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Gene Review

ERCC1  -  excision repair cross-complementation group 1

Homo sapiens

Synonyms: COFS4, DNA excision repair protein ERCC-1, RAD10, UV20
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Disease relevance of ERCC1


High impact information on ERCC1

  • The XPF protein was purified from mammalian cells in a tight complex with ERCC1 [6].
  • Human cell extracts were fractionated to locate active components, including xeroderma pigmentosum (XP) and ERCC factors [7].
  • Mice with DNA repair gene (ERCC-1) deficiency have elevated levels of p53, liver nuclear abnormalities and die before weaning [8].
  • Mice with defective DNA repair were generated by targeting the excision repair cross complementing gene (ERCC-1) in the embryonic stem cell line, HM-1 [8].
  • Homozygous ERCC-1 mutants were runted at birth and died before weaning with liver failure [8].

Chemical compound and disease context of ERCC1


Biological context of ERCC1

  • Action of DNA repair endonuclease ERCC1/XPF in living cells [1].
  • Ultraviolet light-induced DNA damage caused a transient dose-dependent immobilization of ERCC1/XPF, likely due to engagement of the complex in a single repair event [1].
  • We speculate that TDMs are formed through the recombination of telomeres with interstitial telomere-related sequences and that ERCC1/XPF functions to repress this process [14].
  • Finally, the phosphorylation of XPB-S751 does not impair the TFIIH unwinding of the DNA around the lesion, but rather prevents the 5' incision triggered by the ERCC1-XPF endonuclease [15].
  • A long-range restriction map of the human chromosome 19q13 region: close physical linkage between CKMM and the ERCC1 and ERCC2 genes [16].

Anatomical context of ERCC1


Associations of ERCC1 with chemical compounds

  • Possible markers of platinum chemotherapy association [glutathione S-transferase pi (GSTP1) and excision cross-complementing gene 1 (ERCC1)] and 5-fluorouracil association [thymidylate synthase 1 (TS1)] were measured [5].
  • However, the effects of RAD52 2259 CT or TT and ERCC1 354 CT or TT genotypes were more evident for the estrogen/progesterone receptor-negative cases (OR, 2.03; 95% CI, 1.24-3.34 and OR, 1.99; 95% CI, 1.35-2.94, respectively) [4].
  • Among the subunits, the expression of ERCC1 in WI38 cells was up-regulated significantly after emodin treatment [22].
  • To confirm the importance of the ERCC1 gene for cellular resistance to 4HC and PM, UV20 cells were transfected with the human ERCC1 gene and subsequently exposed to 4HC and PM [23].
  • Inhibition of transcription by DRB abolished the radiation-induced increase of ERCC1 and XRCC1 proteins [24].

Physical interactions of ERCC1

  • The XPA-bound fraction complemented cell-free extracts of excision repair cross-complementing 1 (ERCC-1), ERCC-4 (XP-F), and XP-A mutants [25].
  • XPG binding stabilizes the NER preincision complex and is essential for the 5' incision by the ERCC1/XPF endonuclease [26].
  • Our results provide the first insights into how HMGA2 and its aberrant forms bind and regulate the ERCC1 promoter [27].
  • High mobility group A2 protein and its derivatives bind a specific region of the promoter of DNA repair gene ERCC1 and modulate its activity [27].

Enzymatic interactions of ERCC1

  • In mammalian cells XPG cleaves 3' of the DNA lesion while the ERCC1-XPF complex makes the 5' incision [28].

Regulatory relationships of ERCC1

  • Although XP-F cells are sensitive to UV and mitomycin C (MMC), cells overexpressing XPF expressed ERCC1 as well and resistance to UV and MMC was restored to the normal level [29].
  • Furthermore, the regulatory effect of HMGA2 was confirmed by luciferase promoter assays showing that ERCC1 promoter activity is down-regulated by all investigated HMGA2 forms, with the most striking effect exerted by DeltaHMGA2 [27].
  • Among molecular explanatory factors that were examined, the combination gefitinib-radiation therapy totally abolishes DNA-dependent protein kinase expression, and gefitinib attenuates the radiation therapy-induced enhancement of ERCC1 and augments the VTKI-induced CD95 enhancement [30].
  • IL-1 alpha inhibited cisplatin induction of ERCC-1 mRNA levels in our system [31].

