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    ACVRL1 Rabbit pAb (bs-21783R)  
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    產(chǎn)品編號 bs-21783R
    英文名稱(chēng) ACVRL1 Rabbit pAb
    中文名稱(chēng) 激活素受體樣激酶1抗體
    別    名 Activin A receptor; Activin A receptor type II like 1; Activin receptor like kinase 1; Activin receptor-like kinase 1; ACVL1_HUMAN; ACVRL1; ACVRLK1; ALK-1; ALK1; HHT; HHT2; ORW2; Osler Rendu Weber syndrome 2; Serine/threonine protein kinase receptor R3; Serine/threonine-protein kinase receptor R3; SKR3; TGF B superfamily receptor type I; TGF-B superfamily receptor type I; TSR-I; TSR1.  
    研究領(lǐng)域 心血管  細胞生物  免疫學(xué)  信號轉導  轉錄調節因子  激酶和磷酸酶  細胞表面分子  
    抗體來(lái)源 Rabbit
    克隆類(lèi)型 Polyclonal
    克 隆 號
    交叉反應 Mouse (predicted: Human,Rat)
    產(chǎn)品應用 WB=1:500-2000
    not yet tested in other applications.
    optimal dilutions/concentrations should be determined by the end user.
    理論分子量 53 kDa
    檢測分子量
    細胞定位 細胞膜 
    性    狀 Liquid
    濃    度 1mg/ml
    免 疫 原 KLH conjugated synthetic peptide derived from human ACVRL1 : 22-118/503 <Extracellular>
    亞    型 IgG
    純化方法 affinity purified by Protein A
    緩 沖 液 0.01M TBS (pH7.4) with 1% BSA, 0.02% Proclin300 and 50% Glycerol.
    保存條件 Shipped at 4℃. Store at -20℃ for one year. Avoid repeated freeze/thaw cycles.
    注意事項 This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
    PubMed PubMed
    產(chǎn)品介紹 On ligand binding, forms a receptor complex consisting of two type II and two type I transmembrane serine/threonine kinases. Type II receptors phosphorylate and activate type I receptors which autophosphorylate, then bind and activate SMAD transcriptional regulators. Receptor for TGF-beta. May bind activin as well.

    Function:
    On ligand binding, forms a receptor complex consisting of two type II and two type I transmembrane serine/threonine kinases. Type II receptors phosphorylate and activate type I receptors which autophosphorylate, then bind and activate SMAD transcriptional regulators. Receptor for TGF-beta. May bind activin as well.

    Subcellular Location:
    Membrane.

    DISEASE:
    Defects in ACVRL1 are the cause of hereditary hemorrhagic telangiectasia type 2 (HHT2) [MIM:600376]; also known as Osler-Rendu-Weber syndrome 2 (ORW2). HHT2 is an autosomal dominant multisystemic vascular dysplasia, characterized by recurrent epistaxis, muco-cutaneous telangiectases, gastro-intestinal hemorrhage, and pulmonary, cerebral and hepatic arteriovenous malformations; all secondary manifestations of the underlying vascular dysplasia.

    Similarity:
    Belongs to the protein kinase superfamily. TKL Ser/Thr protein kinase family. TGFB receptor subfamily.
    Contains 1 GS domain.
    Contains 1 protein kinase domain.

    SWISS:
    P37023

    Gene ID:
    94

    Database links:

    Entrez Gene: 94 Human

    Omim: 601284 Human

    SwissProt: P37023 Human

    Unigene: 591026 Human



    產(chǎn)品圖片
    Sample: Cerebrum (Mouse) Lysate at 40 ug Liver (Mouse) Lysate at 40 ug Primary: Anti-ACVRL1 (bs-21783R) at 1/1000 dilution Secondary: IRDye800CW Goat Anti-Rabbit IgG at 1/20000 dilution Predicted band size: 53 kD Observed band size: 53 kD
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