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BMPR1B Rabbit pAb(bs-6639R)-50

ul/100ul/200ul
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  • ¥1180 - 2800
  • Bioss已认证
  • bs-6639R
  • 2025年10月16日
  • 产品信息以Bioss网站为准
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      50ul/100ul/200ul

    规格:50ul产品价格:¥1180.0
    规格:100ul产品价格:¥1980.0
    规格:200ul产品价格:¥2800.0
    产品编号bs-6639R
    英文名称BMPR1B Rabbit pAb
    中文名称骨形态发生蛋白受体1B抗体
    英文别名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.
    产品应用WB=1:500-2000

    Not yet tested in other applications.
    Optimal working dilutions must be determined by the end user.

    交叉反应Human (Mouse, Rat, Dog, Cow, Rabbit, Sheep)
    抗体来源Rabbit
    免疫原KLH conjugated synthetic peptide derived from human BMPR1B
    亚型IgG
    性状Liquid
    纯化方法affinity purified by Protein A
    克隆类型Polyclonal
    理论分子量56 kDa
    浓度1mg/ml
    储存液0.01M TBS (pH7.4) with 1% BSA, 0.02% Proclin300 and 50% Glycerol.
    研究领域

    Epigenetics and Nuclear Signaling > Nuclear Signaling Pathways > SMADs

    Signal Transduction > Cytoskeleton / ECM > Extracellular Matrix > Structures > Bone

    Signal Transduction > Growth Factors/Hormones > TGF

    Signal Transduction > Protein Phosphorylation > Ser / Thr Kinases > Other Kinases

    Signal Transduction > Signaling Pathway > Nuclear Signaling > SMADs

    Stem Cells > Mesenchymal Stem Cells > Surface Molecules

    Stem Cells > Signaling Pathways > TGF beta > Surface Molecules

    亚细胞定位Membrane; Single-pass type I membrane protein.
    相似性Belongs to the protein kinase superfamily. TKL Ser/Thr protein kinase family. TGFB receptor subfamily.
    Contains 1 GS domain.
    Contains 1 protein kinase domain.
    功能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.
    保存条件Shipped at 4℃. Store at -20℃ 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.
    背景资料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.

     

    应用推荐稀释比例
    {WB}{1:500-2000}

     

    产品细节图片1
    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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    该产品被引用文献

    [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}

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    BMPR1B Rabbit pAb(bs-6639R)-50ul/100ul/200ul
    ¥1180 - 2800