BMPR2, BMPR-II, BMPR3, BMR2, BRK-3, FLJ41585, FLJ76945, PPH1, T-ALK

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

Human Cells

> 95 % as determined by SDS-PAGE

Lyophilized from sterile PBS, pH 7.4

Samples are stable for up to twelve months from date of receipt at -70 degree C

Manufactured in an ISO 9001:2015 Certified Laboratory.

Small volumes of BMPR2 / BMPR-II recombinant protein 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.

Background: The bone morphogenetic protein type II receptor (BMPR-II, or BMPR2), a receptor for the transforming growth factor (TGF)-beta/bone morphogenetic protein (BMP) superfamily. Reduced expression or function of BMPR2 signaling leads to exaggerated TGF-beta signaling and altered cellular responses to TGF-beta. In endothelial cells, BMPR2 mutation increases the susceptibility of cells to apoptosis. BMPR2 transduces BMP signals by forming heteromeric complexes with and phosphorylating BMP type I receptors. The intracellular domain of BMPR2 is both necessary and sufficient for receptor complex interaction. It had been identified that BMPR2 plays a key role in cell growth. Its mutations lead to hereditary pulmonary hypertension, and knockout of Bmpr-II results in early embryonic lethality. The C-terminal tail of BMPR2 provides s for a number of regulatory proteins that may initiate Smad-independent signalling. 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. BMPR2 gene result in familial Primary pulmonary hypertension (PPH) transmitted as an autosomal dominant trait, albeit with low penetrance. Heterozygous germline mutations of BMPR2 gene have been identified in patients with familial and sporadic PPH, indicating that BMPR2 may contribute to the maintenance of normal pulmonary vascular structure and function. Tctex-1, a light chain of the motor complex dynein, interacts with the cytoplasmic domain of BMPR2 and demonstrate that Tctex-1 is phosphorylated by BMPR-II, a function disrupted by PPH disease causing mutations within exon 12. BMPR2 and Tctex-1 co-localize to endothelium and smooth muscle within the media of pulmonary arterioles, key sites of vascular remodelling in PPH.

Description: A DNA sequence encoding the human BMPR-II (NP_001195.2) extracellular domain (Met 1-Ile 151) was expressed with a C-terminal polyhistidine tag.

The recombinant human BMPR-II consists of 136 amino acids and has a predicted molecular mass of 15.6 kDa. As a result of glycosylation, the apparent molecular mass of rhBMPR-II is approximately 30-40 kDa in SDS-PAGE under reducing conditions.

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: caveola; extracellular space; integral to plasma membrane; intercellular junction; 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

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