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.

journal_name

Genes Dev

journal_title

Genes & development

authors

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

doi

10.1101/gad.378206

subject

Has Abstract

pub_date

2006-06-01 00:00:00

pages

1429-34

issue

11

eissn

0890-9369

issn

1549-5477

pii

20/11/1429

journal_volume

20

pub_type

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