DDB1

Damage-specific DNA binding protein 1, 127kDa
PDB rendering based on 2b5l.
Available structures PDB Ortholog search: Template:Homologene2PDBe PDBe, Template:Homologene2uniprot RCSB List of PDB id codes 2b5l​, 2b5m​, 2b5n​, 2hye​
Identifiers
Symbols	DDB1 ; DDBA; UV-DDB1; XAP1; XPCE; XPE; XPE-BF
External IDs	Template:OMIM5 Template:MGI HomoloGene: 1448
Gene ontology Molecular function Template:GNF GO Template:GNF GO Template:GNF GO Cellular component Template:GNF GO Biological process Template:GNF GO Template:GNF GO
Orthologs
Template:GNF Ortholog box
Species	Human	Mouse
Entrez	n/a	n/a
Ensembl	n/a	n/a
UniProt	n/a	n/a
RefSeq (mRNA)	n/a	n/a
RefSeq (protein)	n/a	n/a
Location (UCSC)	n/a	n/a
PubMed search	n/a	n/a

Damage-specific DNA binding protein 1, 127kDa, also known as DDB1, is a human gene. This gene encodes the large subunit of DNA damage-binding protein which is a heterodimer composed of a large and a small subunit. This protein functions in nucleotide-excision repair. Its defective activity causes the repair defect in the patients with xeroderma pigmentosum complementation group E (XPE). However, it remains for mutation analysis to demonstrate whether the defect in XPE patients is in this gene or the gene encoding the small subunit. In addition, Best vitelliform mascular dystrophy is mapped to the same region as this gene on 11q, but no sequence alternations of this gene are demonstrated in Best disease patients.

