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Product Name Spectrin alpha chain, non-erythrocytic 1 Chinese Name 血影蛋白A链非红细胞型抗体 Alias alpha-II spectrin; SPTN1_HUMAN; Alpha Fodrin; Spectrin-Alpha; SPEC; Spectrin, alpha, non-erythrocy tic 1 variant; Spectrin alpha chain, non-erythrocytic 1; Alpha-II spectrin; Fodrin alpha chain; Spectrin, non-erythroid alpha subunit; SPTAN1; SPTA2; spectrin, non-erythroid alpha chain; alpha-fodrin; spectrin, non-erythroid alpha subunit; spectrin alpha chain, non-erythrocytic 1; EIEE5; NEAS; Spectrin α II; Spectrin α-II; Spectrin αII; Spectrin-α II. Research Area Cardiovascular Cell biology Immunogen Species Rabbit Clonality Polyclonal React Species (predicted: Human, Mouse, Rat, Dog, Cow, ) Applications ELISA=1:5000-10000 IHC-P=1:100-500 IHC-F=1:100-500 ICC=1:100-500 IF=1:100-500 (Paraffin sections need antigen repair)
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.Theoretical molecular weight 272kDa Cellular localization cytoplasmic The cell membrane Form Liquid Concentration 1mg/ml immunogen KLH conjugated synthetic peptide derived from human Spectrin alpha chain, non-erythrocytic 1: 30-110/2472 Lsotype IgG Purification affinity purified by Protein A Buffer Solution 0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol. Storage Shipped at 4℃. Store at -20 °C for one year. Avoid repeated freeze/thaw cycles. Attention This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications. PubMed PubMed Product Detail Spectrins are a family of filamentous cytoskeletal proteins that function as essential scaffold proteins that stabilize the plasma membrane and organize intracellular organelles. Spectrins are composed of alpha and beta dimers that associate to form tetramers linked in a head-to-head arrangement. This gene encodes an alpha spectrin that is specifically expressed in nonerythrocytic cells. The encoded protein has been implicated in other cellular functions including DNA repair and cell cycle regulation. Mutations in this gene are the cause of early infantile epileptic encephalopathy-5. Alternate splicing results in multiple transcript variants.[provided by RefSeq, Sep 2010]
Function:
Fodrin, which seems to be involved in secretion, interacts with calmodulin in a calcium-dependent manner and is thus candidate for the calcium-dependent movement of the cytoskeleton at the membrane.
Subunit:
Like erythrocyte spectrin, the spectrin-like proteins are capable of forming dimers which can further associate to tetramers. Interacts with isoform 1 of ACP1. Interacts with CALM and EMD. Interacts (via C-terminal spectrin repeats) with TRPC4. Identified in a complex with ACTN4, CASK, IQGAP1, MAGI2, NPHS1 and SPTBN1.
Subcellular Location:
Cytoplasm, cytoskeleton. Cytoplasm, cell cortex. Note=Expressed along the cell membrane in podocytes and presumptive tubule cells during glomerulogenesis and is expressed along lateral cell margins in tubule cells.
Post-translational modifications:
Phosphorylation of Tyr-1176 decreases sensitivity to cleavage by calpain in vitro.
DISEASE:
Defects in SPTAN1 are the cause of epileptic encephalopathy early infantile type 5 (EIEE5) [MIM:613477]. EIEE5 is a disorder characterized by seizures associated with hypsarrhythmia profound mental retardation with lack of visual attention and speech development, as well as spastic quadriplegia.
Similarity:
Belongs to the spectrin family.
Contains 3 EF-hand domains.
Contains 1 SH3 domain.
Contains 23 spectrin repeats.
SWISS:
Q13813
Gene ID:
6709
Database links:
Gene ID: 6709 HumanSwissProt: Q13813 Human
血影蛋白(spectrin)又称收缩蛋白,是红The cell membrane骨架的主要成份,参与了Cytoskeleton结构的形成。但不是红The cell membrane蛋白的成份,约占膜提取蛋白的30%。血影蛋白属是类似肌球蛋白的膜的外在性蛋白的一种。Spectrin α是膜里侧结构蛋白质之一,除起支持双层脂质外,还有保持红细胞外形的作用,Spectrin-α占里侧结构蛋白之60—70%。
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