Rabbit Anti-BMPR1B Polyclonal Antibody
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:50ul
Concentration:1mg/ml
Applications:WB(1:500-2000)
ELISA(1:5000-10000)
Cross Reactive Species:Human
Mouse
Rat
Dog
Cow
Rabbit
Sheep
仅供科研使用。不能用于人和动物治疗等其它临床诊断用！
关键字： BMPR1B;Activin receptor lik;Acvrlk6;ALK6;alk6tr;BMP type-1B receptor;CDw293;BMR1B_HUMAN;
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