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Product Name BMPR2 Chinese Name 骨形态发生蛋白2受体抗体 Alias 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. Research Area Cardiovascular immunology Signal transduction Stem cells Growth factors and hormones Cell Surface Molecule Immunogen Species Rabbit Clonality Polyclonal React Species Mouse, (predicted: Human, Rat, Dog, Pig, Horse, Rabbit, ) Applications WB=1:500-2000
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.Theoretical molecular weight 112kDa Cellular localization The nucleus The cell membrane Form Liquid Concentration 1mg/ml immunogen KLH conjugated synthetic peptide derived from human BMPR2: 28-100/1038 <Extracellular> Lsotype IgG Purification affinity purified by Protein A Buffer Solution 0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol. Storage Shipped at 4℃. Store at -20 °C for one year. Avoid repeated freeze/thaw cycles. Attention This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications. PubMed PubMed Product Detail 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.
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.
Subcellular Location:
Membrane.
Tissue Specificity:
Highly expressed in heart and liver.
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.
Similarity:
Belongs to the protein kinase superfamily.
TKL Ser/Thr protein kinase family. TGFB receptor subfamily.
Contains 1 protein kinase domain.
SWISS:
Q13873
Gene ID:
659
Database links:Entrez Gene: 659 Human
Entrez Gene: 12168 Mouse
Omim: 600799 Human
SwissProt: Q13873 Human
SwissProt: O35607 Mouse
Unigene: 471119 Human
Unigene: 391654 Mouse
Unigene: 7106 Mouse
Unigene: 40848 Rat
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