Gene Symbol: Ercc4
Description: ERCC excision repair 4, endonuclease catalytic subunit
Alias: RGD1560340, DNA repair endonuclease XPF, excision repair cross-complementing rodent repair deficiency, complementation group 4, Ercc4 protein-like, excision repair cross-complementation group 4
Species: rat
Products:     Ercc4

Top Publications

  1. Allione A, Guarrera S, Russo A, Ricceri F, Purohit R, Pagnani A, et al. Inter-individual variation in nucleotide excision repair pathway is modulated by non-synonymous polymorphisms in ERCC4 and MBD4 genes. Mutat Res. 2013;751-752:49-54 pubmed publisher
    ..89 ± 5.20). Moreover, DRC was significantly lower in individuals carrying a variant allele for the ERCC4 rs1800124 non-synonymous SNP (nsSNP) (p = 0...
  2. Chen Z, Xu X, Harrison J, Wang G. Defining the function of xeroderma pigmentosum group F protein in psoralen interstrand cross-link-mediated DNA repair and mutagenesis. Biochem J. 2004;379:71-8 pubmed
    ..These results suggest that the XPF protein has important roles in the psoralen ICL-mediated DNA repair and mutagenesis. ..
  3. McCutchen Maloney S, Giannecchini C, Hwang M, Thelen M. Domain mapping of the DNA binding, endonuclease, and ERCC1 binding properties of the human DNA repair protein XPF. Biochemistry. 1999;38:9417-25 pubmed
    ..A working model for the XPF protein is presented illustrating the function of XPF in the nucleotide excision pathway and depicting the two functional domains interacting with DNA and ERCC1. ..
  4. Liu H, Yang S, Sun F. 1-Methyl-4-phenyl-pyridinium time-dependently alters expressions of oxoguanine glycosylase 1 and xeroderma pigmentosum group F protein in PC12 cells. Neurosci Bull. 2010;26:1-7 pubmed publisher
    ..Also, this study indicates that the base and nucleotide excision repair pathways may be compensatory activated in the early stage of pathogenesis in the cells after MPP(+) treatment. ..
  5. Motycka T, Bessho T, Post S, Sung P, Tomkinson A. Physical and functional interaction between the XPF/ERCC1 endonuclease and hRad52. J Biol Chem. 2004;279:13634-9 pubmed
    ..We propose that the ternary complex of hRad52 and XPF/ERCC1 is the active species that processes recombination intermediates generated during the repair of DNA double strand breaks and in homology-dependent gene targeting events. ..
  6. Shannon M, Lamerdin J, Richardson L, McCutchen Maloney S, Hwang M, Handel M, et al. Characterization of the mouse Xpf DNA repair gene and differential expression during spermatogenesis. Genomics. 1999;62:427-35 pubmed
    ..These results support a distinct role for the XPF/ERCC1 junction-specific endonuclease during meiosis, most likely in the resolution of heteroduplex intermediates that arise during recombination. ..
  7. Wang C, George B, Chen S, Feng B, Li X, Chakrabarti S. Genotoxic stress and activation of novel DNA repair enzymes in human endothelial cells and in the retinas and kidneys of streptozotocin diabetic rats. Diabetes Metab Res Rev. 2012;28:329-37 pubmed publisher
    Mammalian excision repair cross-complementing 1 (ERCC1) and ERCC4 (a.k.a xeroderma pigmentosum complementation group F) are nucleotide excision repair enzymes, which excise the 5' end of damaged DNA...
  8. de Laat W, Sijbers A, Odijk H, Jaspers N, Hoeijmakers J. Mapping of interaction domains between human repair proteins ERCC1 and XPF. Nucleic Acids Res. 1998;26:4146-52 pubmed
    ..ERCC1-XPF complex formation is established by a direct interaction between these two binding domains. A mutation from an XP-F patient that alters the ERCC1-binding domain in XPF indeed affects complex formation with ERCC1. ..
  9. Kamileri I, Karakasilioti I, Garinis G. Nucleotide excision repair: new tricks with old bricks. Trends Genet. 2012;28:566-73 pubmed publisher
    ..Together, these findings add new pieces to the puzzle for understanding NER and the relevance of NER defects in development and disease. ..

