ERCC3 |
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Identifiers |
Aliases |
ERCC3, excision repair cross-complementation group 3, BTF2, GTF2H, RAD25, TFIIH, XPB, TTD2, ERCC excision repair 3, TFIIH core complex helicase subunit, Ssl2 |
External IDs |
OMIM: 133510 MGI: 95414 HomoloGene: 96 GeneCards: ERCC3 |
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Gene location (Mouse) |
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Chr. |
Chromosome 18 (mouse) |
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Band |
18|18 B1 |
Start |
32,373,353 bp |
End |
32,403,204 bp |
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Gene ontology |
Molecular function |
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Cellular component |
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Biological process |
- response to hypoxia
- termination of RNA polymerase I transcription
- embryonic organ development
- transcription, DNA-templated
- response to UV
- 7-methylguanosine mRNA capping
- nucleotide-excision repair, DNA incision
- regulation of mitotic cell cycle phase transition
- apoptotic process
- positive regulation of apoptotic process
- protein phosphorylation
- hair cell differentiation
- response to oxidative stress
- UV protection
- cellular response to DNA damage stimulus
- transcription initiation from RNA polymerase II promoter
- global genome nucleotide-excision repair
- protein localization
- transcription elongation from RNA polymerase II promoter
- regulation of transcription, DNA-templated
- positive regulation of transcription by RNA polymerase II
- DNA topological change
- transcription initiation from RNA polymerase I promoter
- transcription by RNA polymerase II
- transcription-coupled nucleotide-excision repair
- nucleotide-excision repair
- nucleotide-excision repair, preincision complex stabilization
- DNA repair
- viral process
- nucleotide-excision repair, preincision complex assembly
- nucleotide-excision repair, DNA incision, 5'-to lesion
- nucleotide-excision repair, DNA duplex unwinding
- regulation of mitotic recombination
- promoter clearance from RNA polymerase II promoter
- transcription open complex formation at RNA polymerase II promoter
- regulation of transposition, RNA-mediated
- phosphorylation of RNA polymerase II C-terminal domain
- regulation of RNA polymerase II regulatory region sequence-specific DNA binding
- nucleotide-excision repair, DNA incision, 3'-to lesion
- transcription elongation from RNA polymerase I promoter
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Sources:Amigo / QuickGO
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Wikidata |
View/Edit Human |
View/Edit Mouse |
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XPB (xeroderma pigmentosum type B) is an ATP-dependent DNA helicase in humans that is a part of the TFIIH transcription factor complex.
Structure
The 3D-structure of the archaeal homolog of XPB has been solved by X-ray crystallography by Dr. John Tainer and his group at The Scripps Research Institute.
Function
XPB plays a significant role in normal basal transcription, transcription coupled repair (TCR), and nucleotide excision repair (NER). Purified XPB has been shown to unwind DNA with 3’-5’ polarity.
The function of the XPB(ERCC3) protein in NER is to assist in unwinding the DNA double helix after damage is initially recognized. NER is a multi-step pathway that removes a wide range of different DNA damages that distort normal base pairing. Such damages include bulky chemical adducts, UV-induced pyrimidine dimers, and several forms of oxidative damage. Mutations in the XPB(ERCC3) gene can lead, in humans, to xeroderma pigmentosum (XP) or XP combined with Cockayne syndrome (XPCS). Mutant XPB cells from individuals with the XPCS phenotype are sensitive to UV irradiation and acute oxidative stress.
Disorders
Mutations in XPB and other related complementation groups, XPA-XPG, leads to a number of genetic disorders such as Xeroderma pigmentosum, Cockayne's syndrome, and trichothiodystrophy.
Interactions
XPB has been shown to interact with:
Small molecule inhibitors
Potent, bioactive natural products like triptolide that inhibit mammalian transcription via inhibition of the XPB subunit of the general transcription factor TFIIH has been recently reported as a glucose conjugate for targeting hypoxic cancer cells with increased glucose transporter expression.
See also