Cockayne syndrome B protein regulates the transcriptional program after UV irradiation

Molecular mechanism of nucleotide excision repair

de Laat, W. L., Jaspers, N. G. J., & Hoeijmakers, J. H. J. (1999) · Genes & Development · doi:10.1101/gad.13.7.768 · Cited by 1,122

Different phosphorylated forms of RNA polymerase II and associated mRNA processing factors during transcriptionPreprint

Komarnitsky, P. B., Cho, E., & Buratowski, S. (2000) · Genes & Development · doi:10.1101/gad.824700 · Cited by 1,098

Cockayne syndrome: Review of 140 cases

Nance, M. A., & Berry, S. A. (1992) · American Journal of Medical Genetics · doi:10.1002/ajmg.1320420115 · Cited by 806

ERCC6, a member of a subfamily of putative helicases, is involved in Cockayne's syndrome and preferential repair of active genes

Troelstra, C., van Gool, A., de Wit, J., Vermeulen, W., Bootsma, D., & Hoeijmakers, J. H. J. (1992) · Cell · doi:10.1016/0092-8674(92)90390-x · Cited by 729

Acetylation of histone H4 by Esa1 is required for DNA double-strand break repair

Bird, A. W., Yu, D. Y., Pray-Grant, M. G., Qiu, Q., Harmon, K. E., Megee, P. C., Grant, P. A., Smith, M. M., & Christman, M. F. (2002) · Nature · doi:10.1038/nature01035 · Cited by 565

DNA repair-deficient diseases, xeroderma pigmentosum, Cockayne syndrome and trichothiodystrophy

Lehmann, A. (2003) · Biochimie · doi:10.1016/j.biochi.2003.09.010 · Cited by 518

The Cockayne syndrome group A gene encodes a WD repeat protein that interacts with CSB protein and a subunit of RNA polymerase II TFIIH

Henning, K. A., Li, L., Iyer, N., McDaniel, L. D., Reagan, M. S., Legerski, R., Schultz, R. A., Stefanini, M., Lehmann, A. R., Mayne, L. V., et al. (1995) · Cell · doi:10.1016/0092-8674(95)90028-4 · Cited by 507

Failure of RNA synthesis to recover after UV irradiation; An early defect in cells from individuals with Cockayne syndrome and xeroderma pigmentosum

Mayne, L. V., & Lehmann, A. R. (1982) · Mutation research. Fundamental and molecular mechanisms of mutagenesis · doi:10.1016/0027-5107(82)90047-1 · Cited by 420

ATP-Dependent Chromatin Remodeling by the Cockayne Syndrome B DNA Repair-Transcription-Coupling Factor

Citterio, E., van den Boom, V., Schnitzler, G. R., Kanaar, R., Bonte, E. J., Kingston, R. E., Hoeijmakers, J. H. J., & Vermeulen, W. (2000) · Molecular and Cellular Biology · doi:10.1128/mcb.20.20.7643-7653.2000 · Cited by 376

UV-induced ubiquitination of RNA polymerase II: a novel modification deficient in Cockayne syndrome cells.

Bregman, D. B., Halaban, R., van Gool, A. J., Henning, K. A., Friedberg, E. C., & Warren, S. L. (1996) · Proceedings of the National Academy of Sciences · doi:10.1073/pnas.93.21.11586 · Cited by 331

A subset of human dermal fibroblasts overexpressing Cockayne syndrome group B protein resist UVB radiation‐mediated premature senescence

Fotopoulou, A., Angelopoulou, M. T., Pratsinis, H., Mavrogonatou, E., & Kletsas, D. (2024) · Aging Cell · doi:10.1111/acel.14422 · Cited by 14

From laboratory tests to functional characterisation of Cockayne syndrome

Lanzafame, M., Vaz, B., Nardò, T., Botta, E., Orioli, D., & Stefanini, M. (2013) · Mechanisms of Ageing and Development · doi:10.1016/j.mad.2013.03.007 · Cited by 13

