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Gene : |
SPTBN2 |
Gene ID : |
6712 |
Uniprot ID : |
O15020 |
Alternative Name : |
Spectrin beta chain, non-erythrocytic 2, Beta-III spectrin, Spinocerebellar ataxia 5 protein |
Format : |
Purified |
Amount : |
100 µg |
Clone name : |
ABM5A16 |
Isotype : |
Mouse IgG2a, kappa |
Immunogen Information : |
A partial length recombinant protein (a.a 1280-1450) of Spectrin beta 3 was used as the immunogen for this antibody. |
Beta-III spectrin is a protein encoded by the gene SPTBN2, spans 2390 amino acids and consists of an amino-terminal actin binding domain (ABD), a central region containing seventeen spectrin repeat domains, and a carboxy-terminal pleckstrin homology domain. It is critical for the correct development and maintenance of Purkinje cell dendritic structure. Beta-III spectrin is expressed predominantly in the brain and is enriched in cerebellar Purkinje cells. In addition, Beta-III spectrin participates in endomembrane trafficking through its interaction with the actin related protein, ARP1. The functional unit of Beta-III spectrin is considered to be a tetrameric complex composed of two Beta-spectrin subunits and two Alpha-II-spectrin subunits. Mutations in the gene encoding Beta-III spectrin give rise to spinocerebellar ataxia type 5, a neurodegenerative disease characterized by progressive thinning of the molecular layer, loss of Purkinje cells and increasing motor deficits.
WB: 0.1-0.5 µg/ml, FACS: 2-4 µg/10^6