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- jlcR19062
- 国内
- 2025年07月14日
- WB,ELISA等
- 人/动物/植物
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- 详细信息
- 文献和实验
- 技术资料
- 供应商：
江西江蓝纯生物试剂有限公司
- 库存：
177
- 克隆性：
单克隆
- 保质期：
1年
- 抗体英文名：
NORRIN
- 抗体名：
诺里病NDP蛋白/早产儿视网膜病蛋白抗体
- 适应物种：
人/动物/植物
- 应用范围：
WB,ELISA等
- 浓度：
1mg/ml
- 保存条件：
-20 °
- 规格：
100ul/200ul
| 规格： | 100ul | 产品价格： | ¥1580.0 |
|---|---|---|---|
| 规格： | 200ul | 产品价格： | ¥2480.0 |
产品货号 : jlcR19062
英文名称 : NORRIN
中文名称 : 诺里病NDP蛋白/早产儿视网膜病蛋白抗体
别 名 : EVR2; Exudative vitreoretinopathy 2 (X linked); FEVR; ND; NDP; NDP_HUMAN; Norrie disease (pseudoglioma); Norrie disease protein; Norrin; Norrin precursor; X linked exudative vitreoretinopathy 2 protein; X-linked exudative vitreoretinopathy 2 protein.
研究领域 : 细胞生物 发育生物学 神经生物学 干细胞
抗体来源 : Rabbit
克隆类型 : Polyclonal
交叉反应 : Human, Mouse, Dog, Pig, Cow, Horse, Rabbit, Sheep,
产品应用 : ELISA=1:500-1000 IHC-P=1:400-800 IHC-F=1:400-800 ICC=1:100-500 IF=1:100-500 （石蜡切片需做抗原修复）
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
分 子 量 : 12kDa
细胞定位 : 分泌型蛋白
性 状 : Lyophilized or Liquid
浓 度 : 1mg/ml
免 疫 原 : KLH conjugated synthetic peptide derived from human NORRIN:51-133/133
亚 型 : IgG
纯化方法 : affinity purified by Protein A
储 存 液 : 0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol.
保存条件 : Store at -20 °C for one year. Avoid repeated freeze/thaw cycles. The lyophilized antibody is stable at room temperature for at least one month and for greater than a year when kept at -20°C. When reconstituted in sterile pH 7.4 0.01M PBS or diluent of antibody the antibody is stable for at least two weeks at 2-4 °C.
PubMed : PubMed
产品介绍 : This gene encodes a secreted protein with a cystein-knot motif that activates the Wnt/beta-catenin pathway. The protein forms disulfide-linked oligomers in the extracellular matrix. Mutations in this gene result in Norrie disease and X-linked exudative vitreoretinopathy. [provided by RefSeq, Feb 2009]
Function:
Activates the canonical Wnt signaling pathway through FZD4 and LRP5 coreceptor. Plays a central role in retinal vascularization by acting as a ligand for FZD4 that signals via stabilizing beta-catenin (CTNNB1) and activating LEF/TCF-mediated transcriptional programs. Acts in concert with TSPAN12 to activate FZD4 independently of the Wnt-dependent activation of FZD4, suggesting the existence of a Wnt-independent signaling that also promote accumulation the beta-catenin (CTNNB1). May be involved in a pathway that regulates neural cell differentiation and proliferation. Possible role in neuroectodermal cell-cell interaction.
Subcellular Location:
Secreted.
Tissue Specificity:
Expressed in the outer nuclear, inner nuclear and ganglion cell layers of the retina, and in fetal and adult brain.
DISEASE:
Defects in NDP are the cause of Norrie disease (ND) [MIM:310600]; also known as atrophia bulborum hereditaria or Episkopi blindness. ND is a recessive disorder characterized by very early childhood blindness due to degenerative and proliferative changes of the neuroretina. Approximately 50% of patients show some form of progressive mental disorder, often with psychotic features, and about one-third of patients develop sensorineural deafness in the second decade. In addition, some patients have more complex phenotypes, including growth failure and seizure. Defects in NDP are the cause of vitreoretinopathy exudative type 2 (EVR2) [MIM:305390]. EVR2 is a disorder of the retinal vasculature characterized by an abrupt cessation of growth of peripheral capillaries, leading to an avascular peripheral retina. This may lead to compensatory retinal neovascularization, which is thought to be induced by hypoxia from the initial avascular insult. New vessels are prone to leakage and rupture causing exudates and bleeding, followed by scarring, retinal detachment and blindness. Clinical features can be highly variable, even within the same family. Patients with mild forms of the disease are asymptomatic, and their only disease related abnormality is an arc of avascular retina in the extreme temporal periphery.
Similarity:
Contains 1 CTCK (C-terminal cystine knot-like) domain.
SWISS:
Q00604
Gene ID:
4693
Important Note:
This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
英文名称 : NORRIN
中文名称 : 诺里病NDP蛋白/早产儿视网膜病蛋白抗体
别 名 : EVR2; Exudative vitreoretinopathy 2 (X linked); FEVR; ND; NDP; NDP_HUMAN; Norrie disease (pseudoglioma); Norrie disease protein; Norrin; Norrin precursor; X linked exudative vitreoretinopathy 2 protein; X-linked exudative vitreoretinopathy 2 protein.