Other interactions of ERCC1


Analytical, diagnostic and therapeutic context of ERCC1


  1. Action of DNA repair endonuclease ERCC1/XPF in living cells. Houtsmuller, A.B., Rademakers, S., Nigg, A.L., Hoogstraten, D., Hoeijmakers, J.H., Vermeulen, W. Science (1999) [Pubmed]
  2. Reconstitution of human excision nuclease with recombinant XPF-ERCC1 complex. Bessho, T., Sancar, A., Thompson, L.H., Thelen, M.P. J. Biol. Chem. (1997) [Pubmed]
  3. Mapping of interaction domains between human repair proteins ERCC1 and XPF. de Laat, W.L., Sijbers, A.M., Odijk, H., Jaspers, N.G., Hoeijmakers, J.H. Nucleic Acids Res. (1998) [Pubmed]
  4. Genetic polymorphisms of selected DNA repair genes, estrogen and progesterone receptor status, and breast cancer risk. Lee, K.M., Choi, J.Y., Kang, C., Kang, C.P., Park, S.K., Cho, H., Cho, D.Y., Yoo, K.Y., Noh, D.Y., Ahn, S.H., Park, C.G., Wei, Q., Kang, D. Clin. Cancer Res. (2005) [Pubmed]
  5. High gene expression of TS1, GSTP1, and ERCC1 are risk factors for survival in patients treated with trimodality therapy for esophageal cancer. Joshi, M.B., Shirota, Y., Danenberg, K.D., Conlon, D.H., Salonga, D.S., Herndon, J.E., Danenberg, P.V., Harpole, D.H. Clin. Cancer Res. (2005) [Pubmed]
  6. Xeroderma pigmentosum group F caused by a defect in a structure-specific DNA repair endonuclease. Sijbers, A.M., de Laat, W.L., Ariza, R.R., Biggerstaff, M., Wei, Y.F., Moggs, J.G., Carter, K.C., Shell, B.K., Evans, E., de Jong, M.C., Rademakers, S., de Rooij, J., Jaspers, N.G., Hoeijmakers, J.H., Wood, R.D. Cell (1996) [Pubmed]
  7. Mammalian DNA nucleotide excision repair reconstituted with purified protein components. Aboussekhra, A., Biggerstaff, M., Shivji, M.K., Vilpo, J.A., Moncollin, V., Podust, V.N., Protić, M., Hübscher, U., Egly, J.M., Wood, R.D. Cell (1995) [Pubmed]
  8. Mice with DNA repair gene (ERCC-1) deficiency have elevated levels of p53, liver nuclear abnormalities and die before weaning. McWhir, J., Selfridge, J., Harrison, D.J., Squires, S., Melton, D.W. Nat. Genet. (1993) [Pubmed]
  9. XPA versus ERCC1 as chemosensitising agents to cisplatin and mitomycin C in prostate cancer cells: role of ERCC1 in homologous recombination repair. Cummings, M., Higginbottom, K., McGurk, C.J., Wong, O.G., Köberle, B., Oliver, R.T., Masters, J.R. Biochem. Pharmacol. (2006) [Pubmed]
  10. Gene expression as a predictive marker of outcome in stage IIB-IIIA-IIIB non-small cell lung cancer after induction gemcitabine-based chemotherapy followed by resectional surgery. Rosell, R., Felip, E., Taron, M., Majo, J., Mendez, P., Sanchez-Ronco, M., Queralt, C., Sanchez, J.J., Maestre, J. Clin. Cancer Res. (2004) [Pubmed]
  11. ERCC1 and thymidylate synthase mRNA levels predict survival for colorectal cancer patients receiving combination oxaliplatin and fluorouracil chemotherapy. Shirota, Y., Stoehlmacher, J., Brabender, J., Xiong, Y.P., Uetake, H., Danenberg, K.D., Groshen, S., Tsao-Wei, D.D., Danenberg, P.V., Lenz, H.J. J. Clin. Oncol. (2001) [Pubmed]
