Anti-BMPR1B antibody
| 英文名称 | BMPR1B |
| 中文名称 | 骨形态发生蛋白受体1B抗体 |
| 别 名 | 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. |
DATASHEET
Host:Rabbit
Target Protein:BMPR1B
IR:Immunogen Range:61-160/502
Clonality:Polyclonal
Isotype:IgG
Entrez Gene:658
Swiss Prot:O00238
Source:KLH conjugated synthetic peptide derived from human BMPR1B:61-160/502
Purification:affinity purified by Protein A
Storage:0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol. Shipped at 4℃. Store at -20 °C for one year. Avoid repeated freeze/thaw cycles.
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.
Size:100ul
Concentration:1mg/ml
Applications:WB(1:500-2000)
ELISA(1:5000-10000)
Cross Reactive Species:Human
Mouse
Rat
Dog
Cow
Rabbit
Sheep
..
For research use only. Not intended for diagnostic or therapeutic use.
VALIDATION IMAGES
Sample:
U251(human)cell Lysate at 30 ug
U87mg(human)cell Lysate at 30 ug
Primary: Anti- BMPR1B (bs-6639R)at 1/500 dilution
Secondary: IRDye800CW Goat Anti-Rabbit IgG at 1/20000 dilution
Predicted band size: 54kD
Observed band size: 56 kD
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