Human BMPR2 Protein, ECD (Extracellular Domain), Fc-fusion, Recombinant

Description :
The TGFβ (transforming growth factor β) superfamily proteins are pleiotropic cytokines that regulate a diverse range of cellular processes, including proliferation, differentiation, migration, adhesion and death. The TGFβ superfamily elicits 4 signaling pathways: TGFβ, Bone Morphogenic Protein (BMP), Activin, and Nodal. Each pathway signals through a heteromeric receptor complex composed of at least two type-I and two type-II transmembrane receptors containing cytoplasmic serine/threonine kinase domains. These receptors are distinguished by the presence of a glycine/serine-rich juxta-membrane domain found only in the type I receptors. Either type receptor may initially bind ligand, followed by the recruitment of an alternate-type receptor counterpart to form a signal-activating complex. BMPR2 (BMP receptor type 2), also known as PPH1 and BMPR-II, is a type II receptor for BMPs that are involved in endochondral bone formation and embryogenesis. The type II TGFRs typically bind ligands with high affinity. In contrast, BMPR2 binds BMPs weakly in the absence of type I receptor, and the binding is enhanced by the presence of the type I receptor. BMPR2 is widely expressed in fetal and adult tissues. Reduced expression or function of BMPR2 signaling leads to exaggerated TGFβ signaling and altered cellular response. BMPR2 may contribute to the maintenance of normal pulmonary vascular structure and function. Mutations in the BMPR2 gene are associated with primary pulmonary hypertension (PPH1) and pulmonary venoocclusive disease 1 (PVOD1). Human and mouse BMPR2 are 97% identical and show cross-species binding.