  12. Characterization of p53 wild-type and null isogenic colorectal cancer cell lines resistant to 5-fluorouracil, oxaliplatin, and irinotecan. Boyer, J., McLean, E.G., Aroori, S., Wilson, P., McCulla, A., Carey, P.D., Longley, D.B., Johnston, P.G. Clin. Cancer Res. (2004) [Pubmed]
  13. Tailored chemotherapy for colorectal cancer: a new approach to therapy. Iqbal, S., Stoehlmacher, J., Lenz, H.J. Cancer Invest. (2004) [Pubmed]
  14. ERCC1/XPF removes the 3' overhang from uncapped telomeres and represses formation of telomeric DNA-containing double minute chromosomes. Zhu, X.D., Niedernhofer, L., Kuster, B., Mann, M., Hoeijmakers, J.H., de Lange, T. Mol. Cell (2003) [Pubmed]
  15. Phosphorylation of XPB helicase regulates TFIIH nucleotide excision repair activity. Coin, F., Auriol, J., Tapias, A., Clivio, P., Vermeulen, W., Egly, J.M. EMBO J. (2004) [Pubmed]
  16. A long-range restriction map of the human chromosome 19q13 region: close physical linkage between CKMM and the ERCC1 and ERCC2 genes. Smeets, H., Bachinski, L., Coerwinkel, M., Schepens, J., Hoeijmakers, J., van Duin, M., Grzeschik, K.H., Weber, C.A., de Jong, P., Siciliano, M.J. Am. J. Hum. Genet. (1990) [Pubmed]
  17. Defective repair of cisplatin-induced DNA damage caused by reduced XPA protein in testicular germ cell tumours. Köberle, B., Masters, J.R., Hartley, J.A., Wood, R.D. Curr. Biol. (1999) [Pubmed]
  18. Purification and characterization of the XPF-ERCC1 complex of human DNA repair excision nuclease. Park, C.H., Bessho, T., Matsunaga, T., Sancar, A. J. Biol. Chem. (1995) [Pubmed]
  19. Irofulven cytotoxicity depends on transcription-coupled nucleotide excision repair and is correlated with XPG expression in solid tumor cells. Koeppel, F., Poindessous, V., Lazar, V., Raymond, E., Sarasin, A., Larsen, A.K. Clin. Cancer Res. (2004) [Pubmed]
  20. ERCC1 mutations in UV-sensitive Chinese hamster ovary (CHO) cell lines. Hayashi, T., Takao, M., Tanaka, K., Yasui, A. Mutat. Res. (1998) [Pubmed]
  21. DNA repair capacity: inconsistency between effect of over-expression of five NER genes and the correlation to mRNA levels in primary lymphocytes. Vogel, U., Dybdahl, M., Frentz, G., Nexo, B.A. Mutat. Res. (2000) [Pubmed]
  22. A novel function of emodin: enhancement of the nucleotide excision repair of UV- and cisplatin-induced DNA damage in human cells. Chang, L.C., Sheu, H.M., Huang, Y.S., Tsai, T.R., Kuo, K.W. Biochem. Pharmacol. (1999) [Pubmed]
  23. Nucleotide excision repair genes as determinants of cellular sensitivity to cyclophosphamide analogs. Andersson, B.S., Sadeghi, T., Siciliano, M.J., Legerski, R., Murray, D. Cancer Chemother. Pharmacol. (1996) [Pubmed]
  24. MAPK dependence of DNA damage repair: ionizing radiation and the induction of expression of the DNA repair genes XRCC1 and ERCC1 in DU145 human prostate carcinoma cells in a MEK1/2 dependent fashion. Yacoub, A., Park, J.S., Qiao, L., Dent, P., Hagan, M.P. Int. J. Radiat. Biol. (2001) [Pubmed]
  25. Formation of a ternary complex by human XPA, ERCC1, and ERCC4(XPF) excision repair proteins. Park, C.H., Sancar, A. Proc. Natl. Acad. Sci. U.S.A. (1994) [Pubmed]
