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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. Note: This description may include information from UniProtKB.
Protein type: Cell cycle regulation; EC; 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: caveola; cell-cell adherens junction; extracellular space; integral to plasma membrane; nucleoplasm; plasma membrane
Molecular Function: activin receptor activity, type II; protein binding
Biological Process: alveolus development; anterior/posterior pattern formation; blood vessel remodeling; BMP signaling pathway; cardiac muscle development; cellular response to starvation; chondrocyte development; endothelial cell proliferation; lymphangiogenesis; mesoderm formation; negative regulation of cell growth; negative regulation of systemic arterial blood pressure; negative regulation of vasoconstriction; positive regulation of BMP signaling pathway; positive regulation of bone mineralization; positive regulation of endothelial cell proliferation; positive regulation of ossification; positive regulation of osteoblast differentiation; positive regulation of transcription from RNA polymerase II promoter; proteoglycan biosynthetic process; regulation of cell proliferation; regulation of lung blood pressure; transcription from RNA polymerase II promoter; transmembrane receptor protein serine/threonine kinase signaling pathway; vascular endothelial growth factor receptor signaling pathway
Disease: Pulmonary Hypertension, Primary, 1; Pulmonary Venoocclusive Disease 1, Autosomal Dominant
Reference #:  Q13873 (UniProtKB)
Alt. Names/Synonyms: BMP type II receptor; BMP type-2 receptor; BMPR-2; BMPR-II; BMPR2; BMPR3; BMPRII; BMR2; Bone morphogenetic protein receptor type II; Bone morphogenetic protein receptor type-2; bone morphogenetic protein receptor, type II (serine/threonine kinase); BRK-3; FLJ41585; FLJ76945; PPH1; T-ALK; type II activin receptor-like kinase; type II receptor for bone morphogenetic protein-4
Gene Symbols: BMPR2
Molecular weight: 115,201 Da
Basal Isoelectric point: 5.82  Predict pI for various phosphorylation states
Protein-Specific Antibodies or siRNAs from Cell Signaling Technology® Total Proteins
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