FAM35A associates with REV7 and modulates DNA damage responses of normal and BRCA1-defective cells

EMBO J. 2018 Jun 15;37(12):e99543. doi: 10.15252/embj.201899543. Epub 2018 May 22.

Abstract

To exploit vulnerabilities of tumors, it is urgent to identify associated defects in genome maintenance. One unsolved problem is the mechanism of regulation of DNA double-strand break repair by REV7 in complex with 53BP1 and RIF1, and its influence on repair pathway choice between homologous recombination and non-homologous end-joining. We searched for REV7-associated factors in human cells and found FAM35A, a previously unstudied protein with an unstructured N-terminal region and a C-terminal region harboring three OB-fold domains similar to single-stranded DNA-binding protein RPA, as novel interactor of REV7/RIF1/53BP1. FAM35A re-localized in damaged cell nuclei, and its knockdown caused sensitivity to DNA-damaging agents. In a BRCA1-mutant cell line, however, depletion of FAM35A increased resistance to camptothecin, suggesting that FAM35A participates in processing of DNA ends to allow more efficient DNA repair. We found FAM35A absent in one widely used BRCA1-mutant cancer cell line (HCC1937) with anomalous resistance to PARP inhibitors. A survey of FAM35A alterations revealed that the gene is altered at the highest frequency in prostate cancers (up to 13%) and significantly less expressed in metastatic cases, revealing promise for FAM35A as a therapeutically relevant cancer marker.

Keywords: DNA repair; camptothecin; cisplatin; olaparib; prostate cancer.

Publication types

  • Research Support, N.I.H., Extramural
  • Research Support, Non-U.S. Gov't

MeSH terms

  • BRCA1 Protein / deficiency*
  • Biomarkers, Tumor / genetics
  • Biomarkers, Tumor / metabolism*
  • Cell Cycle Proteins
  • Cell Line, Tumor
  • DNA Damage*
  • DNA Repair*
  • DNA, Neoplasm / genetics
  • DNA, Neoplasm / metabolism*
  • DNA-Binding Proteins
  • HEK293 Cells
  • Humans
  • Mad2 Proteins / genetics
  • Mad2 Proteins / metabolism*
  • Mutation
  • Neoplasms / genetics
  • Neoplasms / metabolism*
  • Neoplasms / pathology
  • Proteins / genetics
  • Proteins / metabolism*
  • Telomere-Binding Proteins / genetics
  • Telomere-Binding Proteins / metabolism
  • Tumor Suppressor p53-Binding Protein 1 / genetics
  • Tumor Suppressor p53-Binding Protein 1 / metabolism

Substances

  • BRCA1 Protein
  • BRCA1 protein, human
  • Biomarkers, Tumor
  • Cell Cycle Proteins
  • DNA, Neoplasm
  • DNA-Binding Proteins
  • MAD2L2 protein, human
  • Mad2 Proteins
  • Proteins
  • Rif1 protein, human
  • SHLD2 protein, human
  • TP53BP1 protein, human
  • Telomere-Binding Proteins
  • Tumor Suppressor p53-Binding Protein 1