Cockayne syndrome b maintains neural precursor function

Sacco, R., Tamblyn, L., Rajakulendran, N., Bralha, F. N., Tropepe, V., & Laposa, R. R. (2012) · DNA repair · doi:10.1016/j.dnarep.2012.11.004 · Cited by 13

A matter of delicate balance: Loss and gain of Cockayne syndrome proteins in premature aging and cancer

Paccosi, E., Balajee, A. S., & Proietti‐De‐Santis, L. (2022) · Frontiers in Aging · doi:10.3389/fragi.2022.960662 · Cited by 14

DNA Damage-Induced RNAPII Degradation and Its Consequences in Gene Expression

Muñoz, J. C., Beckerman, I., Choudhary, R., Bouvier, L. A., & Muñoz, M. J. (2022) · Genes · doi:10.3390/genes13111951 · Cited by 13

Identification of Novel Proteins Co-Purifying with Cockayne Syndrome Group B (CSB) Reveals Potential Roles for CSB in RNA Metabolism and Chromatin Dynamics

Nicolai, S., Filippi, S., Caputo, M., Čipák, L., Gregáň, J., Ammerer, G., Frontini, M., Willems, D., Prantera, G., Balajee, A. S., et al. (2015) · PLoS ONE · doi:10.1371/journal.pone.0128558 · Cited by 13

Methods to Study Transcription-Coupled Repair in Chromatin

Gaillard, H., Wellinger, R. E., & Aguilera, A. (2009) · Methods in molecular biology · doi:10.1007/978-1-59745-190-1_10 · Cited by 13

Def1 interacts with TFIIH and modulates RNA polymerase II transcription

Damodaren, N., van Eeuwen, T., Zamel, J., Lin-Shiao, E., Kalisman, N., & Murakami, K. (2017) · Proceedings of the National Academy of Sciences · doi:10.1073/pnas.1707955114 · Cited by 13

The emerging role of Cockayne group A and B proteins in ubiquitin/proteasome-directed protein degradation

Paccosi, E., & Proietti‐De‐Santis, L. (2021) · Mechanisms of Ageing and Development · doi:10.1016/j.mad.2021.111466 · Cited by 12

Caffeine Prevents Transcription Inhibition and P-TEFb/7SK Dissociation Following UV-Induced DNA Damage

Napolitano, G., Amente, S., Castiglia, V., Gargano, B., Ruda, V. M., Darzacq, X., Bensaude, O., Majello, B., & Lania, L. (2010) · PLoS ONE · doi:10.1371/journal.pone.0011245 · Cited by 12

Keep calm and reboot – how cells restart transcription after DNA damage and DNA repair

Donnio, L., & Giglia‐Mari, G. (2024) · FEBS Letters · doi:10.1002/1873-3468.14964 · Cited by 11

Sequence-specific double strand breaks trigger P-TEFb-dependent Rpb1-CTD hyperphosphorylation

Napolitano, G., Amente, S., Lavadera, M. L., Palo, G. D., Ambrosio, S., Lania, L., Dellino, G. I., Pelicci, P. G., & Majello, B. (2013) · Mutation research. Fundamental and molecular mechanisms of mutagenesis · doi:10.1016/j.mrfmmm.2013.07.005 · Cited by 11

Studies of lncRNAs in DNA double strand break repair: what is new?

