ITM0074
ITM0074
- Catalog: ITM0074
- Gene/Protein: BMPR2
- Product Description: Immunotag™ BMPR-II Monoclonal Antibody
504.0000
Price in reward points: 504
| Antibody Specification | |
| Datasheet | 
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| Target Protein | BMPR-II |
| Clonality | Monoclonal |
| Storage/Stability | -20°C/1 year |
| Application | WB,IHC-p,ELISA |
| Recommended Dilution | Western Blot: 1/500 - 1/2000. Immunohistochemistry: 1/200 - 1/1000. ELISA: 1/10000. Not yet tested in other applications. |
| Concentration | 1 mg/ml |
| Reactive Species | Human,Mouse,Rat,Monkey |
| Host Species | Mouse |
| Immunogen | Purified recombinant fragment of human BMPR-II expressed in E. Coli. |
| Specificity | BMPR-II Monoclonal Antibody detects endogenous levels of BMPR-II protein. |
| Purification | Affinity purification |
| Form | Ascitic fluid containing 0.03% sodium azide. |
| Gene Name | BMPR2 |
| Accession No. | Q13873 O35607 |
| Alternate Names | BMPR2; PPH1; Bone morphogenetic protein receptor type-2; BMP type-2 receptor; BMPR-2; Bone morphogenetic protein receptor type II; BMP type II receptor; BMPR-II |
| Description | bone morphogenetic protein receptor type 2(BMPR2) Homo sapiens 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 disea |
| Cell Pathway/ Category | Cytokine-cytokine receptor interaction,TGF-beta, |
| Protein Expression | Kidney,Skin,Skin fibroblast,Substantia nigra, |
| Subcellular Localization | extracellular space,cytoplasm,plasma membrane,integral component of plasma membrane,caveola,cell-cell junction,basal plasma membrane,cell surface,postsynaptic density,apical plasma membrane,dendrite,neuro |
| Protein Function | catalytic activity:ATP + [receptor-protein] = ADP + [receptor-protein] phosphate.,cofactor:Magnesium or manganese.,disease: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.,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.,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.,similarity:Belongs to the protein kinase superfamily. TKL Ser/Thr protein kinase family. TGFB receptor subfamily.,similarity:Contains 1 protein kinase domain.,tissue specificity:Highly expressed in heart and liver., |
| Usage | For Research Use Only! Not for diagnostic or therapeutic procedures. |
| Material Safety Data Sheet | |
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