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Spectrin (alpha + beta) Rabbit pAb (bs-10190R)  
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產品編號 bs-10190R
英文名稱 Spectrin (alpha + beta) Rabbit pAb
中文名稱 血影蛋白A鏈紅細胞型抗體
別    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; Sp  
研究領域 心血管  信號轉導  
抗體來源 Rabbit
克隆類型 Polyclonal
交叉反應 Mouse,Rat (predicted: Human,Rabbit,Pig,Horse)
產品應用 WB=1:500-2000
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
理論分子量 280 kDa
檢測分子量
細胞定位 細胞漿 
性    狀 Liquid
濃    度 1mg/ml
免 疫 原 KLH conjugated synthetic peptide derived from Human Spectrin alpha chain, erythrocytic 1: 2151-2350/2419 
亞    型 IgG
純化方法 affinity purified by Protein A
緩 沖 液 0.01M TBS (pH7.4) with 1% BSA, 0.02% Proclin300 and 50% Glycerol.
保存條件 Shipped at 4℃. Store at -20℃ for one year. Avoid repeated freeze/thaw cycles.
注意事項 This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
PubMed PubMed
產品介紹 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.

SWISS:
P11277

Gene ID:
6710

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



產品圖片
Sample: Blood cell(Mouse) Lysate at 40 ug Primary: Anti-Spectrin (alpha + beta) (bs-10190R) at 1/1000 dilution Secondary: IRDye800CW Goat Anti-Rabbit IgG at 1/20000 dilution Predicted band size: 280 kD Observed band size: 280 kD
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