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The COP9 signalosome is vital for timely repair of DNA double-strand breaks.


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Authors

Meir, Michal 
Kashani, Lior 
Blank, Michael 
Khosravi, Rami 

Abstract

The DNA damage response is vigorously activated by DNA double-strand breaks (DSBs). The chief mobilizer of the DSB response is the ATM protein kinase. We discovered that the COP9 signalosome (CSN) is a crucial player in the DSB response and an ATM target. CSN is a protein complex that regulates the activity of cullin ring ubiquitin ligase (CRL) complexes by removing the ubiquitin-like protein, NEDD8, from their cullin scaffold. We find that the CSN is physically recruited to DSB sites in a neddylation-dependent manner, and is required for timely repair of DSBs, affecting the balance between the two major DSB repair pathways-nonhomologous end-joining and homologous recombination repair (HRR). The CSN is essential for the processivity of deep end-resection-the initial step in HRR. Cullin 4a (CUL4A) is recruited to DSB sites in a CSN- and neddylation-dependent manner, suggesting that CSN partners with CRL4 in this pathway. Furthermore, we found that ATM-mediated phosphorylation of CSN subunit 3 on S410 is critical for proper DSB repair, and that loss of this phosphorylation site alone is sufficient to cause a DDR deficiency phenotype in the mouse. This novel branch of the DSB response thus significantly affects genome stability.

Description

Keywords

Animals, Ataxia Telangiectasia Mutated Proteins, COP9 Signalosome Complex, Cell Line, Cells, Cultured, Cullin Proteins, DNA Breaks, Double-Stranded, DNA Repair, Humans, Mice, Multiprotein Complexes, Nuclear Proteins, Peptide Hydrolases, Protein Kinases

Journal Title

Nucleic Acids Res

Conference Name

Journal ISSN

0305-1048
1362-4962

Volume Title

43

Publisher

Oxford University Press (OUP)
Sponsorship
Dr. Miriam and Sheldon G. Adelson Medical Research Foundation; Israel Cancer Research Fund; Israel Science Foundation (grants no. 1900/12 and 998/14); The I-CORE Program of the Planning and Budgeting Committee of the Israel Ministry of Education, to YS laboratory; Spanish Ministry of Economy and Competitivity ( SAF2013-43255-P) and ERC Starting Grant (DSBRECA), to PH laboratory; ERC Starting Grant (NEDD8andCRLs) to TK laboratory; Consejo Nacional de Ciencia y Tecnología (CONACYT), Mexico, to A.C.-G. YS is a Research Professor of the Israel Cancer Research Fund. Funding for open access charge: Donation to our lab.