Author:
Groisman Regina,Kuraoka Isao,Chevallier Odile,Gaye Nogaye,Magnaldo Thierry,Tanaka Kiyoji,Kisselev Alexei F.,Harel-Bellan Annick,Nakatani Yoshihiro
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.
Publisher
Cold Spring Harbor Laboratory
Subject
Developmental Biology,Genetics
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