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- bs-11946P
- 2025年10月16日
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500ug
| 产品编号 | bs-11946P |
| 英文名称 | RPGRIP1L Antibody Blocking Peptide |
| 中文名称 | 梅克尔憩室综合征相关蛋白5封闭多肽 |
| 英文别名 | CORS 3; CORS3; Fantom; FTM; JBTS 1; JBTS 7; JBTS1; JBTS7; Joubert syndrome 1; Joubert syndrome 7; Meckel syndrome, type 1; RPGRIP1-like; Meckel syndrome, type 5; MKS 5; MKS5; NPHP 8; NPHP8; nephrocystin 8; Protein fantom; Retinitis pigmentosa GTPase regulator interacting protein 1 like; RPGR interacting protein 1 like protein; RPGRIP1 like protein; FTM_HUMAN. |
| 纯化方法 | HPLC |
| 研究领域 | Neuroscience > Neurology process > Neurogenesis |
| 亚基 | Interacts with NPHP4 in a complex containing NPHP1, NPHP4 and RPGRIP1L/NPHP8. Interacts with TBXA2R (via C-terminus). Interacts with IQCB1; the interaction likely requires additional interactors. Interacts with RPGR. |
| 亚细胞定位 | Cytoplasm. Cell projection, cilium basal body. Cell projection, cilium axoneme. Centrosome. |
| 组织特异性 | Ubiquitously expressed with relatively high level of expression in hypothalamus and islet. During early development, expressed in multiple organs including brain, eye, forelimb and kidney. |
| 相似性 | Belongs to the RPGRIP1 family. Contains 2 C2 domains. |
| 功能 | RPGRIP1L (retinitis pigmentosa GTPase regulator interacting protein 1 like), also known as Fantom, is a ciliary basal body protein. It is thought to play an important role in development and apoptosis. Mutations in the gene encoding RPGRIP1L are associated with Joubert's Syndrome and Meckel's Syndrome, which are characterised by a number of developmental abnormalities. |
| 保存条件 | Shipped at 4℃. Stored at -20℃ for one year. Avoid repeated freeze/thaw cycles. |
| 注意事项 | This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications. |
| 背景资料 | RPGRIP1L is a 1,315 amino acid protein that belongs to the RPGRIP1 family and is thought to function in programmed cell death, craniofacial development and formation of the left-right axis. Existing as two alternatively spliced isoforms that localize to the cytoplasm, cytoskeleton, centrosome and cilium basal body, RPGRIP1L interacts with nephrocystin-4 and is moderately expressed in brain, retina and kidney. Containing two C2 domains, RPGRIP1L is encoded by a gene that maps to human chromosome 16q12.2. Defects in the gene encoding RPGRIP1L are the cause of Joubert syndrome type 7 (JBTS7), COACH syndrome (COACHS) and Meckel syndrome type 5 (MKS5). |
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RPGRIP1L Antibody Blocking Peptide(bs-11946P)-500ug
￥880
