製品の概要

  • 製品名Anti-ACVRL1 antibody
    ACVRL1 一次抗体 製品一覧
  • 製品の詳細
    Goat polyclonal to ACVRL1
  • アプリケーション適用あり: WBmore details
  • 種交差性
    交差種: Human
  • 免疫原

    Synthetic peptide: KISNSPEKPKVIQ, corresponding to C terminal amino acids 491-503 of Human ACVRL1.

  • ポジティブ・コントロール
    • Lung and liver extracts.
  • 特記事項


    Activin A receptor, type II-like 1 is a type I cell-surface receptor for the TGF-beta superfamily of ligands. ACVRL1 is most highly expressed in human placenta and lung. ACVRL1 deficiency causes hemorrhagic telangiectasia type 2 (HHT2; MIM 600376 ) also known as Rendu-Osler-Weber syndrome 2 (ORW2). Note: ACVRL1 is also known as ALK1 - not to be confused with the similarly named ALK (anaplastic lymphoma kinase).

製品の特性

関連製品

アプリケーション

Our Abpromise guarantee covers the use of ab10179 in the following tested applications.

The application notes include recommended starting dilutions; optimal dilutions/concentrations should be determined by the end user.

アプリケーション Abreviews 特記事項
WB Use a concentration of 1 - 3 µg/ml. Detects a band of approximately 60 kDa (predicted molecular weight: 56 kDa).Can be blocked with Human ACVRL1 peptide (ab23196).

ターゲット情報

  • 機能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.
  • 関連疾患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.
  • 配列類似性Belongs to the protein kinase superfamily. TKL Ser/Thr protein kinase family. TGFB receptor subfamily.
    Contains 1 GS domain.
    Contains 1 protein kinase domain.
  • 細胞内局在Membrane.
  • Information by UniProt
  • 参照データベース
  • 別名
    • Activin A receptor antibody
    • Activin A receptor type II like 1 antibody
    • Activin A receptor, type II like kinase 1 antibody
    • Activin receptor like kinase 1 antibody
    • Activin receptor-like kinase 1 antibody
    • ACVL1_HUMAN antibody
    • ACVRL1 antibody
    • ACVRLK1 antibody
    • ALK-1 antibody
    • ALK1 antibody
    • HHT antibody
    • HHT2 antibody
    • ORW2 antibody
    • Osler Rendu Weber syndrome 2 antibody
    • Serine/threonine protein kinase receptor R3 antibody
    • Serine/threonine-protein kinase receptor R3 antibody
    • SKR3 antibody
    • TGF B superfamily receptor type I antibody
    • TGF-B superfamily receptor type I antibody
    • TSR-I antibody
    • TSR1 antibody
    see all

Anti-ACVRL1 antibody 画像

Anti-ACVRL1 antibody (ab10179) 使用論文

ab10179 has not yet been referenced specifically in any publications.

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