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    BMPR1B Rabbit pAb (bs-6639R)  
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    產(chǎn)品編號 bs-6639R
    英文名稱(chēng) BMPR1B Rabbit pAb
    中文名稱(chēng) 骨形態(tài)發(fā)生蛋白受體1B抗體
    別    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.  
    研究領(lǐng)域 細胞生物  信號轉導  干細胞  轉錄調節因子  激酶和磷酸酶  細胞表面分子  細胞外基質(zhì)  表觀(guān)遺傳學(xué)  
    抗體來(lái)源 Rabbit
    克隆類(lèi)型 Polyclonal
    克 隆 號
    交叉反應 Human (predicted: Mouse,Rat,Rabbit,Sheep,Cow,Dog)
    產(chǎn)品應用 WB=1:500-2000
    not yet tested in other applications.
    optimal dilutions/concentrations should be determined by the end user.
    理論分子量 56 kDa
    檢測分子量
    細胞定位 細胞膜 
    性    狀 Liquid
    濃    度 1mg/ml
    免 疫 原 KLH conjugated synthetic peptide derived from human BMPR1B: 61-160/502 <Extracellular>
    亞    型 IgG
    純化方法 affinity purified by Protein A
    緩 沖 液 0.01M TBS (pH7.4) with 1% BSA, 0.02% Proclin300 and 50% Glycerol.
    保存條件 Shipped at 4℃. Store at -20℃ for one year. Avoid repeated freeze/thaw cycles.
    注意事項 This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
    PubMed PubMed
    產(chǎn)品介紹 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



    產(chǎn)品圖片
    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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