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PMID: 16751180 Published · ppublish English Journal Article Research Support, Non-U.S. Gov't

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

Genes & development ·Vol. 20 ·No. 11 ·2006-06-01 ·Pages 1429-34

Groisman R, Kuraoka I, Chevallier O, Gaye N, Magnaldo T, Tanaka K, Kisselev AF, Harel-Bellan A, Nakatani Y

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.

MeSH Terms
Cockayne Syndrome/genetics DNA Helicases/genetics,metabolism DNA Repair Enzymes/genetics,metabolism Genetic Complementation Test HeLa Cells Humans Poly-ADP-Ribose Binding Proteins Proteasome Endopeptidase Complex/metabolism Transcription Factors/genetics,metabolism Ubiquitin/metabolism
Chemicals
ERCC8 protein, human Poly-ADP-Ribose Binding Proteins Transcription Factors Ubiquitin Proteasome Endopeptidase Complex DNA Helicases ERCC6 protein, human DNA Repair Enzymes
Authors & Affiliations
9 authors, click to expand affiliations / ORCID
Groisman Regina
Dana-Farber Cancer Institute, Harvard Medical School, Boston, Massachusetts 02115, USA. [email protected]
Kuraoka Isao
Chevallier Odile
Gaye Nogaye
Magnaldo Thierry
Tanaka Kiyoji
Kisselev Alexei F
Harel-Bellan Annick
Nakatani Yoshihiro
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26 references, click to expand
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Article Info
Journal
Genes & development
Abbr.
Genes Dev
ISSN
0890-9369
DOI
10.1101/gad.378206
Email
[email protected]
Published
2006-06-01
Pages
1429-34
Language
English
Region
United States
NLM ID
8711660
PMCID
PMC1475755
Subset
IM
External Links
PubMed source DOI: 10.1101/gad.378206 PMC Full Text View journal details
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