Wu, Z., & Wang, Y. (2017) · Oncotarget · doi:10.18632/oncotarget.22090 · Cited by 10

Identification and characterization of Necdin as a target for the Cockayne syndrome B protein in promoting neuronal differentiation and maintenance

Liang, F., Li, B., Xu, Y., Gong, J., Zheng, S., Zhang, Y., & Wang, Y. (2022) · Pharmacological Research · doi:10.1016/j.phrs.2022.106637 · Cited by 9

RNA polymerase between lesion bypass and DNA repair

Deaconescu, A. M. (2013) · Cellular and Molecular Life Sciences · doi:10.1007/s00018-013-1384-3 · Cited by 10

Identification of Two Missense Mutations of ERCC6 in Three Chinese Sisters with Cockayne Syndrome by Whole Exome Sequencing

Yu, S., Chen, L., Ye, L., Fei, L., Tang, W., Tian, Y., Qian, G., Yi, X., & Xie, J. (2014) · PLoS ONE · doi:10.1371/journal.pone.0113914 · Cited by 9

Transcription‐coupled repair: tangled up in convoluted repair

Schiffmacher, D. A. L., Pai, Y. J., Pines, A., & Vermeulen, W. (2025) · FEBS Journal · doi:10.1111/febs.70104 · Cited by 10

Cellular fractionation reveals transcriptome responses of human fibroblasts to UV-C irradiation

Liu, J., Wu, Z., He, J. H., & Wang, Y. (2022) · Cell Death and Disease · doi:10.1038/s41419-022-04634-x · Cited by 8

Arresting transcription and sentencing the cell: The consequences of blocked transcription

McKay, B. C., & Cabrita, M. A. (2013) · Mechanisms of Ageing and Development · doi:10.1016/j.mad.2013.03.003 · Cited by 9

Neuroblastoma Cells Depend on CSB for Faithful Execution of Cytokinesis and Survival

Paccosi, E., Costantino, M., Balzerano, A., Filippi, S., Brancorsini, S., & Proietti‐De‐Santis, L. (2021) · International Journal of Molecular Sciences · doi:10.3390/ijms221810070 · Cited by 7

RNAPII response to transcription‐blocking DNA lesions in mammalian cells

Wang, J., Sadurnì, M. M., & Saponaro, M. (2022) · FEBS Journal · doi:10.1111/febs.16561 · Cited by 7

Methods to Study Transcription-Coupled Repair in Chromatin

Gaillard, H., Wellinger, R. E., & Aguilera, A. (2015) · Methods in molecular biology · doi:10.1007/978-1-4939-2474-5_15 · Cited by 5

Cockayne syndrome group A protein localizes at centrosomes during mitosis and regulates Cyclin B1 ubiquitination

Paccosi, E., Artemi, G., Filippi, S., Balzerano, A., Costanzo, F., Masci, V. L., Proietti, S., & Proietti‐De‐Santis, L. (2023) · European Journal of Cell Biology · doi:10.1016/j.ejcb.2023.151325 · Cited by 6

Multisystem analyses of two Cockayne syndrome associated proteins CSA and CSB reveal shared and unique functions

Wu, Z., Zhu, X., Qian, Y., Xu, Y., & Wang, Y. (2019) · DNA repair · doi:10.1016/j.dnarep.2019.102696 · Cited by 6

A Chromatin-Focused siRNA Screen for Regulators of p53-Dependent Transcription

Sammons, M. A., Zhu, J., & Berger, S. L. (2016) · G3 Genes Genomes Genetics · doi:10.1534/g3.116.031534 · Cited by 5

Yeast RAD2, a homolog of human XPG, plays a key role in the regulation of the cell cycle and actin dynamics

Kang, M.-S., Yu, S.-L., Kim, H.-Y., Gorospe, C. M., Choi, B. H., Lee, S. H., & Lee, S. (2013) · Biology Open · doi:10.1242/bio.20136403 · Cited by 6

The hardwired transcriptional response to DNA damage

Heilbrun, E. E., Merav, M., Parnas, A., & Adar, S. (2020) · Current Opinion in Systems Biology · doi:10.1016/j.coisb.2020.05.001 · Cited by 5

It Takes a Village of Chromatin Remodelers to Regulate rDNA Expression

Levesque, M. G., & Picketts, D. J. (2025) · International Journal of Molecular Sciences · doi:10.3390/ijms26041772 · Cited by 4

