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Mouse Anti-BMPR2 Recombinant Antibody (2C10) (CBMAB-A0837-LY)

The product is antibody recognizes BMPR2. The antibody 2C10 immunoassay techniques such as: sELISA, ELISA.
See all BMPR2 antibodies

Summary

Host Animal
Mouse
Specificity
Human
Clone
2C10
Antibody Isotype
IgG2a, κ
Application
sELISA, ELISA

Basic Information

Immunogen
BMPR2 (NP_001195.2, 939 a.a. ~ 1037 a.a) partial recombinant protein with GST tag. MW of the GST tag alone is 26 KDa.
Specificity
Human
Antibody Isotype
IgG2a, κ
Clonality
Monoclonal
Application Notes
The COA includes recommended starting dilutions, optimal dilutions should be determined by the end user.

Formulations & Storage [For reference only, actual COA shall prevail!]

Format
Liquid
Purity
> 95% Purity determined by SDS-PAGE.
Storage
Store at +4°C short term (1-2 weeks). Aliquot and store at -20°C long term. Avoid repeated freezethaw cycles.

Target

Full Name
Bone Morphogenetic Protein Receptor Type 2
Introduction
This gene encodes a member of the bone morphogenetic protein (BMP) receptor family of transmembrane serine/threonine kinases. The ligands of this receptor are BMPs, which are members of the TGF-beta superfamily. BMPs are involved in endochondral bone formation and embryogenesis. These proteins transduce their signals through the formation of heteromeric complexes of two different types of serine (threonine) kinase receptors: type I receptors of about 50-55 kD and type II receptors of about 70-80 kD. Type II receptors bind ligands in the absence of type I receptors, but they require their respective type I receptors for signaling, whereas type I receptors require their respective type II receptors for ligand binding. Mutations in this gene have been associated with primary pulmonary hypertension, both familial and fenfluramine-associated, and with pulmonary venoocclusive disease. [provided by RefSeq]
Entrez Gene ID
UniProt ID
Alternative Names
BMPR-II; BMPR3; BMR2; BRK-3; FLJ41585; FLJ76945; PPH1; T-ALK
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 BMP7, BMP2 and, less efficiently, BMP4. Binding is weak but enhanced by the presence of type I receptors for BMPs. Mediates induction of adipogenesis by GDF6.
Biological Process
Anterior/posterior pattern specification Source: BHF-UCL
Aortic valve development Source: BHF-UCL
Artery development Source: BHF-UCL
Atrial septum morphogenesis Source: BHF-UCL
Blood vessel development Source: GO_Central
Blood vessel remodeling Source: BHF-UCL
BMP signaling pathway Source: BHF-UCL
Brain development Source: Ensembl
Cellular response to BMP stimulus Source: BHF-UCL
Cellular response to growth factor stimulus Source: GO_Central
Cellular response to starvation Source: BHF-UCL
Chondrocyte development Source: AgBase
Endocardial cushion development Source: BHF-UCL
Endochondral bone morphogenesis Source: AgBase
Endothelial cell apoptotic process Source: UniProtKB
Endothelial cell proliferation Source: UniProtKB
Limb development Source: Ensembl
Lung alveolus development Source: BHF-UCL
Lymphangiogenesis Source: BHF-UCL
Lymphatic endothelial cell differentiation Source: BHF-UCL
Maternal placenta development Source: Ensembl
Mesoderm formation Source: BHF-UCL
Mitral valve morphogenesis Source: BHF-UCL
Negative regulation of BMP signaling pathway Source: Reactome
Negative regulation of cell growth Source: UniProtKB
Negative regulation of cell proliferation involved in heart valve morphogenesis Source: BHF-UCL
Negative regulation of chondrocyte proliferation Source: AgBase
Negative regulation of DNA biosynthetic process Source: BHF-UCL
Negative regulation of muscle cell differentiation Source: Ensembl
Negative regulation of systemic arterial blood pressure Source: BHF-UCL
Negative regulation of vasoconstriction Source: BHF-UCL
Osteoblast differentiation Source: Ensembl
Outflow tract morphogenesis Source: BHF-UCL
Outflow tract septum morphogenesis Source: BHF-UCL
Pharyngeal arch artery morphogenesis Source: BHF-UCL
Positive regulation of axon extension involved in axon guidance Source: Ensembl
Positive regulation of BMP signaling pathway Source: UniProtKB
Positive regulation of bone mineralization Source: BHF-UCL
Positive regulation of cartilage development Source: AgBase
Positive regulation of endothelial cell migration Source: UniProtKB
Positive regulation of endothelial cell proliferation Source: UniProtKB
Positive regulation of epithelial cell migration Source: UniProtKB
Positive regulation of ossification Source: BHF-UCL
Positive regulation of osteoblast differentiation Source: BHF-UCL
Positive regulation of pathway-restricted SMAD protein phosphorylation Source: UniProtKB
Positive regulation of transcription by RNA polymerase II Source: BHF-UCL
Protein phosphorylation Source: GO_Central
Proteoglycan biosynthetic process Source: AgBase
Pulmonary valve development Source: BHF-UCL
Regulation of cell population proliferation Source: HGNC-UCL
Regulation of lung blood pressure Source: BHF-UCL
Retina vasculature development in camera-type eye Source: BHF-UCL
Semi-lunar valve development Source: BHF-UCL
Transmembrane receptor protein serine/threonine kinase signaling pathway Source: BHF-UCL
Tricuspid valve morphogenesis Source: BHF-UCL
Venous blood vessel development Source: BHF-UCL
Ventricular septum morphogenesis Source: BHF-UCL
Cellular Location
Cell membrane
Involvement in disease
Pulmonary hypertension, primary, 1 (PPH1): A rare 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 pulmonary hypertension is rare, cases secondary to known etiologies are more common and include those associated with the appetite-suppressant drugs.
Pulmonary venoocclusive disease 1, autosomal dominant (PVOD1): A disease characterized by widespread fibrous obstruction and intimal thickening of septal veins and preseptal venules, a low diffusing capacity for carbon monoxide, occult alveolar hemorrhage, and nodular ground-glass opacities, septal lines and lymph node enlargement showed by high-resolution computed tomography of the chest. It is frequently associated with pulmonary capillary dilatation and proliferation, and is a rare and devastating cause of pulmonary hypertension.
Topology
Extracellular: 27-150 aa
Helical: 151-171 aa
Cytoplasmic: 172-1038 aa

