Anti human BMP-R2 (#9A10)

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

Chromosome: 2; NC_000002.12 (202376327..202567751). Location: 2q33-q34

Monoclonal

IgG1

(9A10)

Mouse

This antibody was selected for its ability to detect human BMP RII. No cross reactivity was found to hBMP RIA and RIB.

Protein G affinity chromatography

Lyophilized

Lyophilized samples are stable for 2 years from date of receipt when stored at -70 degree C. Reconstituted antibody can be aliquoted and stored frozen at < -20 degree C for at least for six months without detectable loss of activity.

Manufactured in an ISO 9001:2008 Certified Laboratory.

Small volumes of anti-BMP receptor-2 antibody 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.

Like other members of the TGFbeta superfamily, cellular responses to bone morphogenetic proteins (BMPs) have been shown to be mediated by the binding to heteromeric complex of type I and type II serine-threonine kinase receptors. Both receptor types are required for the signl transduction. BMP receptor II (BMPR II or BMPR-2) is one of the five mammalian type II receptors (including TGFbetaRII, ActRII, ActRIIB, BMPRII and MISRII) for the TGFbeta superfamily ligands. The type II receptors for TGFbeta and activin bind ligands with high affinity by itself. In contrast, BMPRII binds BMP2, BMP4 and BMP7 weakly in the absence of type I receptor, and the binding can be facilitated by the presence of the type I receptor. BMPRII mRNA is widely expressed in fetal and ***** tissues. Human and mouse BMPRII are highly conserved sharing 97% sequence identity.

Western Blot (WB)

1. WB: 1:100-1000

115,201 Da

bone morphogenetic protein receptor, type II (serine/threonine kinase)

bone morphogenetic protein receptor type-2; BMPR-2; BMP type-2 receptor; BMP type II receptor; type II activin receptor-like kinase; bone morphogenetic protein receptor type II; type II receptor for bone morphogenetic protein-4

Bone morphogenetic protein receptor type-2

PPH1; BMP type-2 receptor; BMPR-2; BMP type II receptor; BMPR-II??[Similar Products]

BMPR2_HUMAN

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]

Function: 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 BMP-7, BMP-2 and, less efficiently, BMP-4. Binding is weak but enhanced by the presence of type I receptors for BMPs.

Catalytic activity: ATP + [receptor-protein] = ADP + [receptor-protein] phosphate.

Cofactor: Magnesium or manganese

By similarity.

Subcellular location: Membrane; Single-pass type I membrane protein.

Tissue specificity: Highly expressed in heart and liver.

Involvement in disease: Pulmonary hypertension, primary, 1 (PPH1) [MIM:178600]: A rare disorder characterized by plexiform lesions of proliferating endothelial cells in pulmonary arterioles. The lesions lead to elevated pulmonary arterial pression, right ventricular failure, and death. The disease can occur from infancy throughout life and it has a mean age at onset of 36 years. Penetrance is reduced. Although familial pulmonary hypertension is rare, cases secondary to known etiologies are more common and include those associated with the appetite-suppressant drugs.Note: The disease is caused by mutations affecting the gene represented in this entry. Ref.9 Ref.10 Ref.11 Ref.12 Ref.13 Ref.15Pulmonary venoocclusive disease (PVOD) [MIM:265450]: Rare form of pulmonary hypertension in which the vascular changes originate in the small pulmonary veins and venules. The pathogenesis is unknown and any link with PPH1 has been speculative. The finding of PVOD associated with a BMPR2 mutation reveals a possible pathogenetic connection with PPH1.Note: The disease is caused by mutations affecting the gene represented in this entry. Ref.14 Ref.16

Sequence similarities: Belongs to the protein kinase superfamily. TKL Ser/Thr protein kinase family. TGFB receptor subfamily.Contains 1 protein kinase domain.

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