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Rabbit Anti-BMPR1B antibody (bs-6639R)
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说明书: 50ul  100ul  200ul
50ul/1180.00元
100ul/1980.00元
200ul/2800.00元
大包装/询价

产品编号 bs-6639R
英文名称 Rabbit Anti-BMPR1B antibody
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.  
Specific References  (1)     |     bs-6639R has been referenced in 1 publications.
[IF=3.688] Jianshu Lv. et al. Regulatory role of dihydrotestosterone on BMP-6 receptors in granular cells of sheep antral follicles. Gene. 2021 Nov;:146066  WB,IF,IHC ;  Sheep.  
研究领域 细胞生物  信号转导  干细胞  转录调节因子  激酶和磷酸酶  细胞表面分子  细胞外基质  表观遗传学  
抗体来源 Rabbit
克隆类型 Polyclonal
交叉反应 (predicted: Human,Mouse,Rat,Rabbit,Sheep,Cow,Dog)
产品应用 WB=1:500-2000,ELISA=1:5000-10000
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
理论分子量 56kDa
细胞定位 细胞膜 
性    状 Liquid
浓    度 1mg/ml
免 疫 原 KLH conjugated synthetic peptide derived from human BMPR1B: 61-160/502 <Extracellular>
亚    型 IgG
纯化方法 affinity purified by Protein A
缓 冲 液 0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol.
保存条件 Shipped at 4℃. Store at -20 °C for one year. Avoid repeated freeze/thaw cycles.
注意事项 This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
PubMed PubMed
产品介绍 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.

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 BMP7/OP-1 and GDF5.

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

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).
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.

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

SWISS:
O00238

Gene ID:
658

Database links:

Entrez Gene: 658 Human

Entrez Gene: 12167 Mouse

Omim: 603248 Human

SwissProt: O00238 Human

SwissProt: P36898 Mouse

Unigene: 598475 Human

Unigene: 39089 Mouse



产品图片
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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