More Information


  1. Das D, Folkers G, van Dijk M, Jaspers N, Hoeijmakers J, Kaptein R, et al. The structure of the XPF-ssDNA complex underscores the distinct roles of the XPF and ERCC1 helix- hairpin-helix domains in ss/ds DNA recognition. Structure. 2012;20:667-75 pubmed publisher
    ..Combining the two observations gives a model that underscores the asymmetry of the human XPF/ERCC1 heterodimer in binding at an ss/ds DNA junction. ..
  2. McWilliams R, Bamlet W, Cunningham J, Goode E, de Andrade M, Boardman L, et al. Polymorphisms in DNA repair genes, smoking, and pancreatic adenocarcinoma risk. Cancer Res. 2008;68:4928-35 pubmed publisher
    ..Allele and genotype frequencies for 16 SNPs in DNA repair genes ERCC1, XPD/ERCC2, XPC, XPF/ERCC4, OGG1, and XRCC1 were compared after adjusting for age, sex, and smoking history...
  3. Wu Y, Zacal N, Rainbow A, Zhu X. XPF with mutations in its conserved nuclease domain is defective in DNA repair but functions in TRF2-mediated telomere shortening. DNA Repair (Amst). 2007;6:157-66 pubmed
    ..Furthermore, our findings reveal an unanticipated nuclease-independent function of XPF in TRF2-mediated telomere shortening. ..
  4. Sijbers A, de Laat W, Ariza R, Biggerstaff M, Wei Y, Moggs J, et al. Xeroderma pigmentosum group F caused by a defect in a structure-specific DNA repair endonuclease. Cell. 1996;86:811-22 pubmed
    ..These results demonstrate that the XPF, ERCC4, and ERCC11 genes are equivalent, complete the isolation of the XP genes that form the core nucleotide excision ..
  5. Tian M, Shinkura R, Shinkura N, Alt F. Growth retardation, early death, and DNA repair defects in mice deficient for the nucleotide excision repair enzyme XPF. Mol Cell Biol. 2004;24:1200-5 pubmed
    ..These phenotypes are identical to those exhibited by the ERCC1-deficient mice, consistent with the functional association of the two proteins. The complex phenotype suggests that XPF-ERCC1 is involved in multiple DNA repair processes. ..
  6. Sridharan D, Brown M, Lambert W, McMahon L, Lambert M. Nonerythroid alphaII spectrin is required for recruitment of FANCA and XPF to nuclear foci induced by DNA interstrand cross-links. J Cell Sci. 2003;116:823-35 pubmed
    ..These studies suggest that an important role for alphaSpIISigma* in the nucleus is to act as a scaffold, aiding in recruitment and alignment of repair proteins at sites of damage. ..
  7. Mogi S, Oh D. gamma-H2AX formation in response to interstrand crosslinks requires XPF in human cells. DNA Repair (Amst). 2006;5:731-40 pubmed
    ..Furthermore, XPA may be important to allow psoralen interstrand crosslinks to be processed without forming a double strand break intermediate. ..
  8. Sakaguchi K, Ishibashi T, Uchiyama Y, Iwabata K. The multi-replication protein A (RPA) system--a new perspective. FEBS J. 2009;276:943-63 pubmed publisher
    ..Convergent evolution seems to be ubiquitous in these processes. Using recent findings, we review the composition and biological functions of RPA complexes. ..
  9. Zhu X, Niedernhofer L, Kuster B, Mann M, Hoeijmakers J, de Lange T. ERCC1/XPF removes the 3' overhang from uncapped telomeres and represses formation of telomeric DNA-containing double minute chromosomes. Mol Cell. 2003;12:1489-98 pubmed
  10. Kumaresan K, Hwang M, Thelen M, Lambert M. Contribution of XPF functional domains to the 5' and 3' incisions produced at the site of a psoralen interstrand cross-link. Biochemistry. 2002;41:890-6 pubmed
  11. Wu Y, Mitchell T, Zhu X. Human XPF controls TRF2 and telomere length maintenance through distinctive mechanisms. Mech Ageing Dev. 2008;129:602-10 pubmed publisher
    ..Our results further imply that TRF2 association with telomeres may be deregulated in cells derived from XPF patients. ..