Next‐generation sequencing through multi‐gene panel testing for the diagnosis of a Chinese patient with atypical Cockayne syndrome

Wang, X., Li, Y., Zhao, A., Wang, Y., Cao, Q., Pan, C., & Li, M. (2023) · Molecular Genetics & Genomic Medicine · doi:10.1002/mgg3.2254 · Cited by 4

Distribution of UVC-induced chromosome aberrations along the X chromosome of TCR deficient and proficient Chinese hamster cell lines

Martínez‐López, W., Marotta, E., Tomaso, M. V. D., Méndez-Acuña, L., & Palitti, F. (2010) · Mutation Research/Genetic Toxicology and Environmental Mutagenesis · doi:10.1016/j.mrgentox.2010.02.008 · Cited by 4

From silence to symphony: transcriptional repression and recovery in response to DNA damage

Ajit, K., & Gullerová, M. (2024) · Transcription · doi:10.1080/21541264.2024.2406717 · Cited by 3

Cockayne syndrome

Proietti‐De‐Santis, L., Laugel, V., & Prantera, G. (2019) · Elsevier eBooks · doi:10.1016/b978-0-12-813796-3.00007-9 · Cited by 3

PAF1C restores transcription after DNA damage independently of promoting histone mark deposition

van Schie, J. J. M., de Groot, B. A. F. J., van den Heuvel, D., & Luijsterburg, M. S. (2026) · EMBO Reports · doi:10.1038/s44319-026-00761-0 · Cited by 2

Regulation of active genome integrity and expression by Rad26p

Malik, S., & Bhaumik, S. R. (2014) · Nucleus · doi:10.4161/nucl.36230 · Cited by 2

Genome-wide distribution of Rad26 and Rad1-Rad10 reveals their relationship with Mediator and RNA polymerase IIPreprint

Gopaul, D., Wilkes, C. D., Goldar, A., Aiach, N. G., Barrault, M., Novikova, E., & Soutourina, J. (2021) · bioRxiv (Cold Spring Harbor Laboratory) · doi:10.1101/2021.10.20.465181 · Cited by 3

Genomic analysis of Rad26 and Rad1–Rad10 reveals differences in their dependence on Mediator and RNA polymerase IIPreprint

Gopaul, D., Wilkes, C. D., Goldar, A., Aiach, N. G., Barrault, M., Novikova, E., & Soutourina, J. (2022) · Genome Research · doi:10.1101/gr.276371.121 · Cited by 2

Stalling of elongating Pol II triggers Ser7 phosphorylation in trans to drive transcription recoveryPreprint

Shlamovitz, I., Bao, Y., Toteva, T., Crisp, A., Ye, S., Wingett, S., Cacioppo, R., Edmondson, A., Castiblanco, D., Boemo, M. A., et al. (2025) · bioRxiv (Cold Spring Harbor Laboratory) · doi:10.64898/2025.12.04.692291 · Cited by 2

Cockayne syndrome B protein is implicated in transcription and associated chromatin dynamics in homeostatic and genotoxic conditions

Liakos, A., Ntakou‐Zamplara, K. Z., Angelova, N., Konstantopoulos, D., Synacheri, A., Spyropoulou, Z., Tsarmaklis, I. A., Korrou‐Karava, D., Nikolopoulos, G., Lavigne, M. D., et al. (2024) · Aging Cell · doi:10.1111/acel.14341 · Cited by 1

Cockayne Syndrome

Stefanini, M., & Ruggieri, M. (2008) · Springer eBooks · doi:10.1007/978-3-211-69500-5_52 · Cited by 1

Influence of chromatin remodeling in the removal of UVC-induced damage in TCR proficient and deficient Chinese hamster cells

Martínez‐López, W., Moreno-Ortega, D., Valencia-Payan, J., Sammader, P., Meschini, R., & Palitti, F. (2018) · Mutation Research/Genetic Toxicology and Environmental Mutagenesis · doi:10.1016/j.mrgentox.2018.08.003 · Cited by 1

