{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,25]],"date-time":"2026-09-25T21:02:25Z","timestamp":1790370145988,"version":"4.1.0"},"reference-count":79,"publisher":"Oxford University Press (OUP)","issue":"19","license":[{"start":{"date-parts":[[2021,9,28]],"date-time":"2021-09-28T00:00:00Z","timestamp":1632787200000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by-nc\/4.0\/"}],"funder":[{"DOI":"10.13039\/501100005010","name":"Associazione Italiana per la Ricerca sul Cancro","doi-asserted-by":"publisher","award":["IG 21737"],"award-info":[{"award-number":["IG 21737"]}],"id":[{"id":"10.13039\/501100005010","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/501100005010","name":"Associazione Italiana per la Ricerca sul Cancro","doi-asserted-by":"publisher","award":["13537"],"award-info":[{"award-number":["13537"]}],"id":[{"id":"10.13039\/501100005010","id-type":"DOI","asserted-by":"publisher"}]},{"name":"Institutional Agreements\/Regione Lombardia"},{"name":"PRIN","award":["2008P772PW"],"award-info":[{"award-number":["2008P772PW"]}]}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":[],"published-print":{"date-parts":[[2021,11,8]]},"abstract":"<jats:title>Abstract<\/jats:title>\n               <jats:p>CSA and CSB proteins are key players in transcription-coupled nucleotide excision repair (TC-NER) pathway that removes UV-induced DNA lesions from the transcribed strands of expressed genes. Additionally, CS proteins play relevant but still elusive roles in other cellular pathways whose alteration may explain neurodegeneration and progeroid features in Cockayne syndrome (CS). Here we identify a CS-containing chromatin-associated protein complex that modulates rRNA transcription. Besides RNA polymerase I (RNAP1) and specific ribosomal proteins (RPs), the complex includes ferrochelatase (FECH), a well-known mitochondrial enzyme whose deficiency causes erythropoietic protoporphyria (EPP). Impairment of either CSA or FECH functionality leads to reduced RNAP1 occupancy on rDNA promoter that is associated to reduced 47S pre-rRNA transcription. In addition, reduced FECH expression leads to an abnormal accumulation of 18S rRNA that in primary dermal fibroblasts from CS and EPP patients results in opposed rRNA amounts. After cell irradiation with UV light, CSA triggers the dissociation of the CSA\u2013FECH\u2013CSB\u2013RNAP1\u2013RPs complex from the chromatin while it stabilizes its binding to FECH. Besides disclosing a function for FECH within nucleoli, this study sheds light on the still unknown mechanisms through which CSA modulates rRNA transcription.<\/jats:p>","DOI":"10.1093\/nar\/gkab819","type":"journal-article","created":{"date-parts":[[2021,9,12]],"date-time":"2021-09-12T11:06:53Z","timestamp":1631444813000},"page":"10911-10930","source":"Crossref","is-referenced-by-count":10,"title":["Cockayne syndrome group A and ferrochelatase finely tune ribosomal gene transcription and its response to UV irradiation"],"prefix":"10.1093","volume":"49","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-0456-1134","authenticated-orcid":false,"given":"Manuela","family":"Lanzafame","sequence":"first","affiliation":[{"name":"Institute of Molecular Genetics -L.L. Cavalli Sforza, CNR, 27100 Pavia, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Giulia","family":"Branca","sequence":"additional","affiliation":[{"name":"Institute of Molecular Genetics -L.L. Cavalli Sforza, CNR, 27100 Pavia, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Claudia","family":"Landi","sequence":"additional","affiliation":[{"name":"Department of Life Sciences, University of Siena, 53100 Siena, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Mingyue","family":"Qiang","sequence":"additional","affiliation":[{"name":"Department of Dermatology and Allergic Diseases, Ulm University, Albert-Einstein Allee 23, 89081 Ulm, Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Bruno","family":"Vaz","sequence":"additional","affiliation":[{"name":"Institute