XPB
Lua error in Module:Infobox_gene at line 53: attempt to index field 'wikibase' (a nil value). XPB (xeroderma pigmentosum type B) is an ATP-dependent DNA helicase in humans that is a part of the TFIIH transcription factor complex.
Structure
[edit | edit source]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.[1]
Function
[edit | edit source]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).[2] Mutant XPB cells from individuals with the XPCS phenotype are sensitive to UV irradiation and acute oxidative stress.[3]
XPB helicase is also a component of the p53-mediated programmed cell death (apoptosis) pathway.[4]
Disorders
[edit | edit source]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
[edit | edit source]XPB has been shown to interact with:
Small molecule inhibitors
[edit | edit source]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.[15]
See also
[edit | edit source]References
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Further reading
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External links
[edit | edit source]- GeneReviews/NIH/NCBI/UW entry on Xeroderma Pigmentosum
- XPBC-ERCC-3+protein at the U.S. National Library of Medicine Medical Subject Headings (MeSH)
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