| 產(chǎn)品編號(hào) | bs-12351R-BF647 |
| 英文名稱 | Rabbit Anti-STRA6/BF647 Conjugated antibody |
| 中文名稱 | BF647標(biāo)記的維甲酸誘導(dǎo)蛋白6抗體 |
| 別 名 | Stimulated by retinoic acid gene 6 protein homolog; STRA6_HUMAN. |
| 規(guī)格價(jià)格 | 100ul/2980元 購(gòu)買 大包裝/詢價(jià) |
| 說(shuō) 明 書 | 100ul |
| 研究領(lǐng)域 | 腫瘤 細(xì)胞生物 信號(hào)轉(zhuǎn)導(dǎo) 細(xì)胞類型標(biāo)志物 新陳代謝 |
| 抗體來(lái)源 | Rabbit |
| 克隆類型 | Polyclonal |
| 交叉反應(yīng) | (predicted: Human, Mouse, Rat, Dog, Horse, Rabbit, ) |
| 產(chǎn)品應(yīng)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 |
| 性 狀 | Lyophilized or Liquid |
| 濃 度 | 1mg/ml |
| 免 疫 原 | KLH conjugated synthetic peptide derived from Human STRA6 |
| 亞 型 | IgG |
| 純化方法 | affinity purified by Protein A |
| 儲(chǔ) 存 液 | 0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol. |
| 保存條件 | 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. |
| 產(chǎn)品介紹 |
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 applications. |
