BMPRII; BMPR-II; BRK3; T-ALK; BMPR3; PPH1; Primary Pulmonary Hypertension 1; BMP type II receptor

For Research Use Only. Not for use in diagnostic procedures.

Polyclonal

Rabbit

200ug/ml (lot specific)

Manufactured in an ISO 9001:2008 and ISO 13485:2003 Certified Laboratory.

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Small volumes of anti-BMPR2 antibody vial(s) may occasionally become entrapped in the seal of the product vial during shipment and storage. If necessary, briefly centrifuge the vial on a tabletop centrifuge to dislodge any liquid in the container`s cap. Certain products may require to ship with dry ice and additional dry ice fee may apply.

Western Blot (WB), Immunohistochemistry (IHC)

59,963 Da

bone morphogenetic protein receptor type 2

bone morphogenetic protein receptor type-2

Bone morphogenetic protein receptor type-2

PPH1; BMP type-2 receptor; BMPR-2; BMP type II receptor; BMPR-II??[Similar Products]

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, Jul 2008]

BMPR2: a serine/threonine-protein kinase receptor for bone morphogenetic protein (BMP). 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. 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. Defects in BMPR2 are the cause of primary pulmonary hypertension (PPH1), a weakly penetrant dominant disorder, associated with lesions in pulmonary arterioles, elevated pulmonary arterial pressure, and right ventricular failure.

Protein type: Cell cycle regulation; EC 2.7.11.30; Kinase, protein; Membrane protein, integral; Protein kinase, Ser/Thr (receptor); Protein kinase, TKL; STKR family; TKL group; Type2 subfamily

Chromosomal Location of Human Ortholog: 2q33.1-q33.2

Cellular Component: apical plasma membrane; basal plasma membrane; caveola; cell soma; cell surface; cell-cell adherens junction; cytoplasm; dendrite; extracellular space; integral component of plasma membrane; nucleoplasm; plasma membrane; postsynaptic density; spanning component of plasma membrane

Molecular Function: activin receptor activity, type II; ATP binding; BMP receptor activity; growth factor binding; metal ion binding; protein binding; signal transducer, downstream of receptor, with serine/threonine kinase activity

Biological Process: activin receptor signaling pathway; alveolus development; anterior/posterior pattern specification; atrioventricular valve morphogenesis; blood vessel remodeling; BMP signaling pathway; brain development; cardiac muscle tissue development; cellular response to BMP stimulus; cellular response to starvation; chondrocyte development; endocardial cushion development; endothelial cell apoptotic process; endothelial cell proliferation; limb development; lymphangiogenesis; maternal placenta development; mesoderm formation; mitral valve morphogenesis; negative regulation of cell growth; negative regulation of cell proliferation involved in heart valve morphogenesis; negative regulation of chondrocyte proliferation; negative regulation of DNA biosynthetic process; negative regulation of systemic arterial blood pressure; negative regulation of vasoconstriction; outflow tract morphogenesis; outflow tract septum morphogenesis; positive regulation of axon extension involved in axon guidance; positive regulation of BMP signaling pathway; positive regulation of bone mineralization; positive regulation of endothelial cell migration; positive regulation of endothelial cell proliferation; positive regulation of epithelial cell migration; positive regulation of ossification; positive regulation of osteoblast differentiation; positive regulation of pathway-restricted SMAD protein phosphorylation; positive regulation of transcription from RNA polymerase II promoter; proteoglycan biosynthetic process; regulation of cell proliferation; regulation of lung blood pressure; semi-lunar valve development; transcription from RNA polymerase II promoter; transmembrane receptor protein serine/threonine kinase signaling pathway; tricuspid valve morphogenesis; vascular endothelial growth factor receptor signaling pathway

Disease: Pulmonary Hypertension, Primary, 1; Pulmonary Venoocclusive Disease 1, Autosomal Dominant

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