Overview

Description

  • NatureRecombinant
  • SourceEscherichia coli
  • Amino Acid Sequence
    • AccessionQ13873
    • SpeciesHuman
    • Molecular weight29 kDa
    • Amino acids295 to 552

Specifications

Our Abpromise guarantee covers the use of ab126025 in the following tested applications.

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

  • Applications

    SDS-PAGE

  • FormLyophilised
  • Concentration information loading...

Preparation and Storage

  • Stability and Storage

    Shipped at 4°C. Store at -20°C.

    Constituents: 0.32% Tris HCl, 0.58% Sodium chloride

  • ReconstitutionReconstitute with water to desired concentration.

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
  • 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. 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 specificityHighly expressed in heart and liver.
  • Involvement in diseaseDefects 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 similaritiesBelongs to the protein kinase superfamily. TKL Ser/Thr protein kinase family. TGFB receptor subfamily.
    Contains 1 protein kinase domain.
  • Cellular localizationMembrane.
  • Information by UniProt

References for Recombinant Human BMPR2 protein (ab126025)

ab126025 has not yet been referenced specifically in any publications.

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