Correction: Caffeine Prevents Transcription Inhibition and P-TEFb/7SK Dissociation Following UV-Induced DNA Damage

Napolitano, G., Amente, S., Castiglia, V., Gargano, B., Ruda, V. M., Darzacq, X., Bensaude, O., Majello, B., & Lania, L. (2013) · PLoS ONE · doi:10.1371/annotation/753c6a63-2aea-4cb8-b38c-28db972d60df · Cited by 1

lncRNA-ZFAS1, an emerging gate-keeper in DNA damage-dependent transcriptional regulationPreprint

Wang, Y. (2023) · Research Square · doi:10.21203/rs.3.rs-3233912/v1 · Cited by 1

A universal molecular mechanism driving agingPreprint

Jin, W., Zheng, J., Xiao, Y., Ju, L., Chen, F., Fu, J., Jiang, H., & Zhang, Y. (2024) · bioRxiv (Cold Spring Harbor Laboratory) · doi:10.1101/2024.01.06.574476 · Cited by 1

New players at the interface between transcription and DNA repair

Zhovmer, A. S. (2012) · theses.fr (ABES) · doi:10.70675/cc1e96b9ze648z4edbzabccz5daa0423cd5f

Transcriptional Functions of DNA Repair Proteins Involved in Premature Aging

Assfalg, R., & Ibe, S. (2011) · InTech eBooks · doi:10.5772/24388

A possible cranio-oro-facial phenotype in

Bloch‐Zupan, A., Rousseaux, M., Laugel-Haushalter, V., Schmittbuhl, M., Mathis, R., Desforges, E., Koob, M., Dollfus, H., & Laugel, V. (2013)

The roles of transcription factors in Nucleotide excision repair in yeast

Ding, B. (2010) · Louisiana State University · doi:10.31390/gradschool_dissertations.3791

Cockayne syndrome

Regenbogen, L. (2020) · Definitions · doi:10.1002/9781119013112.ch35 · Cited by 57

Cockayne syndrome in an Iranian pedigree with a homozygous missense variant in the ERCC6 gene

Nikfar, A., Mansouri, M., Chiti, H., Abhari, G. F., & Parsamanesh, N. (2022) · Gene Reports · doi:10.1016/j.genrep.2022.101665

Repair Mechanisms and Initiation in Carcinogenesis

Rebok, A., & Spratt, T. E. (2024) · Elsevier eBooks · doi:10.1016/b978-0-323-95488-4.00089-9

Bypass of Blocking Lesions by RNAPII Impairs the Transcriptional DNA Damage ResponsePreprint

Bañuelos, C. P., Caeiro, L. D., Cingaram, P. R., Beckedorff, F., Morey, L., Cortes, D. B., Shiekhattar, R., & Verdún, R. E. (2025) · bioRxiv (Cold Spring Harbor Laboratory) · doi:10.1101/2025.04.28.651040

IncRNA‐ZFAS1, an Emerging Gate‐Keeper in DNA Damage‐Dependent Transcriptional Regulation

Liu, J., Lu, Q., Fan, Z., Lin, J., He, N., Zhang, X., Han, Z., Zhu, T., Wu, Z., Xu, Y., et al. (2025) · Advanced Science · doi:10.1002/advs.202412385 · Cited by 1

PAF1C-driven restoration of RNAPII elongation after DNA damage occurs independently of transcription-associated histone mark depositionPreprint

van Schie, J. J. M., de Groot, B. A. F. J., & Luijsterburg, M. S. (2025) · bioRxiv (Cold Spring Harbor Laboratory) · doi:10.1101/2025.07.23.666359

Transcription Associated Recombination in Mammalian Cells

Savolainen, L. (2010) · DiVA (Stockholm University)

Bypass of blocking lesions by RNAPII reveals a novel stress induced by DNA damagePreprint

