Gene Symbol: Ercc3
Description: ERCC excision repair 3, TFIIH core complex helicase subunit
Alias: general transcription and DNA repair factor IIH helicase subunit XPB, DNA excision repair protein ERCC-3, TFIIH basal transcription factor complex helicase XPB subunit, TFIIH subunit XPB, excision repair cross-complementation group 3, excision repair cross-complementing rodent repair deficiency, complementation group 3
Species: rat
Products:     Ercc3

Top Publications

  1. Jin K, Mao X, Eshoo M, Nagayama T, Minami M, Simon R, et al. Microarray analysis of hippocampal gene expression in global cerebral ischemia. Ann Neurol. 2001;50:93-103 pubmed
    ..induction of several genes implicated previously in cerebral ischemia, including ALG2, APP, CASP3, CLU, ERCC3, GADD34, GADD153, IGFBP2, TIAR, VEGF, and VIM, as well as other genes not so implicated...
  2. Hermon M, Cairns N, Egly J, Fery A, Labudova O, Lubec G. Expression of DNA excision-repair-cross-complementing proteins p80 and p89 in brain of patients with Down Syndrome and Alzheimer's disease. Neurosci Lett. 1998;251:45-8 pubmed
    ..ERCC2-protein 80 kDa and ERCC3-protein p89 were determined in five individual brain regions of controls, aged DS and AD patients...
  3. Fang Kircher S, Labudova O, Kitzmueller E, Rink H, Cairns N, Lubec G. Increased steady state mRNA levels of DNA-repair genes XRCC1, ERCC2 and ERCC3 in brain of patients with Down syndrome. Life Sci. 1999;64:1689-99 pubmed
    ..stress and cells of patients with DS are more susceptible to ionizing irradiation, we decided to study ERCC2, ERCC3 and XRCC1, representatives of repair genes known to be involved in the repair of oxidative DNA-damage...
  4. Iyer N, Reagan M, Wu K, Canagarajah B, Friedberg E. Interactions involving the human RNA polymerase II transcription/nucleotide excision repair complex TFIIH, the nucleotide excision repair protein XPG, and Cockayne syndrome group B (CSB) protein. Biochemistry. 1996;35:2157-67 pubmed
    ..Our analyses demonstrate that the XPB, XPD, p44, and p62 proteins interact with each other. XPG protein interacts with multiple subunits of TFIIH and with CSB protein. ..
  5. Marini F, Nardo T, Giannattasio M, Minuzzo M, Stefanini M, Plevani P, et al. DNA nucleotide excision repair-dependent signaling to checkpoint activation. Proc Natl Acad Sci U S A. 2006;103:17325-30 pubmed
    ..Inhibition of RNA polymerase II transcription significantly reduces the phosphorylation of key checkpoint factors in XP/CS fibroblasts on exposure to UV damage...
  6. Drapkin R, Le Roy G, Cho H, Akoulitchev S, Reinberg D. Human cyclin-dependent kinase-activating kinase exists in three distinct complexes. Proc Natl Acad Sci U S A. 1996;93:6488-93 pubmed
    ..CAK-ERCC2 can efficiently associate with core-TFIIH to reconstitute holo-TFIIH transcription activity. We present evidence proposing a critical role for ERCC2 in mediating the association of CAK with core TFIIH subunits. ..
  7. Weeda G, Ma L, van Ham R, Bootsma D, van der Eb A, Hoeijmakers J. Characterization of the mouse homolog of the XPBC/ERCC-3 gene implicated in xeroderma pigmentosum and Cockayne's syndrome. Carcinogenesis. 1991;12:2361-8 pubmed
    ..The mouse XPBC/ERCC-3 gene is expressed constitutively at low levels in all tissues examined except for testis, where its expression is significantly enhanced. ..
  8. Coin F, Oksenych V, Egly J. Distinct roles for the XPB/p52 and XPD/p44 subcomplexes of TFIIH in damaged DNA opening during nucleotide excision repair. Mol Cell. 2007;26:245-56 pubmed
    ..Our results suggest a mechanism in which the helicase activity of XPB is not used for the opening and repair of damaged DNA, which is instead only driven by its ATPase activity, in combination with the helicase activity of XPD. ..
  9. Maru Y, Kobayashi T, Tanaka K, Shibuya M. BCR binds to the xeroderma pigmentosum group B protein. Biochem Biophys Res Commun. 1999;260:309-12 pubmed
    ..GST-XPB(1-782) was largely in the nucleus and bound to p62 and BCR. Although the biological significance of the binding remains to be uncovered, BCR binds to the XPB/p62 complex. ..

