FITC标记的视网膜色素上皮细胞特异性蛋白65抗体
产品名称： FITC标记的视网膜色素上皮细胞特异性蛋白65抗体
英文名称： Anti-RPE65/FITC
产品编号： HZ-9575R-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-RPE65/FITC Conjugated antibody
FITC标记的视网膜色素上皮细胞特异性蛋白65抗体
| 产品编号 | bs-9575R-FITC |
| 英文名称 | Anti-RPE65/FITC |
| 中文名称 | FITC标记的视网膜色素上皮细胞特异性蛋白65抗体 |
| 别 名 | All-trans-retinyl-palmitate hydrolase; LCA 2; LCA2; Leber congenital amaurosis; mRPE 65; mRPE65; p63; rd 12; rd12; Retinal pigment epithelium specific 61 kDa protein; Retinal pigment epithelium specific 65 kDa protein; Retinal pigment epithelium specific protein; Retinal pigment epithelium specific protein 65kDa; Retinal pigment epithelium-specific 65 kDa protein; Retinitis pigmentosa 20; Retinoid isomerohydrolase; RP 20; RP20; RPE 65; RPE65; RPE65_HUMAN; sRPE 65; sRPE65. |
| 规格价格 | 100ul/2980元 购买 大包装/询价 |
| 说 明 书 | 100ul |
| 研究领域 | 肿瘤 细胞生物 神经生物学 信号转导 |
| 抗体来源 | Rabbit |
| 克隆类型 | Polyclonal |
| 交叉反应 | Human, Mouse, Rat, Chicken, Dog, Cow, Horse, Rabbit, |
| 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 | 59kDa |
| 细胞定位 | 细胞膜 |
| 性 状 | Lyophilized or Liquid |
| 浓 度 | 1mg/ml |
| 免 疫 原 | KLH conjugated synthetic peptide derived from human RPE65 |
| 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: The retinal pigment epithelium (RPE) is a monolayer simple epithelium in proximity to the outer surface of the retinal photoreceptor cells. Retinal pigment epithelium-specific protein (RPE65) is a 65 kDa protein belonging to the β-carotene dioxygenase family. This protein is important in 11-cis retinal production as well as in visual pigment regeneration. RPE65 is attached to the membrane by a lipid anchor when palmitoylated (membrane form) and soluble when unpalmitoylated. The soluble form of the protein binds vitamin A. Defects in RPE65 causes autosomal dominant retinitis pigmentosa and/or Leber congenital amaurosis type 2. Function: Plays important roles in the production of 11-cis retinal and in visual pigment regeneration. The soluble form binds vitamin A (all-trans-retinol), making it available for LRAT processing to all-trans-retinyl ester. The membrane form, palmitoylated by LRAT, binds all-trans-retinyl esters, making them available for IMH (isomerohydrolase) processing to all-cis-retinol. The soluble form is regenerated by transferring its palmitoyl groups onto 11-cis-retinol, a reaction catalyzed by LRAT. The enzymatic activity is linearly dependent of the expression levels and membrane association. Subunit: Interacts with MYO7A; this mediates light-dependent intracellular transport of RPE65. Subcellular Location: Cytoplasm. Cell membrane. Attached to the membrane by a lipid anchor when palmitoylated (membrane form), soluble when unpalmitoylated. Tissue Specificity: Retinal pigment epithelium specific. Post-translational modifications: Palmitoylation by LRAT regulates ligand binding specificity; the palmitoylated form (membrane form) specifically binds all-trans-retinyl-palmitate, while the soluble unpalmitoylated form binds all-trans-retinol (vitamin A). DISEASE: Defects in RPE65 are the cause of Leber congenital amaurosis type 2 (LCA2) [MIM:204100]. LCA designates a clinically and genetically heterogeneous group of childhood retinal degenerations, generally inherited in an autosomal recessive manner. Affected infants have little or no retinal photoreceptor function as tested by electroretinography. LCA represents the most common genetic cause of congenital visual impairment in infants and children. Similarity: Belongs to the carotenoid oxygenase family. Database links: Entrez Gene: 6121 Human Entrez Gene: 19892 Mouse Entrez Gene: 89826 Rat Omim: 180069 Human SwissProt: Q9YGX2 Chicken SwissProt: Q28175 Cow SwissProt: Q16518 Human SwissProt: Q91ZQ5 Mouse SwissProt: O70276 Rat Unigene: 2133 Human Unigene: 131708 Mouse Unigene: 76724 Rat Important Note: This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications |
视网膜色素上皮（RPE）是在视网膜感光细胞的外表面附近的单层简单上皮。视网膜色素上皮特异性蛋白（RPE65）是β-胡萝卜素双加氧酶家族的65 kDa蛋白。这种蛋白质在11顺式视网膜生产中以及视觉色素再生中是重要的。当棕榈酰化（膜形式）和当未棕榈酰化时可溶时，RPE65通过脂质锚附着到膜上。蛋白质的可溶性形式结合维生素A。RPE65中的缺陷导致常染色体显性遗传视网膜色素变性和/或莱伯先天性黑蒙2型。