Bañuelos, C. P., Caeiro, L. D., Cingaram, P. R., Beckedorff, F., Morey, L., Cortes, D. B., Shiekhattar, R., & Verdún, R. E. (2026) · Genes & Development · doi:10.1101/gad.353164.125

Involvement of TFIIH in NER factors mediated chromatin remodeling

Singh, A. (2014) · theses.fr (ABES) · doi:10.70675/9a7cf956z3bdaz44c5za560za206d78e4cea

Role of NER factors in transcription

Costanzo, F. (2017) · theses.fr (ABES) · doi:10.70675/64c5c64bza3c5z49ffzbcb7z49be745e72fd

Le facteur de réparation XPC est un cofacteur de l'ARN polymérase II régulant les modifications post-traductionnelles des histones lors de la transcription

Semer, M. (2018) · theses.fr (ABES) · doi:10.70675/8f882ab9z86fcz4247z8553z252d06504550

Study of the molecular mechanisms linking transcription and DNA repair in Saccharomyces cerevisiae

Gopaul, D. (2018) · theses.fr (ABES) · doi:10.70675/cf586e1bz5dafz40b7zb5c9z33319e0b967b

Contrôle de l'expression génique par la RNP 7SK

Studniarek, C. (2019) · theses.fr (ABES) · doi:10.70675/4bab1779z812bz45d6zbcd4z9eea765b52e7

Mechanisms of precocious ageing in a human progeroid syndrome

Molina, C. F. (2021) · theses.fr (ABES) · doi:10.70675/f78c9fa2z621cz48b6zbf00z1133c36b723a

RNA polymerase II removal: transcription quality control and gene regulation

Haworth, B., Abyaneh, P., & Vidaković, A. T. (2026) · Transcription · doi:10.1080/21541264.2026.2698182

Die Rolle des CSB-Proteins bei der neuronalen Entwicklung und Hypoxie-induzierten Transkription

Schumacher, C. M. (2012) · Univ. Duesseldorf: Duesseldorfer Dokumenten- und Publikationsserver

Transcription-Coupled Repair and Its Defect in Cockayne Syndrome

Ahmad, S. I. (2009) · CRC Press eBooks · doi:10.1201/9781498712705-8

Regulatory interplay of Cockayne syndrome B ATPase and stress-response gene ATF3 following genotoxic stress

Kristensen, U., Epanchintsev, A., Rauschendorf, M., Laugel, V., Stevnsner, T., Bohr, V. A., Coin, F., & Egly, J. (2013) · Proceedings of the National Academy of Sciences · doi:10.1073/pnas.1220071110 · Cited by 63

UV-induced inhibition of transcription involves repression of transcription initiation and phosphorylation of RNA polymerase II

Rockx, D., Mason, R. S., van Hoffen, A., Barton, M. C., Citterio, E., Bregman, D. B., van Zeeland, A. A., Vrieling, H., & Mullenders, L. H. F. (2000) · Proceedings of the National Academy of Sciences · doi:10.1073/pnas.180169797 · Cited by 203

Cockayne syndrome B protein is implicated in transcription and associated chromatin dynamics in homeostatic and genotoxic conditions

Liakos, A., Ntakou‐Zamplara, K. Z., Angelova, N., Konstantopoulos, D., Synacheri, A., Spyropoulou, Z., Tsarmaklis, I. A., Korrou‐Karava, D., Nikolopoulos, G., Lavigne, M. D., et al. (2024) · Aging Cell · doi:10.1111/acel.14341 · Cited by 1

Cockayne’s Syndrome A and B Proteins Regulate Transcription Arrest after Genotoxic Stress by Promoting ATF3 Degradation

Epanchintsev, A., Costanzo, F., Rauschendorf, M., Caputo, M., Ye, T., Donnio, L., Proietti‐De‐Santis, L., Coin, F., Laugel, V., & Egly, J. (2017) · Molecular Cell · doi:10.1016/j.molcel.2017.11.009 · Cited by 104