|
|
货号 | bs-6639R-1 |
品牌 | |
浓度 | |
货期 | 现货 |
英文名称 | Rabbit Anti-BMPR1B antibody |
中文名称 | Rabbit Anti-BMPR1B antibody |
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, |
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 protei |
反应物种(预测) | Human,Mouse,Rat,Dog,Cow,Rabbit,Sheep |
产品应用(已验证) | WB |
产品应用(可推荐) | ELISA |
推荐稀释比例 | WB=1:500-2000,Elisa=1:5000-10000, |
克隆类型 | 多克隆 |
抗体来源 | Rabbit |
理论分子量 | 56 |
细胞定位 | 细胞膜 |
性状 | Liquid |
免疫原 | KLH conjugated synthetic peptide derived from human BMPR1B |
抗原表位 | 61-160/502 |
抗原细胞定位 | Extracellular |
亚型 | IgG |
纯化方法 | affinity purified by Protein A |
SUBCELLULAR | Membrane; Single-pass type I membrane protein. |
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. |
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 |
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 malformat |
SWISS | O00238 |
Gene ID | 658 |
保存条件 | Shipped at 4℃. Store at -20 °C for one year. Avoid repeated freeze/thaw cycles. |
Important Note | This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications. |
英文介绍 | 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. |