Function and Interactions of ERCC1-XPF in DNA Damage Response

Molecules. 2018 Dec 5;23(12):3205. doi: 10.3390/molecules23123205.


Numerous proteins are involved in the multiple pathways of the DNA damage response network and play a key role to protect the genome from the wide variety of damages that can occur to DNA. An example of this is the structure-specific endonuclease ERCC1-XPF. This heterodimeric complex is in particular involved in nucleotide excision repair (NER), but also in double strand break repair and interstrand cross-link repair pathways. Here we review the function of ERCC1-XPF in various DNA repair pathways and discuss human disorders associated with ERCC1-XPF deficiency. We also overview our molecular and structural understanding of XPF-ERCC1.

Keywords: DNA damage response; DSB repair; ERCC1; Fanconi anemia; ICL repair; NER; XPF.

Publication types

  • Review

MeSH terms

  • Amino Acid Sequence
  • Animals
  • DNA Damage*
  • DNA End-Joining Repair
  • DNA Repair
  • DNA-Binding Proteins / chemistry
  • DNA-Binding Proteins / metabolism*
  • Endonucleases / chemistry
  • Endonucleases / metabolism*
  • Helix-Loop-Helix Motifs
  • Humans
  • Models, Molecular
  • Protein Binding
  • Protein Conformation
  • Protein Interaction Domains and Motifs
  • Signal Transduction


  • DNA-Binding Proteins
  • xeroderma pigmentosum group F protein
  • ERCC1 protein, human
  • Endonucleases