验证数据展示
产品信息
67716-1-PBS targets CEP290 in WB, Indirect ELISA applications and shows reactivity with Human, rat, mouse samples.
| 经测试应用 | WB, Indirect ELISA Application Description |
| 经测试反应性 | Human, rat, mouse |
| 免疫原 | CEP290 fusion protein Ag17457 种属同源性预测 |
| 宿主/亚型 | Mouse / IgG2b |
| 抗体类别 | Monoclonal |
| 产品类型 | Antibody |
| 全称 | centrosomal protein 290kDa |
| 别名 | 3H11Ag, BBS14, Cancer/testis antigen 87, centrosomal protein 290kDa, Centrosomal protein of 290 kDa, CEP290, CT87, FLJ13615, FLJ21979, JBTS5, JBTS6, KIAA0373, LCA10, MKS4, Nephrocystin 6, rd16, SLSN6, Tumor antigen se2 2 |
| 计算分子量 | 2479 aa, 290 kDa |
| 观测分子量 | 290 kDa |
| GenBank蛋白编号 | BC008641 |
| 基因名称 | CEP290 |
| Gene ID (NCBI) | 80184 |
| RRID | AB_2918499 |
| 偶联类型 | Unconjugated |
| 形式 | Liquid |
| 纯化方式 | Protein A purification |
| UNIPROT ID | O15078 |
| 储存缓冲液 | PBS only , pH 7.3 |
| 储存条件 | Store at -80°C. The product is shipped with ice packs. Upon receipt, store it immediately at -80°C |
背景介绍
Studies in the Chlamydomonas model system has shown that the ciliary protein CEP290 is a critical component of these Y-link junctions. Additionally, numerous studies have demonstrated that CEP290's function is critical for IFT-in CEP290 knockdown experiments, many proteins that would normally localize to the cilium fail to do so, and cilium formation is disrupted or absent. Mutations in CEP290 are accountable for cases of nephronophthisis, Leber congenital amaurosis and Joubert syndrome. In IF analtsis of HeLa cells, the white arrows show centrosome and cilium staining. Two isoforms were produced by alternative splicing with predicted MW of 290 and 180 kDa according to UniProt.
