FITC标记的维甲酸诱导蛋白6抗体
产品名称： FITC标记的维甲酸诱导蛋白6抗体
英文名称： Anti-STRA6/FITC
产品编号： HZ-12351R-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-STRA6/FITC Conjugated antibody
FITC标记的维甲酸诱导蛋白6抗体
| 英文名称 | Anti-STRA6/FITC |
| 中文名称 | FITC标记的维甲酸诱导蛋白6抗体 |
| 别 名 | Stimulated by retinoic acid gene 6 protein homolog; STRA6_HUMAN. |
| 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 信号转导 细胞类型标志物 新陈代谢 |
| 抗体来源 | Rabbit |
| 克隆类型 | Polyclonal |
| 交叉反应 | Human, Mouse, Rat, Dog, 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 | 74kDa |
| 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 STRA6 |
| 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: STRA6 is a 667 amino acid, multi-pass cell membrane protein. Stra6 functions as a cell-surface receptor for the complex retinol-retinol binding protein (RBP/RBP4). Ultimately increasing cellular retinol uptake from the retinol-RBP complex, Stra6 removes retinol from RBP/RPB4 and transports it across the plasma membrane, where it is metabolized. Stra6 is broadly expressed, with 4 named isoforms that exist as a result of alternative splicing events. Mutations in the gene encoding Stra6 cause Matthew-Wood Syndrome, also known as Spear Syndrome. This syndrome is characterized by anophtalmia, mild facial dysmorphism and malformations of the heart, lung and diaphragm. The Stra6 gene maps to chromosome 15q24.1. Function: Stra6 functions as a high-affinity cell-surface receptor for the complex retinol-retinol binding protein (RBP/RBP4). Stra6 is expressed in the extraembryonic endoderm and expression is also known to be induced by Wnt1. Defects in STRA6 are known to cause of syndromic microphthalmia type 9 (MCOPS9) also known as clinical anophthalmia with mild facial dysmorphism and variable malformations of the lung, heart, and diaphragm or anophthalmia/microphthalmia and pulmonary hypoplasia or Spear syndrome or Matthew-Wood syndrome or pulmonary agenesis, microphthalmia, and diaphragmatic defect. Subcellular Location: Cell membrane; multi-pass membrane protein. Tissue Specificity: Broad expression. In adult eye expressed in sclera, retina, retinal pigment epithelium, and trabecular meshwork but not in choroid and iris. DISEASE: Defects in STRA6 are the cause of microphthalmia syndromic type 9 (MCOPS9) [MIM:601186]; also called Matthew-Wood syndrome or Spear syndrome. Microphthalmia is a clinically heterogeneous disorder of eye formation, ranging from small size of a single eye to complete bilateral absence of ocular tissues (anophthalmia). In many cases, microphthalmia/anophthalmia occurs in association with syndromes that include non-ocular abnormalities. MCOPS9 is a rare clinical entity including as main characteristics anophthalmia or severe microphthalmia, and pulmonary hypoplasia or aplasia. Note=Mutations in STRA6 may be a cause of isolated colobomatous microphthalmia, a disorder of the eye characterized by an abnormally small ocular globe. Database links: Entrez Gene: 64220 Human Omim: 610745 Human SwissProt: Q9BX79 Human Unigene: 24553 Human Important Note: This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic application |
STAR6是一种667氨基酸多通道细胞膜蛋白。STAR6作为复杂的视黄醇-视黄醇结合蛋白（Rbp/RBP4）的细胞表面受体。最终增加视黄醇-RBP复合物对细胞视黄醇的摄取，Stra6将视黄醇从RBP/RPB4中去除，并将其跨质膜运输，并在质膜中代谢。STAR6广泛表达，有4个命名的异构体，作为替代剪接事件的结果存在。STAR6基因突变引起马修·伍德综合征，也称为SPARE综合征。该综合征以无症状、轻度面部畸形和心、肺、膈畸形为特征。STAR6基因定位于染色体15q24.1。