Cockayne syndrome B protein regulates the transcriptional program after UV irradiation
Open accessAbstract
The phenotype of the human genetic disorder Cockayne syndrome (CS) is not only due to DNA repair defect but also (and perhaps essentially) to a severe transcription initiation defect. After UV irradiation, even undamaged genes are not transcribed in CSB cells. Indeed, neither RNA pol II nor the associated basal transcription factors are recruited to the promoters of the housekeeping genes, around of which histone H4 acetylation is also deficient. Transfection of CSB restores the recruitment process of RNA pol II. On the contrary, the p53-responsive genes do not require CSB and are transcribed in both wild-type and CSB cells upon DNA damage. Altogether, our data highlight the pivotal role of CSB in initiating the transcriptional program of certain genes after UV irradiation, and also may explain some of the complex traits of CS patients.
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Proietti-De-Santis et al. showed that CSB is required during the first phases of RNA polymerase II transcription initiation in living human cells.
“In living human cells, Proietti-De-Santis et al. [225] have shown that CSB is required during the first phases of RNA pol II transcription initiation.”
From Molecular Mechanisms of Ultraviolet Radiation‐Induced DNA Damage and Repair (2010) · cites this paper as [225]
In “CSB is required for transcription initiation after UV irradiation,” the paper reports impaired initiation-complex formation and RNA polymerase II recruitment at certain promoters in UV-irradiated CSB cells. The citing sentence omits that UV-irradiation condition and generalizes the requirement: “CSB does not participate in p53-responsive gene expression” says those genes can be transcribed without CSB. Page numbers were not provided in our copy. (LLM, conf=0.87)
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Self-citationCockayne Syndrome group B protein stimulates NEIL2 DNA glycosylase activity
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Truncated Cockayne Syndrome B Protein Represses Elongation by RNA Polymerase I
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A new connection of mRNP biogenesis and export with transcription-coupled repair
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Traveling Rocky Roads: The Consequences of Transcription-Blocking DNA Lesions on RNA Polymerase II
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Transcription-coupled DNA double-strand break repair
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Interaction between the Cockayne syndrome B and p53 proteins: implications for aging
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Self-citationNucleotide excision repair in chromatin: Damage removal at the drop of a HAT
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Etoposide-induced DNA damage affects multiple cellular pathways in addition to DNA damage response
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Conceptual developments in the causes of Cockayne syndrome
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Self-citationA possible cranio-oro-facial phenotype in Cockayne syndrome
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Transcription-coupled repair and the transcriptional response to UV-Irradiation
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Repair of oxidatively generated DNA damage in Cockayne syndrome
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Defective transcription of ATF3 responsive genes, a marker for Cockayne Syndrome
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The current evidence for defective repair of oxidatively damaged DNA in Cockayne syndrome
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Transcription Blockage by DNA Damage in Nucleotide Excision Repair-Related Neurological Dysfunctions
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Impaired Repair of Ionizing Radiation-Induced DNA Damage in Cockayne Syndrome Cells
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CS proteins and ubiquitination: orchestrating DNA repair with transcription and cell division
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Self-citationTranscription-coupled repair of DNA–protein crosslinks
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- Authors
- Proietti‐De‐Santis, L., Drané, P., & Egly, J.
- Affiliations
- Centre National de la Recherche Scientifique (FR)
- Corresponding author
- Egly, Jean‐Marc
- Venue
- The EMBO Journal
- Published
- 2006-04-06
- Volume
- 25
- Issue
- 9
- Pages
- 1915-1923
- ISSN
- 0261-4189 (ISSN-L)
- 1460-2075
- PMC
- PMC1456931
- OpenAlex
- W2070933029
- Cited by
- 163
- References
- 33
- Citations by year
- 2026: 3 · 2025: 7 · 2024: 9 · 2023: 4 · 2022: 7 · 2021: 11 · 2020: 6 · 2019: 5 · 2018: 9 · 2017: 9 · 2016: 5 · 2015: 3 · 2014: 9 · 2013: 18 · 2012: 14
- FWCI
- 3.40
- Type
- journal article
- Language
- en
- Open access
- bronze
- doi.org
- Licence
- Locations
- The EMBO Journal (publishedVersion)
- Europe PMC (PubMed Central) (publishedVersion)
- PubMed Central (publishedVersion)
- PubMed (publishedVersion)
- HAL (submittedVersion)
- Funders
- Institut National de la Santé et de la Recherche Médicale
- Fondation pour la Recherche Médicale
- Ligue Contre le Cancer
- Topics
- Life Sciences › Biochemistry, Genetics and Molecular Biology › Molecular Biology › DNA Repair Mechanisms
- Life Sciences › Biochemistry, Genetics and Molecular Biology › Molecular Biology › Genomics and Chromatin Dynamics
- Life Sciences › Biochemistry, Genetics and Molecular Biology › Molecular Biology › Nuclear Structure and Function
- Keywords
- Cockayne syndrome, UV irradiation, transcription initiation, RNA polymerase II recruitment, histone H4 acetylation, DNA damage response, transcriptional programs, DNA repair deficiency
- MeSH terms
- Poly-ADP-Ribose Binding Proteins, Cockayne Syndrome, DNA, DNA Damage, DNA Repair, DNA Helicases, Gene Expression Regulation, Humans, Promoter Regions, Genetic, RNA Polymerase II, Tetrahydrofolate Dehydrogenase, Transcription, Genetic, Ultraviolet Rays, Tumor Suppressor Protein p53, Receptors, Calcitriol, DNA Repair Enzymes
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