Spectrin alpha chain Recombinant Rabbit mAb (一抗) - WB,Flow-Cyt,ICC/IF | Bioss

bsm-62985R

重组兔单抗

Spectrin alpha chain Recombinant Rabbit mAb

血影蛋白A链红细胞型重组兔单抗

EL2; HPP; HS3; SPH3; SPTA; EL3; HS2; HSPTB1; SPH2; Spna-1; Spna1; ha; ihj; nmf4; sph; D330027P03Rik; Gm1301; Spnb-1; Spnb1; ja; jaundiced; mKIAA4219; SPTA1_HUMAN; SPTA1; Erythroid alpha-spectrin; SPTB1_HUMAN; SPTB; Beta-I spectrin; SPTB1; SPTA1_MOUSE; SPTB1_MOUSE; spectrin alpha, erythrocytic 1; spectrin, alpha, erythrocytic 1 (elliptocytosis 2); elliptocytosis 2

Rabbit

A synthesized peptide derived from human Spectrin alpha chain: 2300-2419/2419

IgG

Liquid

affinity purified by Protein A

Recombinant

3C10

280 kDa

300 kDa

10mM phosphate buffered saline(pH 7.4) with 150mM sodium chloride, 0.05% BSA, 0.02% Proclin300 and 50% glycerol.

Store at 4℃ for short term. Store at -20℃ for long term. Avoid repeated freeze/thaw cycles.

This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.

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.

SPTA1

Spectrin alpha chain, erythrocytic 1

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.

Cytoplasm, cytoskeleton. Cytoplasm, cell cortex.

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.

Belongs to the spectrin family.
Contains 3 EF-hand domains.
Contains 1 SH3 domain.
Contains 21 spectrin repeats.

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.