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TFIIH basal transcription factor complex helicase XPB subunit; basic transcription factor 2 89 kD subunit; BTF2 p89; DNA excision repair protein ERCC-3; DNA repair protein complementing XP-B cells; TFIIH basal transcription factor complex 89 kD subunit; TFIIH 89 kD subunit; TFIIH p89; Xeroderma pigmentosum group B-complementing protein (ERCC3, XPB, XPBC, p89TFIIH)

Function: - ATP-dependent 3'-5' DNA helicase - one of two helicase activities of TFIIH - helicase activity is opposite in polarity to the other helicase, XP D-correcting protein - component of the core-TFIIH basal transcription factor - involved in nucleotide excision repair of DNA - when complexed to CAK, role in RNA transcription by RNA polymerase 2 - acts by opening DNA either around the RNA transcription start site or the DNA damage - interacts with PUF60 Structure: - belongs to the helicase family, RAD25/XPB subfamily - contains 1 helicase ATP-binding domain - contains 1 helicase C-terminal domain Compartment: nucleus Pathology: - defects in ERCC3 are the cause of a) xeroderma pigmentosum complementation group B b) trichothiodystrophy (photosensitive)

Interactions

molecular events

Related

ERCC-3, XP B-correcting protein gene, p89TFIIH gene, haywire or RAD25

General

excision repair cross complement (ERCC) or excision repair cross-complementing rodent repair deficiency, complementation group helicase (DNA unwinding protein, DNA untwisting protein) phosphoprotein transcription factor IIH subunit Xeroderma pigmentosum group complementing protein

Properties

SIZE: entity length = 782 aa MW = 89 kD COMPARTMENT: cell nucleus MOTIF: nuclear translocation signal {6-18} acidic region {20-28} MOTIF: acidic residue (SEVERAL) acidic region {256-265} MOTIF: acidic residue (SEVERAL) helicase NAME: helicase SITE: 327-488 MOTIF: ATP-binding site NAME: ATP-binding site SITE: 340-347 Ser phosphorylation site {S370} DEVH box {441-444} Helicase C-terminal {542-702} MOTIF: acidic region {697-700} MOTIF: acidic residue (SEVERAL) acidic region {721-728} MOTIF: acidic residue (SEVERAL)

References

  1. Schaeffer L, Roy R, Humbert S, Moncollin V, Vermeulen W, Hoeijmakers JH, Chambon P, Egly JM. DNA repair helicase: a component of BTF2 (TFIIH) basic transcription factor. Science. 1993 Apr 2;260(5104):58-63. PMID: 8465201
  2. Bootsma D & Hoeijmakers JH DNA repair. Engagement with transcription. Nature 363:114 1993 PMID: 8483493
  3. Winkler GS, Vermeulen W, Coin F, Egly JM, Hoeijmakers JH, Weeda G. Affinity purification of human DNA repair/transcription factor TFIIH using epitope-tagged xeroderma pigmentosum B protein. J Biol Chem. 1998 Jan 9;273(2):1092-8. PMID: 9422774
  4. UniProt :accession P19447
  5. Atlas of genetics & cytogenetics in oncology & haematology http://atlasgeneticsoncology.org/genes/XPBID296.html
  6. GeneReviews https://www.genecards.org/cgi-bin/carddisp.pl?gene=ERCC3
  7. Allelic variations of the XP genes http://www.xpmutations.org/

Component-of

molecular complex

Databases & Figures

OMIM correlations UniProt P19447 PFAM correlations Entrez Gene 2071 Kegg hsa:2071 Model for nucleotide excision repair (v2)