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
  • xeroderma pigmentosum complementation group B
  • trichothiodystrophy (photosensitive)

More general terms

• helicase (DNA unwinding protein, DNA untwisting protein)
• transcription factor IIH subunit
• excision repair cross complement
• Xeroderma pigmentosum group complementing protein
• phosphoprotein

Database

• UniProt: http://www.uniprot.org/uniprot/P19447.html
• Kegg: http://www.genome.jp/dbget-bin/www_bget?hsa:2071
• OMIM: https://mirror.omim.org/entry/133510
• OMIM: https://mirror.omim.org/entry/601675
• Entrez gene: http://www.ncbi.nlm.nih.gov/sites/entrez?db=gene&cmd=Retrieve&dopt=Graphics&list_uids=2071