  26. Recruitment of the Nucleotide Excision Repair Endonuclease XPG to Sites of UV-Induced DNA Damage Depends on Functional TFIIH. Zotter, A., Luijsterburg, M.S., Warmerdam, D.O., Ibrahim, S., Nigg, A., van Cappellen, W.A., Hoeijmakers, J.H., van Driel, R., Vermeulen, W., Houtsmuller, A.B. Mol. Cell. Biol. (2006) [Pubmed]
  27. High mobility group A2 protein and its derivatives bind a specific region of the promoter of DNA repair gene ERCC1 and modulate its activity. Borrmann, L., Schwanbeck, R., Heyduk, T., Seebeck, B., Rogalla, P., Bullerdiek, J., Wisniewski, J.R. Nucleic Acids Res. (2003) [Pubmed]
  28. Complementation of the DNA repair-deficient swi10 mutant of fission yeast by the human ERCC1 gene. Rödel, C., Jupitz, T., Schmidt, H. Nucleic Acids Res. (1997) [Pubmed]
  29. Sensitivity of group F xeroderma pigmentosum cells to UV and mitomycin C relative to levels of XPF and ERCC1 overexpression. Yagi, T., Katsuya, A., Koyano, A., Takebe, H. Mutagenesis (1998) [Pubmed]
  30. Response of endothelial cells to a dual tyrosine kinase receptor inhibition combined with irradiation. Bozec, A., Formento, P., Ciccolini, J., Fanciullino, R., Padovani, L., Murraciole, X., Fischel, J.L., Milano, G. Mol. Cancer Ther. (2005) [Pubmed]
  31. Effect of interleukin-1 alpha and tumour necrosis factor-alpha on cisplatin-induced ERCC-1 mRNA expression in a human ovarian carcinoma cell line. Li, Q., Bostick-Bruton, F., Reed, E. Anticancer Res. (1998) [Pubmed]
  32. The putative glioma tumor suppressor gene on chromosome 19q maps between APOC2 and HRC. Rubio, M.P., Correa, K.M., Ueki, K., Mohrenweiser, H.W., Gusella, J.F., von Deimling, A., Louis, D.N. Cancer Res. (1994) [Pubmed]
  33. Repair of large insertion/deletion heterologies in human nuclear extracts is directed by a 5' single-strand break and is independent of the mismatch repair system. Littman, S.J., Fang, W.H., Modrich, P. J. Biol. Chem. (1999) [Pubmed]
  34. Identification and characterization of human MUS81-MMS4 structure-specific endonuclease. Oğrünç, M., Sancar, A. J. Biol. Chem. (2003) [Pubmed]
  35. Polymorphisms in genes of nucleotide and base excision repair: risk and prognosis of colorectal cancer. Moreno, V., Gemignani, F., Landi, S., Gioia-Patricola, L., Chabrier, A., Blanco, I., González, S., Guino, E., Capellà, G., Canzian, F. Clin. Cancer Res. (2006) [Pubmed]
  36. Physical and functional interaction between the XPF/ERCC1 endonuclease and hRad52. Motycka, T.A., Bessho, T., Post, S.M., Sung, P., Tomkinson, A.E. J. Biol. Chem. (2004) [Pubmed]
  37. Analysis of mutations at the DNA repair genes in acute childhood leukaemia. Lin, Y.W., Kubota, M., Koishi, S., Sawada, M., Usami, I., Watanabe, K., Akiyama, Y. Br. J. Haematol. (1998) [Pubmed]
  38. Association between polymorphisms of ERCC1 and XPD and survival in non-small-cell lung cancer patients treated with cisplatin combination chemotherapy. Ryu, J.S., Hong, Y.C., Han, H.S., Lee, J.E., Kim, S., Park, Y.M., Kim, Y.C., Hwang, T.S. Lung Cancer (2004) [Pubmed]
  39. Expression of excision repair genes in non-malignant bone marrow from cancer patients. Dabholkar, M., Bostick-Bruton, F., Weber, C., Egwuagu, C., Bohr, V.A., Reed, E. Mutat. Res. (1993) [Pubmed]
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