A Ubiquitin-Binding Domain in Cockayne Syndrome B Required for Transcription-Coupled Nucleotide Excision Repair

• DOI: 10.1016/j.molcel.2010.04.017
• Publication Date: 2010-06-11T10:52:46Z
• Authors: Roy Anindya; Pierre-Olivier Mari; Ulrik Kristensen; Hanneke Kool; Giuseppina Giglia...; Leon H. Mullenders; Maria Fousteri; Wim Vermeulen; Jean-Marc Egly; Jesper Q. Svejstrup
• Keywords: Cockayne syndrome; Transcription

Transcription-coupled nucleotide excision repair (TC-NER) allows RNA polymerase II (RNAPII)-blocking lesions to be rapidly removed from the transcribed strand of active genes. Defective TCR in humans is associated with Cockayne syndrome (CS), typically caused by defects either CSA or CSB. Here, we show that CSB contains a ubiquitin-binding domain (UBD). Cells expressing UBD-less (CSB(del)) have phenotypes similar those cells lacking CSB, but these can suppressed appending heterologous UBD, so ubiquitin binding essential for function. Surprisingly, CSB(del) remains capable assembling factors and synthesis proteins around damage-stalled RNAPII, such complexes fail excise lesion. Together, our results indicate an role protein ubiquitylation CSB's UBD triggering damage incision during TC-NER allow us integrate function model process.