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Xeroderma Pigmentosum, Complementation Group B (XPB)

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ERCC3; XPBC; BTF2; RAD25; TFIIH; GTF2H; Excision Repair Cross-Complementing Rodent Repair Deficiency,Complementation Group 3; Basic transcription factor 2 89 kDa subunit

Xeroderma Pigmentosum, Complementation Group B (XPB)
CCC E732
HGNC 3435
MGI 95414
PubMed 19773279
RGD 1307139
UniProt P19447
The human genes correcting the rodent repair defects are termed excision-repair cross-complementing or ERCC genes. A number appended to the symbol refers to the rodent complementary group that is corrected by the human gene. The human ERCC3 gene product specifically corrects the defect in an early step of the DNA nucleotide excision-repair (NER) pathway of UV-sensitive rodent mutants of complementation group 3. See also ERCC1, ERCC2, ERCC4, ERCC5, and ERCC6, as well as the XRCC1 gene that corrects the x-ray sensitivity of the CHO mutant cell line EM-9. ERCC3 is an ATP-dependent DNA helicase that functions in nucleotide excision repair and complements xeroderma pigmentosum group B mutations. It also is the 89 kDa subunit of basal transcription factor 2 (TFIIH) and thus functions in class II transcription.

Organism species: Homo sapiens (Human)

CATALOG NO. PRODUCT NAME APPLICATIONS
Proteins RPE732Hu01 Recombinant Xeroderma Pigmentosum, Complementation Group B (XPB) Positive Control; Immunogen; SDS-PAGE; WB.
Antibodies n/a Monoclonal Antibody to Xeroderma Pigmentosum, Complementation Group B (XPB) Monoclonal Antibody Customized Service Offer
n/a Polyclonal Antibody to Xeroderma Pigmentosum, Complementation Group B (XPB) Polyclonal Antibody Customized Service Offer
Assay Kits n/a CLIA Kit for Xeroderma Pigmentosum, Complementation Group B (XPB) CLIA Kit Customized Service Offer
n/a ELISA Kit for Xeroderma Pigmentosum, Complementation Group B (XPB) ELISA Kit Customized Service Offer

Organism species: Mus musculus (Mouse)

CATALOG NO. PRODUCT NAME APPLICATIONS
Proteins n/a Recombinant Xeroderma Pigmentosum, Complementation Group B (XPB) Recombinant Protein Customized Service Offer
Antibodies n/a Monoclonal Antibody to Xeroderma Pigmentosum, Complementation Group B (XPB) Monoclonal Antibody Customized Service Offer
n/a Polyclonal Antibody to Xeroderma Pigmentosum, Complementation Group B (XPB) Polyclonal Antibody Customized Service Offer
Assay Kits n/a CLIA Kit for Xeroderma Pigmentosum, Complementation Group B (XPB) CLIA Kit Customized Service Offer
n/a ELISA Kit for Xeroderma Pigmentosum, Complementation Group B (XPB) ELISA Kit Customized Service Offer

Organism species: Rattus norvegicus (Rat)

CATALOG NO. PRODUCT NAME APPLICATIONS
Proteins n/a Recombinant Xeroderma Pigmentosum, Complementation Group B (XPB) Recombinant Protein Customized Service Offer
Antibodies n/a Monoclonal Antibody to Xeroderma Pigmentosum, Complementation Group B (XPB) Monoclonal Antibody Customized Service Offer
n/a Polyclonal Antibody to Xeroderma Pigmentosum, Complementation Group B (XPB) Polyclonal Antibody Customized Service Offer
Assay Kits n/a CLIA Kit for Xeroderma Pigmentosum, Complementation Group B (XPB) CLIA Kit Customized Service Offer
n/a ELISA Kit for Xeroderma Pigmentosum, Complementation Group B (XPB) ELISA Kit Customized Service Offer
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  2. "A presumed DNA helicase encoded by ERCC-3 is involved in the human repair disorders Xeroderma pigmentosum and Cockayne's syndrome."Cell 62:777-791(1990) [PubMed] [Europe PMC] [Abstract]
  3. "Structure and expression of the human XPBC/ERCC-3 gene involved in DNA repair disorders xeroderma pigmentosum and Cockayne's syndrome."Nucleic Acids Res. 19:6301-6308(1991) [PubMed] [Europe PMC] [Abstract]
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  6. "Correction of xeroderma pigmentosum repair defect by basal transcription factor BTF2 (TFIIH)."EMBO J. 13:1645-1653(1994) [PubMed] [Europe PMC] [Abstract]
  7. "The 62- and 80-kDa subunits of transcription factor IIH mediate the interaction with Epstein-Barr virus nuclear protein 2."Proc. Natl. Acad. Sci. U.S.A. 92:3259-3263(1995) [PubMed] [Europe PMC] [Abstract]
  8. "Immunoaffinity purification and functional characterization of human transcription factor IIH and RNA polymerase II from clonal cell lines that conditionally express epitope-tagged subunits of the multiprotein complexes."J. Biol. Chem. 273:34444-34453(1998) [PubMed] [Europe PMC] [Abstract]
  9. "Reconstitution of the transcription factor TFIIH: assignment of functions for the three enzymatic subunits, XPB, XPD, and cdk7."Mol. Cell 3:87-95(1999) [PubMed] [Europe PMC] [Abstract]
  10. "The FBP interacting repressor targets TFIIH to inhibit activated transcription."Mol. Cell 5:331-341(2000) [PubMed] [Europe PMC] [Abstract]
  11. "Defective interplay of activators and repressors with TFIH in xeroderma pigmentosum."Cell 104:353-363(2001) [PubMed] [Europe PMC] [Abstract]
  12. "A summary of mutations in the UV-sensitive disorders: xeroderma pigmentosum, Cockayne syndrome, and trichothiodystrophy."Hum. Mutat. 14:9-22(1999) [PubMed] [Europe PMC] [Abstract]
  13. "MCAF1/AM is involved in Sp1-mediated maintenance of cancer-associated telomerase activity."J. Biol. Chem. 284:5165-5174(2009) [PubMed] [Europe PMC] [Abstract]
  14. "An enzyme assisted RP-RPLC approach for in-depth analysis of human liver phosphoproteome."J. Proteomics 96:253-262(2014) [PubMed] [Europe PMC] [Abstract]
  15. "Structure of the C-terminal half of human XPB helicase and the impact of the disease-causing mutation XP11BE."Acta Crystallogr. D 69:237-246(2013) [PubMed] [Europe PMC] [Abstract]
  16. "Clinical heterogeneity within xeroderma pigmentosum associated with mutations in the DNA repair and transcription gene ERCC3."Am. J. Hum. Genet. 54:191-200(1994) [PubMed] [Europe PMC] [Abstract]
  17. "A mutation in the XPB/ERCC3 DNA repair transcription gene, associated with trichothiodystrophy."Am. J. Hum. Genet. 60:320-329(1997) [PubMed] [Europe PMC] [Abstract]
  18. "Identification of four single nucleotide polymorphisms in DNA repair genes: XPA and XPB (ERCC3) in Polish population."Hum. Mutat. 15:577-578(2000) [PubMed] [Europe PMC] [Abstract]
  19. "Phenotypic heterogeneity in the XPB DNA helicase gene (ERCC3): xeroderma pigmentosum without and with Cockayne syndrome."Hum. Mutat. 27:1092-1103(2006) [PubMed] [Europe PMC] [Abstract]
  20. "The consensus coding sequences of human breast and colorectal cancers." Science 314:268-274(2006) [PubMed] [Europe PMC] [Abstract]