The Ubiquitin E3/E4 Ligase UBE4A Adjusts Protein Ubiquitylation and Accumulation at Sites of DNA Damage, Facilitating Double-Strand Break Repair

Mol Cell. 2018 Mar 1;69(5):866-878.e7. doi: 10.1016/j.molcel.2018.02.002.

Abstract

Double-strand breaks (DSBs) are critical DNA lesions that robustly activate the elaborate DNA damage response (DDR) network. We identified a critical player in DDR fine-tuning: the E3/E4 ubiquitin ligase UBE4A. UBE4A's recruitment to sites of DNA damage is dependent on primary E3 ligases in the DDR and promotes enhancement and sustainment of K48- and K63-linked ubiquitin chains at these sites. This step is required for timely recruitment of the RAP80 and BRCA1 proteins and proper organization of RAP80- and BRCA1-associated protein complexes at DSB sites. This pathway is essential for optimal end resection at DSBs, and its abrogation leads to upregulation of the highly mutagenic alternative end-joining repair at the expense of error-free homologous recombination repair. Our data uncover a critical regulatory level in the DSB response and underscore the importance of fine-tuning the complex DDR network for accurate and balanced execution of DSB repair.

Keywords: DNA damage; UBE4A; double-strand breaks; genome stability; ubiquitin.

Publication types

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

MeSH terms

  • BRCA1 Protein / genetics
  • BRCA1 Protein / metabolism*
  • Carrier Proteins / genetics
  • Carrier Proteins / metabolism*
  • DNA Breaks, Double-Stranded*
  • DNA-Binding Proteins
  • HeLa Cells
  • Histone Chaperones
  • Humans
  • Nuclear Proteins / genetics
  • Nuclear Proteins / metabolism*
  • Recombinational DNA Repair / physiology*
  • Ubiquitin-Protein Ligases / genetics
  • Ubiquitin-Protein Ligases / metabolism*
  • Ubiquitination / physiology*
  • Ubiquitins / genetics
  • Ubiquitins / metabolism

Substances

  • BRCA1 Protein
  • BRCA1 protein, human
  • Carrier Proteins
  • DNA-Binding Proteins
  • Histone Chaperones
  • Nuclear Proteins
  • UIMC1 protein, human
  • Ubiquitins
  • UBE4A protein, human
  • Ubiquitin-Protein Ligases