PreprintCited by 268DNA Repair MechanismsUbiquitin and proteasome pathwaysNuclear Structure and Function

CSA-dependent degradation of CSB by the ubiquitin-proteasome pathway establishes a link between complementation factors of the Cockayne syndrome.

Open access
Groisman, Regina, Kuraoka, Isao, Chevallier, Odile, Gaye, Nogaye, Magnaldo, Thierry, Tanaka, Kiyoji, Kisselev, Alexei F., Harel-Bellan, Annick +1 more·2006·Genes & Development

Abstract

Mutations in the CSA or CSB complementation genes cause the Cockayne syndrome, a severe genetic disorder that results in patients' death in early adulthood. CSA and CSB act in a transcription-coupled repair (TCR) pathway, but their functional relationship is not understood. We have previously shown that CSA is a subunit of an E3 ubiquitin ligase complex. Here we demonstrate that CSB is a substrate of this ligase: Following UV irradiation, CSB is degraded at a late stage of the repair process in a proteasome- and CSA-dependent manner. Moreover, we demonstrate the importance of CSB degradation for post-TCR recovery of transcription and for the Cockayne syndrome. Our results unravel for the first time the functional relationship between CSA and CSB.

The claims and statistics of this paper haven’t been checked yet.

Run the check — free, no account needed. It reads the open-access text and checks the sources it cites.

A few free checks a day · papers already verified open instantly · results are public

Integrity signals

Registry

Crossref · 10.1101/gad.378206

Cites

27 of 27 references verified0 citation errors · 0 not checkable

Statistics

Claims the paper makes

The claims and statistics of this paper haven’t been checked yet.

Run the check — free, no account needed. It reads the open-access text and checks the sources it cites.

A few free checks a day · papers already verified open instantly · results are public

Claims other papers make about this one

Each is checked against this paper’s own text: does it say what the citing paper says it does?

  • ERCC6 degradation following UV irradiation is more rapid in the absence of USP7 or UVSSA.

    “Furthermore, while ERCC6 is degraded in cells following UV irradiation (Groisman et al., 2006; Wei et al., 2011), this degradation is more rapid in the absence of either protein.”

    From USP7 Is a Master Regulator of Genome Stability (2020) · cites this paper as Groisman et al., 2006

Self-check

Percentages, sums and reported differences re-computed from the paper’s own numbers.

The self-check needs the paper’s full text.

Cites

Flagged first, then by citations

Research works printed but not linked: 1

  1. Bootsma, D., Kreamer, K.H., Cleaver, J.E., and Hoeijmakers, J.H. 1998. Nucleotide excision repair syndromes: Xeroderma pigmentosum, Cockayne syndrome, and trichothiodystrophy. In The gentetic basis of human cancer (eds. B. Vogelsteain and K.W. Kinzler), pp. 254-257.

Cited by

Sorted by citations

Authors
Groisman, R., Kuraoka, I., Chevallier, O., Gaye, N., Magnaldo, T., Tanaka, K., Kisselev, A. F., Harel-Bellan, A., & Nakatani, Y.
Affiliations
  • Centre National de la Recherche Scientifique (FR)
  • Japan Science and Technology Agency (JP)
  • Dartmouth College (US)
  • Dana-Farber Cancer Institute (US)
Venue
Genes & Development
Published
2006-06-01
Volume
20
Issue
11
Pages
1429-1434
ISSN
  • 0890-9369 (ISSN-L)
  • 1549-5477
OpenAlex
W2143989418
Cited by
268
References
27
Citations by year
2026: 6 · 2025: 13 · 2024: 15 · 2023: 16 · 2022: 10 · 2021: 18 · 2020: 10 · 2019: 14 · 2018: 10 · 2017: 10 · 2016: 14 · 2015: 16 · 2014: 9 · 2013: 12 · 2012: 9
Type
posted content
Language
en
Open access
Locations
  • Genes & Development (publishedVersion)
  • Europe PMC (PubMed Central) (publishedVersion)
  • PubMed (publishedVersion)
  • HAL (submittedVersion)
Funders
Leukemia and Lymphoma Society
Topics
  • Life Sciences › Biochemistry, Genetics and Molecular Biology › Molecular Biology › DNA Repair Mechanisms
  • Life Sciences › Biochemistry, Genetics and Molecular Biology › Molecular Biology › Ubiquitin and proteasome pathways
  • Life Sciences › Biochemistry, Genetics and Molecular Biology › Molecular Biology › Nuclear Structure and Function
Keywords
Cockayne syndrome, CSA, CSB, ubiquitin-proteasome system, E3 ubiquitin ligase, transcription-coupled repair, protein degradation, UV-induced DNA damage, DNA repair
MeSH terms
Poly-ADP-Ribose Binding Proteins, Cockayne Syndrome, DNA Helicases, Genetic Complementation Test, HeLa Cells, Humans, Transcription Factors, Ubiquitin, DNA Repair Enzymes, Proteasome Endopeptidase Complex

About these checks

Registry, retraction, Cites and statistics results are computed, not judged, with one exception: a printed reference that no index lists is read once by a model before it counts as a citation error. Claims are extracted by a model and kept only when their quote appears verbatim in the text; each cited source is then checked, with a model’s help, for whether it says what the claim relies on. Those verdicts can be wrong — read the claim’s quote and the cited source before relying on one.

Statistics and arithmetic are recomputed, not judged.

Confidence bands are shown only for fields calibrated on our annotated test set. None is calibrated yet, so no band is shown.

What counts as a claim ›How we measure confidence ›How we check this ›

Are you an author, or do you see a problem with this page? Request a review