FITC标记的X连锁先天性视网膜劈裂XLRS1蛋白抗体
产品名称： FITC标记的X连锁先天性视网膜劈裂XLRS1蛋白抗体
英文名称： Anti-RS1/FITC
产品编号： HZ-18863R-FITC
产品价格： null
产品产地： 中国/上海
品牌商标： HZbscience
更新时间： 2023-08-17T10:24:20
使用范围： ICC=1:50-200 IF=1:50-200
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Rabbit Anti-RS1/FITC Conjugated antibody
FITC标记的X连锁先天性视网膜劈裂XLRS1蛋白抗体
| 英文名称 | Anti-RS1/FITC |
| 中文名称 | FITC标记的X连锁先天性视网膜劈裂XLRS1蛋白抗体 |
| 别 名 | Retinoschisin; RS1; X-linked juvenile retinoschisis protein; XLRS1_HUMAN. |
| 规格价格 | 100ul/2980元 购买 大包装/询价 |
| 说 明 书 | 100ul |
| 研究领域 | 免疫学 神经生物学 |
| 抗体来源 | Rabbit |
| 克隆类型 | Polyclonal |
| 交叉反应 | Human, Mouse, Rat, Chicken, Dog, Cow, Horse, Sheep, |
| 产品应用 | ICC=1:50-200 IF=1:50-200 not yet tested in other applications. optimal dilutions/concentrations should be determined by the end user. |
| 分 子 量 | 23kDa |
| 性 状 | Lyophilized or Liquid |
| 浓 度 | 1mg/ml |
| 免 疫 原 | KLH conjugated synthetic peptide derived from human RS1 |
| 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. |
| 产品介绍 | background: This gene encodes an extracellular protein that plays a crucial role in the cellular organization of the retina. The encoded protein is assembled and secreted from photoreceptors and bipolar cells as a homo-oligomeric protein complex. Mutations in this gene are responsible for X-linked retinoschisis, a common, early-onset macular degeneration in males that results in a splitting of the inner layers of the retina and severe loss in vision. [provided by RefSeq, Oct 2008] Function: May be active in cell adhesion processes during retinal development. Subcellular Location: Secreted. Tissue Specificity: Restricted to the retina (at protein level). At the mRNA level, detected only within the photoreceptor cell layer, most prominently within the inner segments of the photoreceptors. Undetectable in the inner plexiform layers and the inner nuclear layer. At the protein level, found in the inner segment of the photoreceptors, the inner nuclear layer, the inner plexiform layer and the ganglion cell layer. At the macula, expressed in both the outer and inner nuclear layers and in the inner plexiform layer (at protein level). DISEASE: Defects in RS1 are the cause of retinoschisis juvenile X-linked type 1 (XLRS1) [MIM:312700]. A vitreo-retinal dystrophy characterized by macular pathology and by splitting of the superficial layer of the retina. Macular changes are present in almost all cases. In the fundi, radially oriented intraretinal foveomacular cysts are seen in a spoke-wheel configuration, with the absence of foveal reflex in most cases. In addition, approximately half of cases have bilateral peripheral retinoschisis in the inferotemporal part of the retina. Aside from the typical fundus appearance, strabismus, nystagmus, axial hyperopia, defective color vision and foveal ectopy can be present. The most important complications are vitreous hemorrhage, retinal detachment, and neovascular glaucoma. Similarity: Contains 1 F5/8 type C domain. Database links: Entrez Gene: 6247 Human Entrez Gene: 20147 Mouse Omim: 300839 Human SwissProt: O15537 Human SwissProt: Q9Z1L4 Mouse Unigene: 715725 Human Important Note: This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications |
该基因编码的胞外蛋白在视网膜的细胞组织中起着至关重要的作用。编码的蛋白质从感光体和双极细胞组装并分泌为同源寡聚蛋白复合物。该基因的突变导致X连锁视网膜劈裂，一种常见的男性早发性黄斑变性，导致视网膜内层分裂和视力严重丧失。[ RefSeq，OCT 2008提供]