骨形态发生蛋白受体1B抗体Anti-BMPR1B
360TIYO-上海田源公司

性能参数：

中文名称：骨形态发生蛋白受体1B抗体
英文名称：Anti-BMPR1B
别名：BMPR-IB;Activin receptor like kinase 6; Acvrlk6; ALK 6; ALK6; alk6tr; BMP type...
产品货号：
包装号：G320
产地：中国大陆/进口产品
商标/品牌：TIUE、TIYO、新试剂、田源试剂
供应商：上海田源试剂、新试剂
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产品详细描述

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.

骨形态发生蛋白受体1B抗体的质量标准：

浓度：1mg/1ml
交叉反应：hu
产品类型：一抗
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
蛋白分子量：predicted molecular weight: 57kDa
性状：Lyophilized or Liquid
免疫原：KLH conjugated synthetic peptide derived from hu BMPR1B
亚型：polyclonal
纯化方法：affinity purified by Protein A
储存液：Preservative: 15mM Sodium Azide, Constituents: 1% BSA, 0.01M PBS, pH 7.4
产品应用：WB=1:100-500ELISA=1:500-1000IP=1:20-100IHC-P=1:100-500IHC-F=1:100-500IF=1:100-500 （石蜡切片需做抗原修复） not yet tested in other applications. optimal dilutions/concentrations should be determined by the end user.
保存条件：Store at -20 °C for one year. Avoid repeated freeze/thaw cycles. The lyophilized antibody is stable at room temperature for at least one month ａnd for greater than a year when kept at -20°C. When reconstituted in sterile pH 7.4 0.01M PBS or diluent of antibody the antibody is stable for at least two weeks at 2-4 °C.
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（1mg/1ml骨形态发生蛋白受体1B抗体Anti-BMPR1B，BMPR-IB;Activin receptor like kinase 6; Acvrlk6; ALK 6; ALK6; alk6tr; BMP type...的来源为Rabbit，其相关产品：免疫组化常用试剂免疫印迹常用试剂各种标记二抗）

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