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DNA polymerase ι compensates for Fanconi anemia pathway deficiency by countering DNA replication stress

Rui Wang, Walter F. Lenoir, Chao Wang, Dan Su, Megan McLaughlin, Qianghua Hu, Xi Shen, Yanyan Tian, Naeh L. Klages-Mundt, Erica J. Lynn, Richard D. Wood, Junjie Chen, Traver Hart, Lei Li

2020Proceedings of the National Academy of Sciences19 citationsDOIOpen Access PDF

Abstract

Significance Pol ι is a highly conserved Y-family DNA polymerase. Disruptions of the Pol ι gene in cellular and mouse models, however, have so far failed to produce any detectable phenotype. Using unbiased whole genome fitness screens, we find that Pol ι is essential for the genome stability and survival of Fanconi anemia mutant cells. This finding reveals the physiological role for Pol ι as a compensatory factor of Fanconi anemia deficiency. It implies a novel genetic connection between lesion bypass and replication stress response. Furthermore, the identified synthetic lethality between Pol ι and the Fanconi anemia mechanism suggests an enzymatic and therapeutic target for eliminating tumor cells arising from the Fanconi anemia pathway defect.

Topics & Concepts

DNA replicationBiologyGenome instabilityFanconi anemiaDNA polymeraseDNA damageDNA repairDNA synthesisCell biologyGeneticsMolecular biologyGeneDNADNA Repair MechanismsCRISPR and Genetic EngineeringCarcinogens and Genotoxicity Assessment
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