EL2; HPP; HS3; SPH3; SPTA

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

Polyclonal

Rabbit

The antibody detects endogenous levels of total SPTA1 protein.

Antigen affinity purification

Rabbit IgG in pH7.4 PBS, 0.05% NaN3, 40% Glycerol.

0.6mg/ml (lot specific)

Store at -20 degree C

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.

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.

Total protein Ab

Immunohistochemistry (IHC), Western Blot (WB)

Western Blot: 1:200-1000
Immunohistochemistry: 1: 20-100

Gel: 6%SDS-PAGE Lysate: 40 ug, Lane 1-2: Human heart tissue￡?Mouse heart tissue lysates, Primary antibody:SPTA1 antibody at dilution 1/250 dilution, Secondary antibody: Goat anti rabbit IgG at 1/8000 dilution, Exposure time: 5 seconds

The image on the left is immunohistochemistry of paraffin-embedded Human thyroid cancer tissue using SPTA1 Antibody at dilution 1/25, on the right is treated with synthetic peptide. (Original magnification: x200)

The image on the left is immunohistochemistry of paraffin-embedded Human tonsil tissue using SPTA1 Antibody at dilution 1/25, on the right is treated with synthetic peptide. (Original magnification: x200)

279,674 Da

spectrin alpha, erythrocytic 1

spectrin alpha chain, erythrocytic 1

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: actin cytoskeleton; cytosol

Molecular Function: actin filament binding; protein binding; Ras guanyl-nucleotide exchange factor activity; structural constituent of cytoskeleton

Biological Process: actin filament organization; axon guidance; ER to Golgi vesicle-mediated transport; MAPKKK cascade

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

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