of Molecular Genetics -L.L. Cavalli Sforza, CNR, 27100 Pavia, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Tiziana","family":"Nardo","sequence":"additional","affiliation":[{"name":"Institute of Molecular Genetics -L.L. Cavalli Sforza, CNR, 27100 Pavia, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Debora","family":"Ferri","sequence":"additional","affiliation":[{"name":"Institute of Molecular Genetics -L.L. Cavalli Sforza, CNR, 27100 Pavia, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Manuela","family":"Mura","sequence":"additional","affiliation":[{"name":"Institute of Molecular Genetics -L.L. Cavalli Sforza, CNR, 27100 Pavia, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-5207-2861","authenticated-orcid":false,"given":"Sebastian","family":"Iben","sequence":"additional","affiliation":[{"name":"Department of Dermatology and Allergic Diseases, Ulm University, Albert-Einstein Allee 23, 89081 Ulm, Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Miria","family":"Stefanini","sequence":"additional","affiliation":[{"name":"Institute of Molecular Genetics -L.L. Cavalli Sforza, CNR, 27100 Pavia, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Fiorenzo\u00a0A","family":"Peverali","sequence":"additional","affiliation":[{"name":"Institute of Molecular Genetics -L.L. Cavalli Sforza, CNR, 27100 Pavia, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Luca","family":"Bini","sequence":"additional","affiliation":[{"name":"Department of Life Sciences, University of Siena, 53100 Siena, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-3830-3408","authenticated-orcid":false,"given":"Donata","family":"Orioli","sequence":"additional","affiliation":[{"name":"Institute of Molecular Genetics -L.L. Cavalli Sforza, CNR, 27100 Pavia, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"286","published-online":{"date-parts":[[2021,9,28]]},"reference":[{"key":"2021110312465192800_B1","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":"2021110312465192800_B2","doi-asserted-by":"crossref","first-page":"3","DOI":"10.1016\/j.arr.2016.08.002","article-title":"Cockayne syndrome: clinical features, model systems and pathways","volume":"33","author":"Karikkineth","year":"2017","journal-title":"Ageing Res. Rev."},{"key":"2021110312465192800_B3","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. Med. Genet."},{"key":"2021110312465192800_B4","doi-asserted-by":"crossref","first-page":"465","DOI":"10.1038\/nrm3822","article-title":"Understanding nucleotide excision repair and its roles in cancer and ageing","volume":"15","author":"Marteijn","year":"2014","journal-title":"Nat. Rev. Mol. Cell Biol."},{"key":"2021110312465192800_B5","doi-asserted-by":"crossref","first-page":"202","DOI":"10.1016\/j.mad.2013.02.004","article-title":"Structure, function and regulation of CSB: a multi-talented gymnast","volume":"134","author":"Lake","year":"2013","journal-title":"Mech. Ageing Dev."},{"key":"2021110312465192800_B6","doi-asserted-by":"crossref","first-page":"1429","DOI":"10.1101\/gad.378206","article-title":"CSA-dependent degradation of CSB by the ubiquitin-proteasome pathway establishes a link between complementation factors of the Cockayne syndrome","volume":"20","author":"Groisman","year":"2006","journal-title":"Genes Dev."},{"key":"2021110312465192800_B7","doi-asserted-by":"crossref","first-page":"1024","DOI":"10.1016\/j.cell.2011.10.035","article-title":"The molecular basis of CRL4DDB2\/CSA ubiquitin ligase architecture, targeting, and activation","volume":"147","author":"Fischer","year":"2011","journal-title":"Cell"},{"key":"2021110312465192800_B8","doi-asserted-by":"crossref","first-page":"1054","DOI":"10.1016\/j.molcel.2017.11.009","article-title":"Cockayne's syndrome A and B proteins regulate transcription arrest after genotoxic stress by promoting ATF3 degradation","volume":"68","author":"Epanchintsev","year":"2017","journal-title":"Mol. 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