More Information


  1. Weeda G, van Ham R, Vermeulen W, Bootsma D, van der Eb A, Hoeijmakers J. A presumed DNA helicase encoded by ERCC-3 is involved in the human repair disorders xeroderma pigmentosum and Cockayne's syndrome. Cell. 1990;62:777-91 pubmed
    ..Because XP is associated with predisposition to skin cancer, ERCC-3 can be considered a tumor-preventing gene. ..
  2. Sullivan I, Salazar J, Majem M, Pallares C, del Rio E, Paez D, et al. Pharmacogenetics of the DNA repair pathways in advanced non-small cell lung cancer patients treated with platinum-based chemotherapy. Cancer Lett. 2014;353:160-6 pubmed publisher
    ..We analyzed 17 SNPs in eight genes (ERCC1, ERCC2, ERCC3, ERCC4, ERCC5, XPA, XRCC1 and XRCC2) involved in DNA repair mechanisms and its association with outcome in NSCLC...
  3. Oh K, Imoto K, Boyle J, Khan S, Kraemer K. Influence of XPB helicase on recruitment and redistribution of nucleotide excision repair proteins at sites of UV-induced DNA damage. DNA Repair (Amst). 2007;6:1359-70 pubmed
    ..Ineffectual repair of UV-induced photoproducts resulting from delayed recruitment and impaired redistribution of NER proteins may contribute to the markedly increased frequency of skin cancer in XP patients. ..
  4. Hu Z, Xu L, Shao M, Yuan J, Wang Y, Wang F, et al. Polymorphisms in the two helicases ERCC2/XPD and ERCC3/XPB of the transcription factor IIH complex and risk of lung cancer: a case-control analysis in a Chinese population. Cancer Epidemiol Biomarkers Prev. 2006;15:1336-40 pubmed
    The transcription factor IIH (TFIIH) helicases ERCC2/XPD and ERCC3/XPB are responsible for opening the DNA strand around the lesion site during nucleotide excision repair process...
  5. Reardon J, Ge H, Gibbs E, Sancar A, Hurwitz J, Pan Z. Isolation and characterization of two human transcription factor IIH (TFIIH)-related complexes: ERCC2/CAK and TFIIH. Proc Natl Acad Sci U S A. 1996;93:6482-7 pubmed
    ..TFIIH* consists of a subset of the TFIIH complex proteins including ERCC3 (XPB), p62, p44, p41, and p34 but is devoid of detectable levels of ERCC2 (XPD) and CAK...
  6. Serizawa H, Conaway R, Conaway J. Multifunctional RNA polymerase II initiation factor delta from rat liver. Relationship between carboxyl-terminal domain kinase, ATPase, and DNA helicase activities. J Biol Chem. 1993;268:17300-8 pubmed
  7. Egly J, Coin F. A history of TFIIH: two decades of molecular biology on a pivotal transcription/repair factor. DNA Repair (Amst). 2011;10:714-21 pubmed publisher
    ..This review intends to give a non-exhaustive survey of the most prominent discoveries on the molecular functioning of TFIIH. ..
  8. Wang X, Vermeulen W, Coursen J, Gibson M, Lupold S, Forrester K, et al. The XPB and XPD DNA helicases are components of the p53-mediated apoptosis pathway. Genes Dev. 1996;10:1219-32 pubmed
    ..Direct microinjection of the p53 carboxy-terminal-derived peptide (amino acid residues 319-393) resulted in apoptosis of primary normal human fibroblasts. These results disclose a novel pathway of p53-induced apoptosis. ..
  9. Hu G, Liu L, Zhang J, Hu X, Duan H, Deng H, et al. The role of XPB in cell apoptosis and viability and its relationship with p53, p21(waf1/cip1) and c-myc in hepatoma cells. Dig Liver Dis. 2006;38:755-61 pubmed
    ..1-XPB cells. Our results indicated that XPB could inhibit the proliferation of hepatoma cells and had a positive effect on the expression of p53 and p21(waf1/cip1) but a negative effect on c-myc. ..
  10. Weeda G, Rossignol M, Fraser R, Winkler G, Vermeulen W, van T Veer L, et al. The XPB subunit of repair/transcription factor TFIIH directly interacts with SUG1, a subunit of the 26S proteasome and putative transcription factor. Nucleic Acids Res. 1997;25:2274-83 pubmed
    ..Since SUG1 is an integral component of the 26S proteasome and may be part of the mediator, our findings disclose a SUG1-dependent link between TFIIH and the cellular machinery involved in protein modelling/degradation. ..
