Spectrin alpha chain, erythrocytic 1; Spectrin beta chain, erythrocytic; spectrin, alpha, erythrocytic 1(elliptocytosis 2); spectrin, beta, erythrocytic; EL2; HPP; HS3; SPH3; SPTA; EL3; HS2; SPH2; HSPTB1; Erythroid alpha-spectrin

For Research Use Only. Not for use in diagnostic procedures.

Monoclonal

IgG1

Spe 1/2

Mouse

Purified by the goat anti-mouse IgG affinity chromatography

Lyophilized from 1.2% sodium acetate, with 2 mg BSA and 0.01mg NaN3 as a preservative

At -20 degree C for one year. After reconstitution, at 4 degree C for one month. It can also be aliquotted and stored frozen at -20 degree C for a longer time.

Manufactured in an ISO 13485:2003 and EN ISO 13485:2012 Certified Laboratory.

Small volumes of anti-SPTA1 antibody vial(s) may occasionally become entrapped in the seal of the product vial during shipment and storage. If necessary, briefly centrifuge the vial on a tabletop centrifuge to dislodge any liquid in the container`s cap. Certain products may require to ship with dry ice and additional dry ice fee may apply.

Description: Mouse IgG monoclonal antibody for Spectrin(alpha and beta), spectrin, alpha, erythrocytic 1 (elliptocytosis 2), spectrin, beta, erythrocytic (SPTA1, SPTB) detection. Tested with WB in Human. No cross reactivity with other proteins. Uniprot ID: P02549
Background: Spectrin, the predominant component of the membrane skeleton of the red cell, is essential in determining the properties of the membrane including its shape and deformability. It consists of 2 nonidentical subunits, alpha and beta. Spectrin is present in the red cell membrane in a tetrameric or possibly higher polymeric form through head-to-head self-association of heterodimers that are linked by actin polymers and protein 4.1 to form a 2-dimensional network. Non-erythroid spectrin gene is mapped to human chromosome 2. Spectrin mutations cause spinocerebellar ataxia type 5.

Western Blot (WB)

At 2-4ug/ml with the appropriate system to detect spectrin in cells and tissues.
Other applications have not been tested. Optimal dilutions should be determined by end user.

279,674 Da

spectrin, alpha, erythrocytic 1

spectrin alpha chain, erythrocytic 1; alpha-I spectrin; elliptocytosis 2; erythroid alpha-spectrin; spectrin alpha chain, erythrocyte; spectrin, alpha, erythrocytic 1 (elliptocytosis 2)

Spectrin alpha chain, erythrocytic 1

SPTA??[Similar Products]

SPTA1_HUMAN

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]

SPTA1: 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. Defects in SPTA1 are the cause of elliptocytosis type 2 (EL2). 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). 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); 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. 2 isoforms of the human protein are produced by alternative splicing.

Protein type: Cytoskeletal; Motility/polarity/chemotaxis

Chromosomal Location of Human Ortholog: 1q21

Cellular Component: spectrin; cytosol; actin cytoskeleton

Molecular Function: actin filament binding; protein binding; structural constituent of cytoskeleton; protein heterodimerization activity; calcium ion binding

Biological Process: regulation of cell shape; axon guidance; positive regulation of protein binding; plasma membrane organization and biogenesis; actin filament capping; actin filament organization; positive regulation of T cell proliferation; hemopoiesis; lymphocyte homeostasis; porphyrin biosynthetic process

Disease: Elliptocytosis 2; Pyropoikilocytosis, Hereditary; Spherocytosis, Type 3

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