Spectrin alpha chain non-erythrocytic 1; fodrin alpha chain

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

Monoclonal

IgG1

2A5A7H8

Phosphate-buffered solution, pH 7.2.

Store lyophilized antibody at -20 degree C. After reconstitution, store at 4 degree C. May be sub-aliquoted and stored at -20 degree C to avoid multiple freeze thaw cycles.

Small volumes of anti-SPTAN1 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.

The?spectrins?are a family of widely distributed?cytoskeletal?proteins which are involved in?actin?crosslinking. Spectrin alpha-chain heterodimerizes with tissue-specific beta-chains.?It is specifically expressed in nonerythrocytic cells. The protein has been implicated in other cellular functions including DNA repair and cell cycle regulation. Mutations in the gene are the cause of early infantile epileptic encephalopathy. Fodrin, which seems to be involved in secretion, interacts with calmodulin in a calcium-dependent manner and is thus a candidate for the calcium-dependent movement of the cytoskeleton at the membrane.

Western Blot (WB), ELISA (EIA)

Each lot of this antibody is quality control tested by Western blotting. To reconstitute, add 100 ul of ddH2O to the tube containing antibody and mix to dissolve. For Western blotting, the suggested dilution is 1:1000 after reconsitution. It is recommended that the reagent be titrated for optimal performance for each application.

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: axon guidance; regulation of cell shape; 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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