FITC标记的血影蛋白A链红细胞型抗体
产品名称： FITC标记的血影蛋白A链红细胞型抗体
英文名称： Anti-Spectrin (alpha + beta)/FITC
产品编号： HZ-10190R-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-Spectrin (alpha + beta)/FITC Conjugated antibody
FITC标记的血影蛋白A链红细胞型抗体
| 英文名称 | Anti-Spectrin (alpha + beta)/FITC |
| 中文名称 | FITC标记的血影蛋白A链红细胞型抗体 |
| 别 名 | Spectrin alpha chain, erythrocytic 1; Alpha I spectrin; EL 2; EL2; Elliptocytosis 2; Elliptocytosis2; Erythrocyte alpha spectrin; erythrocyte; Erythroid alpha spectrin; Erythroid alpha-spectrin; Erythroid spectrin alpha; HPP; HS3; Spectrin alpha chain; Spectrin alpha chain erythrocyte; Spectrin alpha erythrocytic 1; SPH3; SPTA 1; SPTA; SPTA1; SPTA1_HUMAN; Spectrin alpha chain, erythrocytic 1. |
| 规格价格 | 100ul/2980元 购买 大包装/询价 |
| 说 明 书 | 100ul |
| 研究领域 | 心血管 信号转导 |
| 抗体来源 | Rabbit |
| 克隆类型 | Polyclonal |
| 交叉反应 | Human, Mouse, Rat, Pig, 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 | 280kDa |
| 性 状 | Lyophilized or Liquid |
| 浓 度 | 1mg/ml |
| 免 疫 原 | KLH conjugated synthetic peptide derived from Human Spectrin alpha chain, erythrocytic 1 |
| 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: Spectrin is an actin crosslinking and molecular scaffold protein that links the plasma membrane to the actin cytoskeleton, and functions in the determination of cell shape, arrangement of transmembrane proteins, and organization of organelles. It is a tetramer made up of alpha-beta dimers linked in a head-to-head arrangement. This gene is one member of a family of alpha-spectrin genes. The encoded protein is primarily composed of 22 spectrin repeats which are involved in dimer formation. It forms weaker tetramer interactions than non-erythrocytic alpha spectrin, which may increase the plasma membrane elasticity and deformability of red blood cells. Mutations in this gene result in a variety of hereditary red blood cell disorders, including elliptocytosis type 2, pyropoikilocytosis, and spherocytic hemolytic anemia. [provided by RefSeq, Jul 2008]. Function: Spectrin is the major constituent of the cytoskeletal network underlying the erythrocyte plasma membrane. It associates with band 4.1 and actin to form the cytoskeletal superstructure of the erythrocyte plasma membrane. Subunit: Composed of non-homologous chains, alpha and beta, which aggregate side-to-side in an antiparallel fashion to form dimers, tetramers, and higher polymers. Interacts with FASLG. Subcellular Location: Cytoplasm, cytoskeleton. Cytoplasm, cell cortex. DISEASE: Defects in SPTA1 are the cause of elliptocytosis type 2 (EL2) [MIM:130600]. EL2 is a Rhesus-unlinked form of hereditary elliptocytosis, a genetically heterogeneous, autosomal dominant hematologic disorder. It is characterized by variable hemolytic anemia and elliptical or oval red cell shape. Defects in SPTA1 are a cause of hereditary pyropoikilocytosis (HPP) [MIM:266140]. HPP is an autosomal recessive disorder characterized by hemolytic anemia, microspherocytosis, poikilocytosis, and an unusual thermal sensitivity of red cells. Defects in SPTA1 are the cause of spherocytosis type 3 (SPH3) [MIM:270970]; also known as hereditary spherocytosis type 3 (HS3). Spherocytosis is a hematologic disorder leading to chronic hemolytic anemia and characterized by numerous abnormally shaped erythrocytes which are generally spheroidal. SPH3 is characterized by severe hemolytic anemia. Inheritance is autosomal recessive. Similarity: Belongs to the spectrin family. Contains 3 EF-hand domains. Contains 1 SH3 domain. Contains 21 spectrin repeats. Database links: Entrez Gene: 6708 Human Entrez Gene: 6710 Human Entrez Gene: 20739 Mouse Entrez Gene: 20741 Mouse Omim: 182860 Human Omim: 182870 Human SwissProt: P02549 Human SwissProt: P11277 Human SwissProt: P08032 Mouse SwissProt: P15508 Mouse Unigene: 119825 Human Unigene: 417303 Human Important Note: This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications |
.in是一种肌动蛋白交联和分子支架蛋白，将细胞膜与肌动蛋白细胞骨架相连，在细胞形态、跨膜蛋白排列和细胞器组织中起作用。它是由α-β二聚体组成的四聚体，从头到头排列连接。这个基因是α谱基因家族的成员之一。编码的蛋白质主要由22个参与二聚体形成的血影蛋白重复组成。它形成比非红细胞α光谱更弱的四聚体相互作用，这可能增加红细胞质膜的弹性和变形能力。该基因的突变导致多种遗传性红细胞疾病，包括椭圆形细胞增多症2型、嗜酸性粒细胞增多症和球形溶血性贫血。[ RefSeq，JUL 2008 ]提供。