Anti-BMPR2 Antibody Picoband® Fluoro647 Conjugated

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.

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

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

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.

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.