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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
a) xeroderma pigmentosum complementation group F
b) XFE progeroid syndrome
Related
DNA excision repair protein ERCC1; Excision Repair Cross Complement-1 protein (ERCC1)
XP F-correcting protein gene
General
excision repair cross complement (ERCC) or excision repair cross-complementing rodent repair deficiency, complementation group
protein subunit
Xeroderma pigmentosum group complementing protein
Properties
SIZE: entity length = 916 aa
MW = 104 kD
COMPARTMENT: cell nucleus
MOTIF: leucine zipper
SITE: 233-254
FOR-BINDING-VIA: leucine zipper
leucine zipper
SITE: 270-298
FOR-BINDING-VIA: leucine zipper
peptide motif {486-491}
Ser phosphorylation site {S520}
Ser phosphorylation site {S521}
Ser phosphorylation site {S524}
EME1/ERCC1 {617-916}
Database Correlations
OMIM correlations
UniProt Q92889
Pfam PF02732
Entrez Gene 2072
Kegg hsa:2072
References
- Lehmann AR.
Nucleotide excision repair and the link with transcription.
Trends Biochem Sci. 1995 Oct;20(10):402-5. Review.
PMID: 8533152
- Entrez Gene :accession 2072
Component-of
ERCC1/XPF heterodimer