Further reading

• Chu G, Chang E (1988). "Xeroderma pigmentosum group E cells lack a nuclear factor that binds to damaged DNA". Science. 242 (4878): 564–7. PMID 3175673.
• Lee TH, Elledge SJ, Butel JS (1995). "Hepatitis B virus X protein interacts with a probable cellular DNA repair protein". J. Virol. 69 (2): 1107–14. PMID 7815490.CS1 maint: Multiple names: authors list (link)
• Keeney S, Eker AP, Brody T; et al. (1994). "Correction of the DNA repair defect in xeroderma pigmentosum group E by injection of a DNA damage-binding protein". Proc. Natl. Acad. Sci. U.S.A. 91 (9): 4053–6. PMID 8171034.CS1 maint: Explicit use of et al. (link) CS1 maint: Multiple names: authors list (link)
• Keeney S, Chang GJ, Linn S (1993). "Characterization of a human DNA damage binding protein implicated in xeroderma pigmentosum E.". J. Biol. Chem. 268 (28): 21293–300. PMID 8407967.CS1 maint: Multiple names: authors list (link)
• Dualan R, Brody T, Keeney S; et al. (1996). "Chromosomal localization and cDNA cloning of the genes (DDB1 and DDB2) for the p127 and p48 subunits of a human damage-specific DNA binding protein". Genomics. 29 (1): 62–9. doi:10.1006/geno.1995.1215. PMID 8530102.CS1 maint: Explicit use of et al. (link) CS1 maint: Multiple names: authors list (link)
• Hwang BJ, Liao JC, Chu G (1996). "Isolation of a cDNA encoding a UV-damaged DNA binding factor defective in xeroderma pigmentosum group E cells". Mutat. Res. 362 (1): 105–17. PMID 8538642.CS1 maint: Multiple names: authors list (link)
• Nichols AF, Ong P, Linn S (1996). "Mutations specific to the xeroderma pigmentosum group E Ddb- phenotype". J. Biol. Chem. 271 (40): 24317–20. PMID 8798680.CS1 maint: Multiple names: authors list (link)
• Seki N, Hayashi A, Hattori A; et al. (2000). "cDNA cloning, tissue expression, and chromosomal assignment of a mouse gene, encoding a 127 kDa UV-damaged DNA binding protein which is defective in XPE cells". DNA Res. 6 (5): 319–22. PMID 10574459.CS1 maint: Explicit use of et al. (link) CS1 maint: Multiple names: authors list (link)
• Liu W, Nichols AF, Graham JA; et al. (2000). "Nuclear transport of human DDB protein induced by ultraviolet light". J. Biol. Chem. 275 (28): 21429–34. doi:10.1074/jbc.M000961200. PMID 10777491.CS1 maint: Explicit use of et al. (link) CS1 maint: Multiple names: authors list (link)
• Martinez E, Palhan VB, Tjernberg A; et al. (2001). "Human STAGA complex is a chromatin-acetylating transcription coactivator that interacts with pre-mRNA splicing and DNA damage-binding factors in vivo". Mol. Cell. Biol. 21 (20): 6782–95. doi:10.1128/MCB.21.20.6782-6795.2001. PMID 11564863.CS1 maint: Explicit use of et al. (link) CS1 maint: Multiple names: authors list (link)
• Chen X, Zhang Y, Douglas L, Zhou P (2002). "UV-damaged DNA-binding proteins are targets of CUL-4A-mediated ubiquitination and degradation". J. Biol. Chem. 276 (51): 48175–82. doi:10.1074/jbc.M106808200. PMID 11673459.CS1 maint: Multiple names: authors list (link)
• Rapić-Otrin V, McLenigan MP, Bisi DC; et al. (2002). "Sequential binding of UV DNA damage binding factor and degradation of the p48 subunit as early events after UV irradiation". Nucleic Acids Res. 30 (11): 2588–98. PMID 12034848.CS1 maint: Explicit use of et al. (link) CS1 maint: Multiple names: authors list (link)
• Bergametti F, Sitterlin D, Transy C (2002). "Turnover of hepatitis B virus X protein is regulated by damaged DNA-binding complex". J. Virol. 76 (13): 6495–501. PMID 12050362.CS1 maint: Multiple names: authors list (link)
• Bontron S, Lin-Marq N, Strubin M (2002). "Hepatitis B virus X protein associated with UV-DDB1 induces cell death in the nucleus and is functionally antagonized by UV-DDB2". J. Biol. Chem. 277 (41): 38847–54. doi:10.1074/jbc.M205722200. PMID 12151405.CS1 maint: Multiple names: authors list (link)
• Andrejeva J, Poole E, Young DF; et al. (2002). "The p127 subunit (DDB1) of the UV-DNA damage repair binding protein is essential for the targeted degradation of STAT1 by the V protein of the paramyxovirus simian virus 5". J. Virol. 76 (22): 11379–86. PMID 12388698.CS1 maint: Explicit use of et al. (link) CS1 maint: Multiple names: authors list (link)
• Strausberg RL, Feingold EA, Grouse LH; et al. (2003). "Generation and initial analysis of more than 15,000 full-length human and mouse cDNA sequences". Proc. Natl. Acad. Sci. U.S.A. 99 (26): 16899–903. doi:10.1073/pnas.242603899. PMID 12477932.CS1 maint: Explicit use of et al. (link) CS1 maint: Multiple names: authors list (link)
• Groisman R, Polanowska J, Kuraoka I; et al. (2003). "The ubiquitin ligase activity in the DDB2 and CSA complexes is differentially regulated by the COP9 signalosome in response to DNA damage". Cell. 113 (3): 357–67. PMID 12732143.CS1 maint: Explicit use of et al. (link) CS1 maint: Multiple names: authors list (link)
• Leupin O, Bontron S, Strubin M (2003). "Hepatitis B virus X protein and simian virus 5 V protein exhibit similar UV-DDB1 binding properties to mediate distinct activities". J. Virol. 77 (11): 6274–83. PMID 12743284.CS1 maint: Multiple names: authors list (link)
• Wertz IE, O'Rourke KM, Zhang Z; et al. (2004). "Human De-etiolated-1 regulates c-Jun by assembling a CUL4A ubiquitin ligase". Science. 303 (5662): 1371–4. doi:10.1126/science.1093549. PMID 14739464.CS1 maint: Explicit use of et al. (link) CS1 maint: Multiple names: authors list (link)
• Bouwmeester T, Bauch A, Ruffner H; et al. (2004). "A physical and functional map of the human TNF-alpha/NF-kappa B signal transduction pathway". Nat. Cell Biol. 6 (2): 97–105. doi:10.1038/ncb1086. PMID 14743216.CS1 maint: Explicit use of et al. (link) CS1 maint: Multiple names: authors list (link)