DNA repair endonuclease XPF; DNA excision repair protein ERCC-4; DNA repair protein complementing XP-F cells; Xeroderma pigmentosum group F-complementing protein (ERCC4, ERCC11, XPF)

Function

• structure-specific DNA repair endonuclease
• responsible for 5-prime incision during DNA repair
• role in homologous recombination that assists in removing interstrand cross-link
• heterodimer composed of ERCC1 & XPF/ERCC4
• interacts with EME1

Cofactor: Mg+2

Structure

belongs to the XPF family

Compartment

nucleus (probable)

Pathology

• defects in ERCC4 are the cause of
  • xeroderma pigmentosum complementation group F
  • XFE progeroid syndrome

More general terms

• protein subunit
• excision repair cross complement
• Xeroderma pigmentosum group complementing protein

Additional terms

• DNA excision repair protein ERCC1; Excision Repair Cross Complement-1 protein (ERCC1)

Component of

• ERCC1/XPF heterodimer

Database

• UniProt: http://www.uniprot.org/uniprot/Q92889.html
• Entrez gene: http://www.ncbi.nlm.nih.gov/sites/entrez?db=gene&cmd=Retrieve&dopt=Graphics&list_uids=2072
• Kegg: http://www.genome.jp/dbget-bin/www_bget?hsa:2072
• OMIM: https://mirror.omim.org/entry/133520
• OMIM: https://mirror.omim.org/entry/278760
• OMIM: https://mirror.omim.org/entry/610965