Recombinant人BMPR2 protein (ab126025)

概述

描述

  • 性质Recombinant
  • 来源Escherichia coli
  • 氨基酸序列
    • AccessionQ13873
    • 种属Human
    • 分子量29 kDa
    • 氨基酸295 to 552

技术指标

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.

  • 应用

    SDS-PAGE

  • 形式Lyophilised
  • Concentration information loading...

制备和贮存

  • 稳定性和存储

    Shipped at 4°C. Store at -20°C.

    Constituents: 0.32% Tris HCl, 0.58% Sodium chloride

  • 复溶Reconstitute with water to desired concentration.

常规信息

  • 别名
    • 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
  • 功能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.
  • 组织特异性Highly expressed in heart and liver.
  • 疾病相关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.
  • 序列相似性Belongs to the protein kinase superfamily. TKL Ser/Thr protein kinase family. TGFB receptor subfamily.
    Contains 1 protein kinase domain.
  • 细胞定位Membrane.
  • Information by UniProt

Recombinant Human BMPR2 protein (ab126025)参考文献

ab126025 has not yet been referenced specifically in any publications.

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