BMPR2 / BMPR-II / PPH1抗体

Human bone morphogenetic protein type I I serine (threonine) kinase receptor (BMPR-II) is a transmembrane serine/threonine kinase that binds BMP-2 and BMP-7 in association with multiple type I receptors, including BMPR-IA/Brk1, BMPR-IB, and ActR-I. BMPR-II is characterized by lacking of a GS domain, and presence of a C-terminal extension typical of type I I receptors. Expression of BMPRII enhances or is essential for BMP binding to ActR-I, BMPR-IA, and BMPR-IB, and ligand binding BMPR-II is also enhanced by coexpression of BMP type I receptors, indicating that BMP receptors I and II jointly form a high-affinity binding site. BMPR-II plays a key role in cell growth. BMPR2 mutations were predicted to alter the BMP and TGF-b1/SMAD signalling pathways, resulting in proliferation rather than apoptosis of vascular cells, and greatly increase the risk of developing severe pulmonary arterial hypertension.
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