BMPR1B Polyclonal Antibody, HRP Conjugated
Cat# bs-6639R-HRP
Size : 100ul
Brand : Bioss
BMPR1B Polyclonal Antibody, HRP Conjugated
Applications
Predicted Reactivity
| Overview | |
| Catalog # | bs-6639R-HRP |
| Product Name | BMPR1B Polyclonal Antibody, HRP Conjugated |
| Applications | WB, ELISA |
| Predicted Reactivity | Human, Mouse, Rat, Dog, Cow, Sheep, Rabbit |
| Specifications | |
| Conjugation | HRP |
| Host | Rabbit |
| Source | KLH conjugated synthetic peptide derived from human BMPR1B |
| Immunogen Range | 61-160/502 |
| Clonality | Polyclonal |
| Isotype | IgG |
| Concentration | 1ug/ul |
| Purification | Purified by Protein A. |
| Storage Buffer | Aqueous buffered solution containing 0.01M TBS (pH 7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol. |
| Storage Condition | Store at -20°C. Aliquot into multiple vials to avoid repeated freeze-thaw cycles. |
| Target | |
| Gene ID | 658 |
| Subcellular location | Cell membrane |
| Synonyms | BMPR-IB; Activin receptor like kinase 6; Acvrlk6; ALK 6; ALK6; alk6tr; BMP type-1B receptor; BMPR IB; BMPR-1B; Bmpr1b; BMPRIB; BMR1B_HUMAN; Bone morphogenetic protein receptor type 1B; Bone morphogenetic protein receptor type IB; Bone morphogenetic protein receptor type-1B; BR 1b; BR1b; CDw 293; CDw293; CDw293 antigen; CFK 43a; CFK43a; Serine/threonine receptor kinase; zALK 6; zALK6. |
| Background | 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 BMP7/OP-1 and GDF5.Involvement in disease; Defects in BMPR1B are the cause of acromesomelic chondrodysplasia with genital anomalies (AMDGA). 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).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. |
| Application Dilution | |
| WB | 1:300-5000 |
| ELISA | 1:500-1000 |