| Description : |
The protein encoded by this gene is the large subunit (p127) of the heterodimeric DNA damage-binding (DDB) complex while another protein (p48) forms the small subunit. This protein complex functions in nucleotide-excision repair and binds to DNA following UV damage. Defective activity of this complex causes the repair defect in patients with xeroderma pigmentosum complementation group E (XPE) - an autosomal recessive disorder characterized by photosensitivity and early onset of carcinomas. 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. The protein encoded by this gene also functions as an adaptor molecule for the cullin 4 (CUL4) ubiquitin E3 ligase complex by facilitating the binding of substrates to this complex and the ubiquitination of proteins. |
| Source : |
In Vitro Cell Free System |
| Species : |
Human |
| Tag : |
GST |
| Form : |
50 mM Tris-HCl, 10 mM reduced Glutathione, pH=8.0 in the elution buffer. |
| Molecular Mass : |
153.4 kDa |
| Protein Length : |
1140 amino acids |
| AA Sequence : |
MSYNYVVTAQKPTAVNGCVTGHFTS AEDLNLLIAKNTRLEIYVVTAEGLR PVKEVGMYGKIAVMELFRPKGESKD LLFILTAKYNACILEYKQSGESIDI ITRAHGNVQDRIGRPSETGIIGIID PECRMIGLRLYDGLFKVIPLDRDNK ELKAFNIRLEELHVIDVKFLYGCQA PTICFVYQDPQGRHVKTYEVSLREK EFNKGPWKQENVEAEASMVIAVPEP FGGAIIIGQESITYHNGDKYLAIAP PIIKQSTIVCHNRVDPNGSRYLLGD MEGRLFMLLLEKEEQMDGTVTLKDL RVELLGETSIAECLTYLDNGVVFVG SRLGDSQLVKLNVDSNEQGSYVVAM ETFTNLGPIVDMCVVDLERQGQGQL VTCSGAFKEGSLRIIRNGIGIHEHA SIDLPGIKGLWPLRSDPNRETDDTL VLSFVGQTRVLMLNGEEVEETELMG FVDDQQTFFCGNVAHQQLIQITSAS VRLVSQEPKALVSEWKEPQAKNISV ASCNSSQVVVAVGRALYYLQIHPQE LRQISHTEMEHEVACLDITPLGDSN GLSPLCAIGLWTDISARILKLPSFE LLHKEMLGGEIIPRSILMTTFESSH YLLCALGDGALFYFGLNIETGLLSD RKKVTLGTQPTVLRTFRSLSTTNVF ACSDRPTVIYSSNHKLVFSNVNLKE VNYMCPLNSDGYPDSLALANNSTLT IGTIDEIQKLHIRTVPLYESPRKIC YQEVSQCFGVLSSRIEVQDTSGGTT ALRPSASTQALSSSVSSSKLFSSST APHETSFGEEVEVHNLLIIDQHTFE VLHAHQFLQNEYALSLVSCKLGKDP NTYFIVGTAMVYPEEAEPKQGRIVV FQYSDGKLQTVAEKEVKGAVYSMVE FNGKLLASINSTVRLYEWTTEKELR TECNHYNNIMALYLKTKGDFILVGD LMRSVLLLAYKPMEGNFEEIARDFN PNWMSAVEILDDDNFLGAENAFNLF VCQKDSAATTDEERQHLQEVGLFHL GEFVNVFCHGSLVMQNLGETSTPTQ GSVLFGTVNGMIGLVTSLSESWYNL LLDMQNRLNKVIKSVGKIEHSFWRS FHTERKTEPATGFIDGDLIESFLDI SRPKMQEVVANLQYDDGSGMKREAT ADDLIKVVEELTRIH |
| Applications : |
Enzyme-linked Immunoabsorbent; Assay Western Blot (Recombinant protein); Antibody Production; Protein Array |
| Notes : |
Best use within three months from the date of receipt of this protein. |
| Storage : |
Store at -80 centigrade. Aliquot to avoid repeated freezing and thawing. |
