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

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.
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https://www.hal.inserm.fr/inserm-00091583
Contributor : Annick Harel-Bellan <>
Submitted on : Wednesday, September 6, 2006 - 4:44:37 PM
Last modification on : Thursday, January 11, 2018 - 6:20:36 AM

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Regina Groisman, Isao Kuraoka, Odile Chevallier, Nogaye Gaye, Thierry Magnaldo, et al.. CSA-dependent degradation of CSB by the ubiquitin-proteasome pathway establishes a link between complementation factors of the Cockayne syndrome.. Genes and Development, Cold Spring Harbor Laboratory Press, 2006, 20, pp.1429-34. ⟨10.1101/gad.378206⟩. ⟨inserm-00091583⟩

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