研究领域 : 细胞生物 发育生物学 神经生物学 干细胞
抗体来源 : Rabbit
克隆类型 : Polyclonal
交叉反应 : Human, Mouse, Dog, Pig, Cow, Horse, Rabbit, Sheep,
产品应用 : ELISA=1:500-1000 IHC-P=1:400-800 IHC-F=1:400-800 ICC=1:100-500 IF=1:100-500 （石蜡切片需做抗原修复）
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
分 子 量 : 12kDa
细胞定位 : 分泌型蛋白
性 状 : Lyophilized or Liquid
浓 度 : 1mg/ml
免 疫 原 : KLH conjugated synthetic peptide derived from human NORRIN:51-133/133
亚 型 : IgG
纯化方法 : affinity purified by Protein A
储 存 液 : 0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol.
保存条件 : Store at -20 °C for one year. Avoid repeated freeze/thaw cycles. The lyophilized antibody is stable at room temperature for at least one month and for greater than a year when kept at -20°C. When reconstituted in sterile pH 7.4 0.01M PBS or diluent of antibody the antibody is stable for at least two weeks at 2-4 °C.
PubMed : PubMed
产品介绍 : This gene encodes a secreted protein with a cystein-knot motif that activates the Wnt/beta-catenin pathway. The protein forms disulfide-linked oligomers in the extracellular matrix. Mutations in this gene result in Norrie disease and X-linked exudative vitreoretinopathy. [provided by RefSeq, Feb 2009]
Function:
Activates the canonical Wnt signaling pathway through FZD4 and LRP5 coreceptor. Plays a central role in retinal vascularization by acting as a ligand for FZD4 that signals via stabilizing beta-catenin (CTNNB1) and activating LEF/TCF-mediated transcriptional programs. Acts in concert with TSPAN12 to activate FZD4 independently of the Wnt-dependent activation of FZD4, suggesting the existence of a Wnt-independent signaling that also promote accumulation the beta-catenin (CTNNB1). May be involved in a pathway that regulates neural cell differentiation and proliferation. Possible role in neuroectodermal cell-cell interaction.
Subcellular Location:
Secreted.
Tissue Specificity:
Expressed in the outer nuclear, inner nuclear and ganglion cell layers of the retina, and in fetal and adult brain.
DISEASE:
Defects in NDP are the cause of Norrie disease (ND) [MIM:310600]; also known as atrophia bulborum hereditaria or Episkopi blindness. ND is a recessive disorder characterized by very early childhood blindness due to degenerative and proliferative changes of the neuroretina. Approximately 50% of patients show some form of progressive mental disorder, often with psychotic features, and about one-third of patients develop sensorineural deafness in the second decade. In addition, some patients have more complex phenotypes, including growth failure and seizure. Defects in NDP are the cause of vitreoretinopathy exudative type 2 (EVR2) [MIM:305390]. EVR2 is a disorder of the retinal vasculature characterized by an abrupt cessation of growth of peripheral capillaries, leading to an avascular peripheral retina. This may lead to compensatory retinal neovascularization, which is thought to be induced by hypoxia from the initial avascular insult. New vessels are prone to leakage and rupture causing exudates and bleeding, followed by scarring, retinal detachment and blindness. Clinical features can be highly variable, even within the same family. Patients with mild forms of the disease are asymptomatic, and their only disease related abnormality is an arc of avascular retina in the extreme temporal periphery.
Similarity:
Contains 1 CTCK (C-terminal cystine knot-like) domain.
SWISS:
Q00604
Gene ID:
4693
Important Note:
This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
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文献和实验相关实验
致病的多属于 a 、 b 、 c 群，我国 95% 以上病例为 a 群，有的地区发现 b 群和 c 群。 x 、 y 、 z 等经常在带菌者中发现，极少致病。以根据外膜蛋白抗原（型特异性）将各血清群分为若干血清型。 脑膜炎球菌的主要致病物质为荚膜、菌毛、内毒素。脑膜炎球菌通常寄居于正常人鼻咽腔，在约有 5 ～ 10% 的健康人鼻咽部带有本菌，流行期高达 20 ～ 70% ，但带菌者 90% 并不病，少数引起鼻咽炎，严重者造成菌血症，仅 1 ～ 2%
根据脑膜炎球菌表面荚膜多糖抗原的不同，将本菌分为A、B、C、D、X、Y、29E及W135九个血清群，对人类致病的多属于A、B、C群，我国95%以上病例为A群，有的地区发现B群和C群。X、Y、Z等经常在带菌者中发现，极少致病。以根据外膜蛋白抗原（型特异性）将各血清群分为若干血清型。 （二）致病性与免疫性 脑膜炎球菌的主要致病物质为荚膜、菌毛、内毒素。 本菌通常寄居于正常人鼻咽腔，在约有5～10%的健康人鼻咽部带有本菌，流行期高达20～70%，但带菌者90%并不病，少数引起鼻咽
多糖抗原的不同，将本菌分为A、B、C、D、X、Y、29E及W135九个血清群，对人类致病的多属于A、B、C群，我国95%以上病例为A群，有的地区发现B群和C群。X、Y、Z等经常在带菌者中发现，极少致病。以根据外膜蛋白抗原(型特异性)将各血清群分为若干血清型。 (二)致病性与免疫性 脑膜炎球菌的主要致病物质为荚膜、菌毛、内毒素。 本菌通常寄居于正常人鼻咽腔，在约有5～10%的健康人鼻咽部带有本菌，流行期高达20～70%，但带菌者90%并不病，少数引起鼻咽炎，严重者造成菌血症，仅1～2%
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诺里病NDP蛋白/早产儿视网膜病蛋白抗体
¥1580 - 2480
