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Overview
| 别名 | XPE; DDBA; XAP1; XPCE; XPE-BF; UV-DDB1; WHIKERS |
| 基因名 | DDB1 |
| UniProt ID | Q16531 |
| 反应种属 | Human,Mouse,Rat |
| 应用 | WB,IHC-P,ICC/IF |
| 宿主 | Rabbit |
| 偶联物 | Unconjugated |
| 修饰 | Unmodified |
| 亚型 | IgG |
| 克隆号 | R02-3T-6 |
| 克隆性 | Monoclonal Antibody |
| 分子量 | Calculated MW: 127 kDa; Observed MW: 127 kDa |
| 纯化方式 | Affinity Chromatography |
| 产品形式 | Liquid |
| 推荐稀释比 | WB-1:1000-1:5000; IHC-P-1:100-1:200; ICC/IF-1:50-1:100 |
| 存储缓冲液 | Liquid in 50mM Tris-Glycine(pH 7.4), 0.15M NaCl, 40%Glycerol, 0.01% sodium azide and 0.05% BSA. |
| 保存温度 | Store at 4°C short term. Aliquot and store at -20°C long term. Avoid freeze/thaw cycles. |
| 背景信息 | 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. |
检测原理