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骨形态发生蛋白受体1B抗体
  • 产品货号：
    BN41945R
  • 中文名称：
    骨形态发生蛋白受体1B抗体
  • 英文名称：
    Rabbit anti-BMPR1B Polyclonal antibody

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  • BN41945R-50ul

    50ul

    ¥1486.00

    交叉反应：nan(predicted:Human,Mouse,Rat,Dog,Cow,Rabbit,Sheep) 推荐应用：WB,ELISA

  • BN41945R-100ul

    100ul

    ¥2360.00

    交叉反应：nan(predicted:Human,Mouse,Rat,Dog,Cow,Rabbit,Sheep) 推荐应用：WB,ELISA

  • BN41945R-200ul

    200ul

    ¥3490.00

    交叉反应：nan(predicted:Human,Mouse,Rat,Dog,Cow,Rabbit,Sheep) 推荐应用：WB,ELISA

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.  
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, Dog, Cow, Rabbit, Sheep, .)
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.
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
储 存 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.
PubMedPubMed
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



Important Note:
This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.


























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