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| 规格： | 20ug | 产品价格： | ￥1280.0 |
|---|---|---|---|
| 规格： | 50ug | 产品价格： | ￥2580.0 |
| 规格： | 100ug | 产品价格： | ￥4180.0 |
| 产品编号 | bs-103105P |
| 英文名称 | Recombinant Human CDw293/BMPR1B Protein, C-Fc |
| 英文别名 | BMP type-1B receptor; BMPR1B; CDw293; BMPR-1B; Bone morphogenetic protein receptor type-1B |
| 性状 | Lyophilized |
| 纯化方法 | AC |
| 理论分子量 | 41.34 kDa |
| 储存液 | Lyophilized from a solution in PBS pH 7.4, 1mM EDTA, 4% Trehalose, 1% Mannitol. |
| 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._x000D_
Contains 1 GS domain._x000D_ 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. _x000D_ Involvement in disease; _x000D_ 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). _x000D_ 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 | Use a manual defrost freezer and avoid repeated freeze thaw cycles. Store at 2 to 8°C for frequent use. Store at -20 to -80°C for twelve months from the date of receipt. |
| 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. |
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文献和实验may become powerful tools in anti-cancer therapeutics. Lef1 contributes to the differentiation of bulge stem cells by nuclear translocation and cross-talk with the notch signaling pathway. 作者：Zhang Y, Yu J, Shi C, Huang Y, Wang Y, Yang T, Yang J. Lymphoid
.de/ Integrated database of protein families, EST clusters and their genomic positions 292. TMPDB http://bioinfo.si.hirosaki-u.ac.jp/~TMPDB/ Experimentally-characterized transmembrane topologies l Proteome Resources 293. AAindex http://www.genome.ad
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