Overview

Description

  • NatureRecombinant
  • SourceBaculovirus infected Sf9 cells
  • Amino Acid Sequence
    • SpeciesHuman
    • Amino acids144 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 activityThe specific activity of ab70317 was determined to be 22 nmol /min/mg.
  • Applications

    Western blot

    Functional Studies

  • FormLiquid
  • 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
  • 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.
  • Information by UniProt

Recombinant human ACVRL1 protein images

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

References for Recombinant human ACVRL1 protein (ab70317)

ab70317 has not yet been referenced specifically in any publications.

Product Wall

There are currently no Abreviews or Questions for ab70317.
Please use the links above to contact us or submit feedback about this product.

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