Overview

  • Product name
    Anti-ACVRL1 antibody
    See all ACVRL1 primary antibodies
  • Description
    Goat polyclonal to ACVRL1
  • Host species
    Goat
  • Tested applications
    Suitable for: WBmore details
  • Species reactivity
    Reacts with: Human
  • Immunogen

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

    Run BLAST with BLAST the sequence with ExPASy Run BLAST with BLAST the sequence with NCBI
  • Positive control
    • Lung and liver extracts.
  • General notes


    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).

Properties

Applications

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.

Application Abreviews Notes
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).

Target

  • 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.
  • Involvement in 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.
  • Sequence similarities
    Belongs to the protein kinase superfamily. TKL Ser/Thr protein kinase family. TGFB receptor subfamily.
    Contains 1 GS domain.
    Contains 1 protein kinase domain.
  • Cellular localization
    Membrane.
  • Information by UniProt
  • Database links
    • Alternative names
      • 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

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    References

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