概述

  • 产品名称Anti-ACVRL1抗体
    参阅全部 ACVRL1 一抗
  • 描述
    山羊多克隆抗体to ACVRL1
  • 经测试应用适用于: WBmore details
  • 种属反应性
    与反应: Human
  • 免疫原

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

  • 阳性对照
    • Lung and liver extracts.
  • 常规说明


    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).

性能

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应用

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.

应用 Ab评论 说明
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).

靶标

  • 功能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.
  • 疾病相关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.
  • 序列相似性Belongs to the protein kinase superfamily. TKL Ser/Thr protein kinase family. TGFB receptor subfamily.
    Contains 1 GS domain.
    Contains 1 protein kinase domain.
  • 细胞定位Membrane.
  • Information by UniProt
  • 数据库链接
  • 别名
    • 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 图像

Anti-ACVRL1 antibody (ab10179)参考文献

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