| 货号 | AF3014-SP |
| 别名 | EVR2; exudative vitreoretinopathy 2 (X-linked); FEVR; ND; NDP; Norrie disease (pseudoglioma); Norrie disease protein; norrin; X-linked exudative vitreoretinopathy 2 protein |
| 反应种属 | Human |
| 应用 | Western Blot(0.1 µg/mL) Immunohistochemistry(5-15 µg/mL) |
| 目标/特异性 | Detects human Norrin in direct ELISAs and Western blots. In direct ELISAs, approximately 50% cross-reactivity with recombinant mouse Norrin is observed. |
| 使用方法 | Western Blot: 0.1 µg/mL Immunohistochemistry: 5-15 µg/mL Blockade of Receptor-ligand Interaction: In a functional ELISA, 2-6 µg/mL of this antibody will block 50% of the binding of 0.25 μg/mL of Recombinant Mouse Frizzled-4 Fc Chimera to immobilized Recombinant Human Norrin coated at 0.5 µg/mL (100 µL/well). At 50 μg/mL, this antibody will block >80% of the binding. |
| 来源 | Polyclonal Goat IgG |
| 产品组分 |
| 供应商 | R&D Systems |
| Entrez Gene IDs | 4693 (Human) |
| 应用文献 | |
| R&D Systems personnel manually curate a database that contains references using R&D Systems products. The data collected includes not only links to publications in PubMed, but also provides information about sample types, species, and experimental conditions. Norrin promotes vascular regrowth after oxygen-induced retinal vessel loss and suppresses retinopathy in mice. | |
| 纯化方式 | Antigen Affinity-purified |
| 免疫原 | E. coli-derived recombinant human Norrin Lys25-Ser133 Accession # Q00604 |
| 内毒素水平 | <0.10 EU per 1 μg of the antibody by the LAL method. |
| 生物活性 | Human |
| 标记 | Unconjugated |
| 溶解方法 | Reconstitute at 0.2 mg/mL in sterile PBS. |
| 背景 | Norrin (also called Norrie Disease Protein or NDP) is a secreted regulatory protein that remains tightly associated with the extracellular matrix (1). At least 70 mutations of Norrin have been identified in Norrie disease (X-linked retinal dysplasia) or in a minority of X-linked familial exudative vitreoretinopathy (FEVR). Both are disorders of retinal vascularization that cause blindness (2, 3). Norrin consists of disulfide-linked homodimers that oligomerize further via disulfide bridges to form higher order oligomers containing up to ten units of 12 kDa each. The cysteine-rich C-terminal domain of Norrin is homologous to von Willebrand factor, several extracellular mucin proteins, and members of the TGF-beta family. Molecular modeling studies predict that Norrin assumes a cysteine-knot structure typical for the TGF‑ beta family (4). Although Norrin is not related to Wnt family proteins, it functions like a Wnt protein in that it binds with high affinity to the receptor Frizzled-4, requires LDL receptor-related protein (LRP) as a co-receptor, and induces activation of the canonical Wnt signaling pathway (5). Norrin and Frizzled-4 are expressed at relatively low levels in tissues displaying vascular phenotypes and genetic disruption of either gene in mice gives phenotypes with marked similarities (5, 6, 7). Human Norrin shares 100%, 96%, 95%, 94% and 90% amino acid identity with rhesus macaque, canine, bovine, mouse, and chick Norrin, respectively. |
| 运输条件 | Blue Ice |
| 存放说明 | 4℃ |
| 参考文献 |
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