Overview

Description

  • Nature

    Recombinant
  • Source

    Baculovirus infected Sf9 cells
  • Amino Acid Sequence
    • Accession
    • Species

      Human
    • Molecular weight

      115 kDa including tags
    • Amino acids

      174 to 1038
    • Tags

      His tag N-Terminus

Specifications

Our Abpromise guarantee covers the use of ab126926 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 ab126926 was determined to be 14 nmol/min/mg.
  • Applications

    SDS-PAGE

    Functional Studies

    Western blot

  • Purity

    > 75 % Densitometry.

  • Form

    Liquid
  • Additional notes

    ab64311 (Myelin Basic Protein 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.

    pH: 7.00
    Preservative: 1.02% Imidazole
    Constituents: 0.002% PMSF, 0.81% Sodium phosphate, 0.0038% DTT, 25% Glycerol, 1.76% Sodium chloride

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

General Info

  • Alternative names

    • BMP type II receptor
    • BMP type-2 receptor
    • BMPR 2
    • BMPR 3
    • BMPR II
    • BMPR-2
    • BMPR-II
    • Bmpr2
    • BMPR2_HUMAN
    • BMPR3
    • BMPRII
    • BMR 2
    • BMR2
    • Bone morphogenetic protein receptor type 2
    • Bone morphogenetic protein receptor type II
    • Bone morphogenetic protein receptor type-2
    • Bone morphogenic protein receptor type II serine threonine kinase
    • BRK 3
    • BRK3
    • PPH 1
    • PPH1
    • Serine threonine kinase type II activin receptor like kinase
    • T ALK
    • TALK
    • Type II activin receptor like kinase
    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. Binds to BMP-7, BMP-2 and, less efficiently, BMP-4. Binding is weak but enhanced by the presence of type I receptors for BMPs.
  • Tissue specificity

    Highly expressed in heart and liver.
  • Involvement in disease

    Defects in BMPR2 are the cause of primary pulmonary hypertension (PPH1) [MIM:178600]. PPH1 is a rare autosomal dominant disorder characterized by plexiform lesions of proliferating endothelial cells in pulmonary arterioles. The lesions lead to elevated pulmonary arterial pression, right ventricular failure, and death. The disease can occur from infancy throughout life and it has a mean age at onset of 36 years. Penetrance is reduced. Although familial PPH1 is rare, cases secondary to known etiologies are more common and include those associated with the appetite-suppressant drugs.
    Defects in BMPR2 are a cause of pulmonary venoocclusive disease (PVOD) [MIM:265450]. PVOD is a rare form of pulmonary hypertension in which the vascular changes originate in the small pulmonary veins and venules. The pathogenesis is unknown and any link with PPH1 has been speculative. The finding of PVOD associated with a BMPR2 mutation reveals a possible pathogenetic connection with PPH1.
  • Sequence similarities

    Belongs to the protein kinase superfamily. TKL Ser/Thr protein kinase family. TGFB receptor subfamily.
    Contains 1 protein kinase domain.
  • Cellular localization

    Membrane.
  • Information by UniProt

Images

  • The specific activity of ab126926 was determined to be 14 nmol/min/mg.
  • SDS-PAGE analysis of ab126926.

References

ab126926 has not yet been referenced specifically in any publications.

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