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. 2012 May;44(5):598-602.
doi: 10.1038/ng.2230.

UV-sensitive syndrome protein UVSSA recruits USP7 to regulate transcription-coupled repair

Petra Schwertman  1 , Anna Lagarou, Dick H W Dekkers, Anja Raams, Adriana C van der Hoek, Charlie Laffeber, Jan H J Hoeijmakers, Jeroen A A Demmers, Maria Fousteri, Wim Vermeulen, Jurgen A Marteijn
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UV-sensitive syndrome protein UVSSA recruits USP7 to regulate transcription-coupled repair

Petra Schwertman et al. Nat Genet. 2012 May.

Abstract

Transcription-coupled nucleotide-excision repair (TC-NER) is a subpathway of NER that efficiently removes the highly toxic RNA polymerase II blocking lesions in DNA. Defective TC-NER gives rise to the human disorders Cockayne syndrome and UV-sensitive syndrome (UV(S)S). NER initiating factors are known to be regulated by ubiquitination. Using a SILAC-based proteomic approach, we identified UVSSA (formerly known as KIAA1530) as part of a UV-induced ubiquitinated protein complex. Knockdown of UVSSA resulted in TC-NER deficiency. UVSSA was found to be the causative gene for UV(S)S, an unresolved NER deficiency disorder. The UVSSA protein interacts with elongating RNA polymerase II, localizes specifically to UV-induced lesions, resides in chromatin-associated TC-NER complexes and is implicated in stabilizing the TC-NER master organizing protein ERCC6 (also known as CSB) by delivering the deubiquitinating enzyme USP7 to TC-NER complexes. Together, these findings indicate that UVSSA-USP7–mediated stabilization of ERCC6 represents a critical regulatory mechanism of TC-NER in restoring gene expression.

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