ERCC3 Antibody – Mouse Monoclonal

This gene encodes an ATP-dependent DNA helicase that functions in nucleotide excision repair. The encoded protein is a subunit of basal transcription factor 2 (TFIIH) and, therefore, also functions in class II transcription. Mutations in this gene are associated with Xeroderma pigmentosum B, Cockayne's syndrome, and trichothiodystrophy. Alternative splicing results in multiple transcript variants. [provided by RefSeq, Dec 2014]

XPB: ATP-dependent 3'-5' DNA helicase, component of the core- TFIIH basal transcription factor, involved in nucleotide excision repair (NER) of DNA and, when complexed to CAK, in RNA transcription by RNA polymerase II. Acts by opening DNA either around the RNA transcription start site or the DNA damage. One of the 6 subunits forming the core-TFIIH basal transcription factor which associates with the CAK complex composed of CDK7, CCNH/cyclin H and MNAT1 to form the TFIIH basal transcription factor. Interacts with PUF60. Interacts with ATF7IP. Interacts with Epstein-Barr virus EBNA2. Belongs to the helicase family. RAD25/XPB subfamily.

Protein type: EC 3.6.4.12; DNA repair, damage; Helicase; Transcription factor

Chromosomal Location of Human Ortholog: 2q21

Cellular Component: nucleoplasm; holo TFIIH complex; nucleus

Molecular Function: protein C-terminus binding; DNA-dependent ATPase activity; GTP binding; ATPase activity; 3'-5' DNA helicase activity; dATP binding; protein N-terminus binding; peptide binding; transcription factor binding; protein kinase activity; RNA polymerase subunit kinase activity; ATP-dependent DNA helicase activity; protein binding; DNA binding; damaged DNA binding; ATP binding

Biological Process: transcription from RNA polymerase II promoter; DNA topological change; viral reproduction; positive regulation of apoptosis; apoptosis; positive regulation of viral transcription; protein amino acid phosphorylation; regulation of gene expression, epigenetic; protein localization; mRNA capping; UV protection; negative regulation of gene expression, epigenetic; transcription-coupled nucleotide-excision repair; nucleotide-excision repair, DNA damage removal; response to UV; transcription initiation from RNA polymerase II promoter; RNA elongation from RNA polymerase I promoter; hair cell differentiation; nucleotide-excision repair, DNA incision; transcription from RNA polymerase I promoter; termination of RNA polymerase I transcription; DNA repair; nucleotide-excision repair, DNA duplex unwinding; nucleotide-excision repair; RNA elongation from RNA polymerase II promoter; response to hypoxia; positive regulation of transcription from RNA polymerase II promoter; gene expression; response to oxidative stress; transcription initiation from RNA polymerase I promoter

Disease: Trichothiodystrophy 2, Photosensitive; Xeroderma Pigmentosum, Complementation Group B