  11. Qadri I, Conaway J, Conaway R, Schaack J, Siddiqui A. Hepatitis B virus transactivator protein, HBx, associates with the components of TFIIH and stimulates the DNA helicase activity of TFIIH. Proc Natl Acad Sci U S A. 1996;93:10578-83 pubmed
    ..We have identified ERCC3 and ERCC2 DNA helicase subunits of holoenzyme TFIIH as targets of HBx interactions...
  12. Oh K, Khan S, Jaspers N, Raams A, Ueda T, Lehmann A, et al. Phenotypic heterogeneity in the XPB DNA helicase gene (ERCC3): xeroderma pigmentosum without and with Cockayne syndrome. Hum Mutat. 2006;27:1092-103 pubmed
    Defects in the xeroderma pigmentosum type B (XPB) gene (ERCC3), a DNA helicase involved in nucleotide excision repair (NER) and an essential subunit of the basal transcription factor, TFIIH, have been described in only three families...
  13. Bergmann E, Egly J. Trichothiodystrophy, a transcription syndrome. Trends Genet. 2001;17:279-86 pubmed
    ..In a third group of photosensitive patients, TTD-A, no mutation has been identified, although TFIIH amount is reduced. ..
  14. Decordier I, De Bont K, De Bock K, Mateuca R, Roelants M, Ciardelli R, et al. Genetic susceptibility of newborn daughters to oxidative stress. Toxicol Lett. 2007;172:68-84 pubmed
    ..However, these conclusions might not be extrapolable to other types of DNA damage and need confirmation in a study on a larger population. ..
  15. Compe E, Egly J. TFIIH: when transcription met DNA repair. Nat Rev Mol Cell Biol. 2012;13:343-54 pubmed publisher
    ..In this way, studies of TFIIH have revealed tight molecular connections between transcription and DNA repair and have helped to define the concept of 'transcription diseases'. ..
  16. Chiappe Gutierrez M, Kitzmueller E, Labudova O, Fuerst G, Hoeger H, Hardmeier R, et al. mRNA levels of the hypoxia inducible factor (HIF-1) and DNA repair genes in perinatal asphyxia of the rat. Life Sci. 1998;63:1157-67 pubmed occur in PA, we determined mRNA levels of two genes representing DNA nucleotide excision repair, ERCC2 and ERCC3, and a DNA repair gene involved in the repair of oxidation mediated DNA damage, XRCC1...
  17. Weeda G, Eveno E, Donker I, Vermeulen W, Chevallier Lagente O, Taieb A, et al. A mutation in the XPB/ERCC3 DNA repair transcription gene, associated with trichothiodystrophy. Am J Hum Genet. 1997;60:320-9 pubmed
    ..quot; ..
  18. Busso D, Keriel A, Sandrock B, Poterszman A, Gileadi O, Egly J. Distinct regions of MAT1 regulate cdk7 kinase and TFIIH transcription activities. J Biol Chem. 2000;275:22815-23 pubmed
  19. Yang S, Zhang Y, Zhang L, Huang Y, Sun F. Immunohistochemical analysis of nucleotide excision repair factors XPA and XPB in adult rat brain. Anat Rec (Hoboken). 2008;291:775-80 pubmed publisher
    ..This study provides the first evidence that NER factors XPA and XPB exist in the nuclei of neurons in the brain, suggesting that the NER may play important roles in the process of DNA repair in adult brain neurons. ..
  20. 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. ..
  21. Vinson R, Hales B. Nucleotide excision repair gene expression in the rat conceptus during organogenesis. Mutat Res. 2001;486:113-23 pubmed
    ..Exposure of the conceptus to a teratogen, 4-OOHCPA, induced malformations without affecting NER transcript levels. Thus, NER gene expression in the conceptus was unresponsive to regulation by DNA alkylation. ..
  22. Hwang J, Moncollin V, Vermeulen W, Seroz T, van Vuuren H, Hoeijmakers J, et al. A 3' --> 5' XPB helicase defect in repair/transcription factor TFIIH of xeroderma pigmentosum group B affects both DNA repair and transcription. J Biol Chem. 1996;271:15898-904 pubmed
  23. Andressoo J, Weeda G, de Wit J, Mitchell J, Beems R, van Steeg H, et al. An Xpb mouse model for combined xeroderma pigmentosum and cockayne syndrome reveals progeroid features upon further attenuation of DNA repair. Mol Cell Biol. 2009;29:1276-90 pubmed publisher
    ..The double-mutant cells exhibit sensitivity to oxidative stress, suggesting a role for endogenous DNA damage in the onset of XPB-associated CS...