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BMPR-IB Polyclonal Antibody
Ucallm_WP15957-50
Price range: $268.00 through $328.00
Product details
Background:
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 2 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. Several transcript variants encoding two different isoforms have been found for thi
Specifications
| Size | 50μL/100μL |
| Target | BMPR-IB |
| Reactivity | Human, Mouse |
| Applications | IHC, IF, ELISA |
| MW(Calculated) | |
| MW(Observed) | |
| Host Species | Rabbit |
| Isotype | IgG |
| Conjugate/Modification | Unmodified |
| Recommended Dilution Ratio | IHC 1:50-200; ELISA 1:10000-20000; IF 1:50-200 / Note: For IHC, we suggest antigen retrieval with TE buffer pH 9.0 (Cat#WT1004) |
| Formulation | Liquid in PBS containing 50% glycerol, 0.5% BSA and 0.02% sodium azide. |
| Purification | The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen. |
| Storage | -15°C to -25°C/1 year(Do not lower than -25°C) |
| Concentration | 1 mg/ml |
| Clonality | Polyclonal |
| Clone Number | |
| Immunogen | Synthetic peptide from human protein at AA range: 21-70 |
| Specificity | The antibody detects endogenous BMPR-IB |
| Gene Name | BMPR1B |
| Protein Name | Bone morphogenetic protein receptor type-1B (BMP type-1B receptor) (BMPR-1B) (CD antigen CDw293) |
| Other Name | Bone morphogenetic protein receptor type-1B; / BMP type-1B receptor; / BMPR-1B; / CD antigen CDw293 |
| Species | Human |
| Gene ID | 658 |
| Uniprot | O00238, |
| Species.1 | Mouse |
| Gene ID.1 | 12167 |
| Uniprot.1 | P36898 |
| Species.2 | |
| Gene ID.2 | |
| Uniprot.2 | |
| Cellular Localization | Cell membrane; Single-pass type I membrane protein . |
| Tissue Expression | Ovary,PNS,Prostate, |
| Signaling_pathway | Cellular Processes >> Cellular community - eukaryotes >> Signaling pathways regulating pluripotency of stem cells;Environmental Information Processing >> Signal transduction >> TGF-beta signaling pathway;Environmental Information Processing >> Signal transduction >> Hippo signaling pathway;Environmental Information Processing >> Signaling molecules and interaction >> Cytokine-cytokine receptor interaction |
| Research Areas | >>Cytokine-cytokine receptor interaction; / >>TGF-beta signaling pathway; / >>Axon guidance; / >>Hippo signaling pathway; / >>Signaling pathways regulating pluripotency of stem cells; / >>Fluid shear stress and atherosclerosis |
| Function | Catalytic activity:ATP + [receptor-protein] = ADP + [receptor-protein] phosphate.,cofactor:Magnesium or manganese.,Disease:Defects in BMPR1B are a cause of brachydactyly type A2 (BDA2) [MIM:112600]. Brachydactylies (BDs) are a group of inherited malformations characterized by shortening of the digits due to abnormal development of the phalanges and/or the metacarpals. They have been classified on an anatomic and genetic basis into five groups, A to E, including three subgroups (A1 to A3) that usually manifest as autosomal dominant traits. BDA2 was described first in a large Norwegian kindred. BDA2 is caused by mutations in BMPR1B gene and studies demonstrate that these mutations function as dominant negatives in vitro and in vivo.,Disease:Defects in BMPR1B are the cause of acromesomelic chondrodysplasia with genital anomalies (AMDGA) [MIM:609441]. Acromesomelic chondrodysplasias are rare hereditary skeletal disorders characterized by short stature, very short limbs, and hand/foot malformations. The severity of limb abnormalities increases from proximal to distal with profoundly affected hands and feet showing brachydactyly and/or rudimentary fingers (knob-like fingers).,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. Receptor for BMPS/OP-1.,similarity:Belongs to the protein kinase superfamily. TKL Ser/Thr protein kinase family. TGFB receptor subfamily.,similarity:Contains 1 GS domain.,similarity:Contains 1 protein kinase domain., |
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