Tran, H. B., Maiolo, S., Harper, R., Zalewski, P. D., Reynolds, P., & Hodge, S. (2021). Dysregulated zinc and sphingosine‐1‐phosphate signalling in pulmonary hypertension: potential effects by targeting of bone morphogenetic protein receptor type 2 in pulmonary microvessels. Cell Biology International.

Wang, Y., Guo, H., Zhang, Z., Wang, Q., Tian, X., & Yang, Y. (2021). Inhibition of bone morphogenetic protein receptor 2 suppresses pancreatic ductal adenocarcinoma growth by regulating GRB2/PI3K/AKT axis. Annals of Translational Medicine, 9(7).

Jang, A. Y., Kim, B. G., Kwon, S., Seo, J., Kim, H. K., Chang, H. J., ... & Chung, W. J. (2020). Prevalence and clinical features of bone morphogenetic protein receptor type 2 mutation in Korean idiopathic pulmonary arterial hypertension patients: The PILGRIM explorative cohort. PloS one, 15(9), e0238698.

Theilmann, A. L., Hawke, L. G., Hilton, L. R., Whitford, M. K., Cole, D. V., Mackeil, J. L., ... & Ormiston, M. L. (2020). Endothelial BMPR2 Loss Drives a Proliferative Response to BMP (Bone Morphogenetic Protein) 9 via Prolonged Canonical Signaling. Arteriosclerosis, thrombosis, and vascular biology, 40(11), 2605-2618.

Tian, W., Jiang, X., Sung, Y. K., Shuffle, E., Wu, T. H., Kao, P. N., ... & Nicolls, M. R. (2019). Phenotypically silent bone morphogenetic protein receptor 2 mutations predispose rats to inflammation-induced pulmonary arterial hypertension by enhancing the risk for neointimal transformation. Circulation, 140(17), 1409-1425.

Choi, S. H., Jung, Y. K., Jang, J. A., & Han, S. (2019). Idiopathic pulmonary arterial hypertension associated with a novel frameshift mutation in the bone morphogenetic protein receptor II gene and enhanced bone morphogenetic protein signaling: a case report. Medicine, 98(42).

Chinnappan, M., Mohan, A., Agarwal, S., Dalvi, P., & Dhillon, N. K. (2018). Network of Micro RNA s Mediate Translational Repression of Bone Morphogenetic Protein Receptor‐2: Involvement in HIV‐Associated Pulmonary Vascular Remodeling. Journal of the American Heart Association, 7(5), e008472.

Tojais, N. F., Cao, A., Lai, Y. J., Wang, L., Chen, P. I., Alcazar, M. A. A., ... & Rabinovitch, M. (2017). Codependence of bone morphogenetic protein receptor 2 and transforming growth factor-β in elastic fiber assembly and its perturbation in pulmonary arterial hypertension. Arteriosclerosis, thrombosis, and vascular biology, 37(8), 1559-1569.

Hwangbo, C., Lee, H. W., Kang, H., Ju, H., Wiley, D. S., Papangeli, I., ... & Jin, S. W. (2017). Modulation of endothelial bone morphogenetic protein receptor type 2 activity by vascular endothelial growth factor receptor 3 in pulmonary arterial hypertension. Circulation, 135(23), 2288-2298.

Kim, M. J., Park, S. Y., Chang, H. R., Jung, E. Y., Munkhjargal, A., Lim, J. S., ... & Kim, Y. (2017). Clinical significance linked to functional defects in bone morphogenetic protein type 2 receptor, BMPR2. BMB reports, 50(6), 308.

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For research use only. Not intended for any clinical use.

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