Overview

Description

  • Nature
    Recombinant
  • Source
    Baculovirus infected Sf9 cells
  • Amino Acid Sequence
    • Species
      Human
    • Amino acids
      144 to 503

Associated products

Specifications

Our Abpromise guarantee covers the use of ab70317 in the following tested applications.

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

  • Biological activity
    The specific activity of ab70317 was determined to be 22 nmol /min/mg.
  • Applications

    Western blot

    Functional Studies

  • Form
    Liquid
  • Additional notes

    ab91090 (Cow Casein full length protein) can be utilized as a substrate for assessing Kinase activity

  • Concentration information loading...

Preparation and Storage

  • Stability and Storage

    Shipped on dry ice. Upon delivery aliquot and store at -80ºC. Avoid freeze / thaw cycles.

    Preservative: None
    Constituents: 25% Glycerol, 50mM Tris HCl, 150mM Sodium chloride, 0.25mM DTT, 0.1mM EGTA, 0.1mM EDTA, 0.1mM PMSF, pH 7.5

    This product is an active protein and may elicit a biological response in vivo, handle with caution.

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

Images

  • SDS showing ACVRL1 at approximately 64kDa.
  • Kinase assay to demonstrate specific activity.

References

ab70317 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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