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Rabbit Anti-Spectrin alpha chain, non-erythrocytic 1/BF647 Conjugated antibody (bs-0381R-BF647)
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說 明 書: 100ul  
100ul/2980.00元
大包裝/詢價
產(chǎn)品編號 bs-0381R-BF647
英文名稱 Rabbit Anti-Spectrin alpha chain, non-erythrocytic 1/BF647 Conjugated antibody
中文名稱 BF647標(biāo)記的血影蛋白A鏈非紅細(xì)胞型/收縮蛋白/Spectrin α抗體
別    名 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.  
規(guī)格價格 100ul/2980元 購買        大包裝/詢價
說 明 書 100ul  
研究領(lǐng)域 心血管  細(xì)胞生物  
抗體來源 Rabbit
克隆類型 Polyclonal
交叉反應(yīng) (predicted: Human, Mouse, Rat, Dog, Cow, )
產(chǎn)品應(yīng)用 ICC=1:50-200 IF=1:50-200 
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
分 子 量 272kDa
性    狀 Lyophilized or Liquid
濃    度 1mg/ml
免 疫 原 KLH conjugated synthetic peptide derived from human Spectrin alpha chain, non-erythrocytic 1 (20-65aa)
亞    型 IgG
純化方法 affinity purified by Protein A
儲 存 液 0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol.
保存條件 Store at -20 °C for one year. Avoid repeated freeze/thaw cycles. The lyophilized antibody is stable at room temperature for at least one month and for greater than a year when kept at -20°C. When reconstituted in sterile pH 7.4 0.01M PBS or diluent of antibody the antibody is stable for at least two weeks at 2-4 °C.
產(chǎn)品介紹 background:
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.

Database links:
Gene ID: 6709 Human

SwissProt: Q13813 Human



Important Note:
This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.

血影蛋白(spectrin)又稱收縮蛋白,是紅細(xì)胞膜骨架的主要成份,參與了細(xì)胞骨架結(jié)構(gòu)的形成。但不是紅細(xì)胞膜蛋白的成份,約占膜提取蛋白的30%。血影蛋白屬是類似肌球蛋白的膜的外在性蛋白的一種。Spectrin α是膜里側(cè)結(jié)構(gòu)蛋白質(zhì)之一,除起支持雙層脂質(zhì)外,還有保持紅細(xì)胞外形的作用,Spectrin-α占里側(cè)結(jié)構(gòu)蛋白之60—70%。
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