- Catalog number
- A00324-2
- Size / count
- 100 µg/Vial
- Dry ice
- No
- Hazardous
- No
- Discontinued
- No
- Background
- Bone morphogenetic protein receptor type II or BMPR2 is a serine/threonine receptor kinase. 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.
- Synonyms
- Bone morphogenetic protein receptor type-2; BMP type-2 receptor; BMPR-2; Bone morphogenetic protein receptor type II; BMP type II receptor; BMPR-II; BMPR2; PPH1
- Gene Name
- BMPR2
- Gene ID
- 659
- UniProt
- Q13873
- Host
- Rabbit
- Reactivity
- Human, Mouse, Rat
- Cross Reactivity
- No cross-reactivity with other proteins.
- Immunogen
- E.coli-derived human BMPR2 recombinant protein (Position: Q28-L1038) .
- Clonality
- Polyclonal
- Tissue Specificity
- Highly expressed in heart and liver.
- Applications
- ELISA, Flow Cytometry, WB
- Field of Research
- Cardiovascular, Cytoskeleton/ECM, Extracellular Matrix, Growth Factors/Hormones, Hypertrophy, Mesenchymal Stem Cells, Protein Phosphorylation, Ser/Thr Kinases, Signal Transduction, Signaling Pathways, Stem Cells, Structures, Surface Molecules, TGF Beta
- Purification
- Immunogen affinity purified.
- Concentration
- Adding 0.2 ml of distilled water will yield a concentration of 500 μg/ml.
- Form
- Lyophilized
- Reconstitution
- Add 0.2ml of distilled water will yield a concentration of 500ug/ml.
- 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.
- References & Citations
- 1. Aldred, M. A., Vijayakrishnan, J., James, V., Soubrier, F., Gomez-Sanchez, M. A., Martensson, G., Galie, N., Manes, A., Corris, P., Simonneau, G., Humbert, M., Morrell, N. W., Trembath, R. C. BMPR2 gene rearrangements account for a significant proportion of mutations in familial and idiopathic pulmonary arterial hypertension. (Abstract) Hum. Mutat. 27: 212-213, 2006. 2. Machado, R. D., Pauciulo, M. W., Thomson, J. R., Lane, K. B., Morgan, N. V., Wheeler, L., Phillips, J. A., III, Newman, J., Williams, D., Galie, N., Manes, A., McNeil, K., and 11 others.BMPR2 haploinsufficiency as the inherited molecular mechanism for primary pulmonary hypertension. Am. J. Hum. Genet. 68: 92-102, 2001.
- Storage Conditions
- Store at -20°C for one year from date of receipt. After reconstitution, at 4°C for one month. It can also be aliquotted and stored frozen at -20°C for six months. Avoid repeated freeze-thaw cycles.
- Calculated Molecular Weight
- 29041 MW
- Observed Molecular Weight
- 150 kDa
- Applications Notes
- 6
- Gene Name Synonym
- Bone morphogenetic protein receptor type 2
- Subcellular Location
- Cell membrane.
- Protein Name
- Copper chaperone for superoxide dismutase
- Isotype
- Rabbit IgG
- Contents
- Each vial contains 4mg Trehalose, 0.9mg NaCl, 0.2mg Na2HPO4.