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Overview

  • Product nameAnti-ACVRL1 antibodySee all ACVRL1 primary antibodies ...
  • Description
    Goat polyclonal to ACVRL1
  • Tested applicationsWB more 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 controlLung 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 Notes
WB 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 ACVRL1 peptide (491-503) (ab23196).

Target

  • FunctionOn 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 diseaseDefects 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 similaritiesBelongs to the protein kinase superfamily. TKL Ser/Thr protein kinase family. TGFB receptor subfamily.
    Contains 1 GS domain.
    Contains 1 protein kinase domain.
  • Cellular localizationMembrane.

    • Target information above from: UniProt accession P37023 The UniProt Consortium
    The Universal Protein Resource (UniProt) in 2010
    Nucleic Acids Res. 38:D142-D148 (2010) .

    Information by UniProt
  • Database links
    • Alternative names
        Activin A receptor antibodyActivin A receptor type II like 1 antibodyActivin A receptor, type II like kinase 1 antibody
        Activin receptor like kinase 1 antibodyActivin receptor-like kinase 1 antibodyACVL1_HUMAN antibodyACVRL1 antibodyACVRLK1 antibodyALK-1 antibodyALK1 antibodyHHT antibodyHHT2 antibodyORW2 antibodyOsler Rendu Weber syndrome 2 antibodySerine/threonine protein kinase receptor R3 antibodySerine/threonine-protein kinase receptor R3 antibodySKR3 antibodyTGF B superfamily receptor type I antibodyTGF-B superfamily receptor type I antibodyTSR-I antibodyTSR1 antibody
      see all

    Anti-ACVRL1 antibody images

    References for Anti-ACVRL1 antibody (ab10179)

    ab10179 has not yet been referenced specifically in any publications.

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