血影蛋白A链红细胞型抗体

血影蛋白A链红细胞型抗体

规格：1mg/1ml

英文名： Spectrin (alpha + beta)

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; Eryth

分子量： 280kDa

储存液：0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glyce

克隆类型：Polyclonal

亚型：IgG

纯化方法：affinity purified by Protein A

**原：KLH conjugated synthetic peptide derived from Human Spectrin

交叉反应：Human, Mouse, Rat, Pig, Horse, Rabbit,

细胞定位：细胞浆

血影蛋白A链红细胞型抗体产品介绍：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 血影蛋白A链红细胞型抗体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]; 血影蛋白A链红细胞型抗体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.

血影蛋白A链红细胞型抗体产品应用：WB=1:100-500 ELISA=1:500-1000 IHC-P=1:100-500 IHC-F=1:100-500 ICC=1:100-500 IF=1:100-500 （石蜡切片需做抗原修复） not yet tested in other applications. optimal dilutions/concentrations should be determined by the end user.

研究领域：心血管  信号转导  

储存条件： Store at -20 °C for one year. Avoid repeated freeze/thaw cycles.

来源： Rabbit

外观： Lyophilized or Liquid

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