FANCD2-Associated Nuclease 1 Partially Compensates for the Lack of Exonuclease 1 in Mismatch Repair

Author:

Kratz Katja1,Artola-Borán Mariela1,Kobayashi-Era Saho12,Koh Gene345,Oliveira Goncalo6,Kobayashi Shunsuke12,Oliveira Andreia12,Zou Xueqing345,Richter Julia2,Tsuda Masataka7,Sasanuma Hiroyuki7,Takeda Shunichi7,Loizou Joanna I.6,Sartori Alessandro A.1,Nik-Zainal Serena45,Jiricny Josef12ORCID

Affiliation:

1. Institute of Molecular Cancer Research, University of Zurich, Zurich, Switzerland

2. Institute of Biochemistry of the ETH Zurich, Zurich, Switzerland

3. Wellcome Sanger Institute, Wellcome Genome Campus, Hinxton, United Kingdom

4. Academic Department of Medical Genetics, The Clinical School, University of Cambridge, Cambridge, United Kingdom

5. MRC Cancer Unit, The Clinical School, University of Cambridge, Cambridge, United Kingdom

6. Institute of Cancer Research, Department of Medicine I, Comprehensive Cancer Centre, Medical University of Vienna, Vienna, Austria

7. Department of Radiation Genetics, Graduate School of Medicine, Kyoto University, Kyoto, Japan

Abstract

Germline mutations in the mismatch repair (MMR) genes MSH2 , MSH6 , MLH1 , and PMS2 are linked to cancer of the colon and other organs, characterized by microsatellite instability and a large increase in mutation frequency. Unexpectedly, mutations in EXO1 , encoding the only exonuclease genetically implicated in MMR, are not linked to familial cancer and cause a substantially weaker mutator phenotype.

Publisher

American Society for Microbiology

Subject

Cell Biology,Molecular Biology

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