations such as neurodegeneration, premature aging, developmental impairment, photosensitivity and other symptoms. Mutations in Cockayne syndrome protein B (CSB) are present in the vast majority of CS patients and in other DNA repair-related pathologies. In the literature, the role of CSB in different DNA repair pathways has been highlighted, however, new CSB functions have been identified in DNA transcription, mitochondrial biology, telomere maintenance and p53 regulation. Herein, we present an overview of identified structural elements and processes that impact on CSB activity and its post-translational modifications, known to balance the different roles of the protein not only during normal conditions but most importantly in stress situations. Moreover, since CSB has been found to be overexpressed in a number of different tumors, its role in cancer is presented and possible therapeutic targeting is discussed.<\/jats:p>","DOI":"10.3390\/cells10040866","type":"journal-article","created":{"date-parts":[[2021,4,12]],"date-time":"2021-04-12T03:04:06Z","timestamp":1618196646000},"page":"866","update-policy":"https:\/\/doi.org\/10.3390\/mdpi_crossmark_policy","source":"Crossref","is-referenced-by-count":19,"title":["Cockayne Syndrome Group B (CSB): The Regulatory Framework Governing the Multifunctional Protein and Its Plausible Role in Cancer"],"prefix":"10.3390","volume":"10","author":[{"given":"Zoi","family":"Spyropoulou","sequence":"first","affiliation":[{"name":"Biomedical Sciences Research Center Alexander Fleming, Institute for Fundamental Biomedical Research, 16672 Athens, Greece"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Angelos","family":"Papaspyropoulos","sequence":"additional","affiliation":[{"name":"Molecular Carcinogenesis Group, Department of Histology and Embryology, School of Medicine, National and Kapodistrian University of Athens, 11527 Athens, Greece"},{"name":"Biomedical Research Foundation of the Academy of Athens, 11527 Athens, Greece"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Nefeli","family":"Lagopati","sequence":"additional","affiliation":[{"name":"Molecular Carcinogenesis Group, Department of Histology and Embryology, School of Medicine, National and Kapodistrian University of Athens, 11527 Athens, Greece"},{"name":"Biomedical Research Foundation of the Academy of Athens, 11527 Athens, Greece"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-8359-9634","authenticated-orcid":false,"given":"Vassilios","family":"Myrianthopoulos","sequence":"additional","affiliation":[{"name":"Department of Pharmacy, National and Kapodistrian University of Athens, 15771 Athens, Greece"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-5971-0010","authenticated-orcid":false,"given":"Alexandros G.","family":"Georgakilas","sequence":"additional","affiliation":[{"name":"DNA Damage Laboratory, Physics Department, School of Applied Mathematical and Physical Sciences, National Technical University of Athens (NTUA), 15780 Athens, Greece"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-1729-5899","authenticated-orcid":false,"given":"Maria","family":"Fousteri","sequence":"additional","affiliation":[{"name":"Biomedical Sciences Research Center Alexander Fleming, Institute for Fundamental Biomedical Research, 16672 Athens, Greece"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Athanassios","family":"Kotsinas","sequence":"additional","affiliation":[{"name":"Molecular Carcinogenesis Group, Department of Histology and Embryology, School of Medicine, National and Kapodistrian University of Athens, 11527 Athens, Greece"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-9001-4112","authenticated-orcid":false,"given":"Vassilis G.","family":"Gorgoulis","sequence":"additional","affiliation":[{"name":"Molecular Carcinogenesis Group, Department of Histology and Embryology, School of Medicine, National and Kapodistrian University of Athens, 11527 Athens, Greece"},{"name":"Biomedical Research Foundation of the Academy of Athens, 11527 Athens, Greece"},{"name":"Faculty of Biology, Medicine and Health, Manchester Academic Health Science Centre, University of Manchester, Manchester M20 4GJ, UK"},{"name":"Center for New Biotechnologies and Precision Medicine, Medical School, National and Kapodistrian University of Athens, 11527 Athens, Greece"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"1968","published-online":{"date-parts":[[2021,4,10]]},"reference":[{"key":"ref_1","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":"Kraemer","year":"2007","journal-title":"Neuroscience"},{"key":"ref_2","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 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