Overview

Description

  • Nature
    Synthetic
  • Amino Acid Sequence
    • Species
      Human
    • Sequence
      C-KISNSPEKPKVIQ
    • Amino acids
      491 to 503

Associated products

Specifications

Our Abpromise guarantee covers the use of ab23196 in the following tested applications.

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

  • Applications

    Blocking - Blocking peptide for Anti-ACVRL1 antibody (ab10179)

  • Form
    Liquid
  • Concentration information loading...

Preparation and Storage

  • Stability and Storage

    Shipped at 4°C. Upon delivery aliquot and store at -20°C or -80°C. Avoid repeated freeze / thaw cycles.

General Info

  • Alternative names
    • Activin A receptor
    • Activin A receptor type II like 1
    • Activin A receptor, type II like kinase 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
    see all
  • 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

References

ab23196 has not yet been referenced specifically in any publications.

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Please note: All products are "FOR RESEARCH USE ONLY AND ARE NOT INTENDED FOR DIAGNOSTIC OR THERAPEUTIC USE"

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