Rabbit Anti-BMPR1B antibody |
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(可尝试) |
ELISA |
推荐稀释比例 |
WB=1:500-2000,Elisa=1:5000-10000, |
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, |
标签 |
Array |
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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
RRID:AB_11053330
产品名称:Rabbit Anti-BMPR1B antibody
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
中文名称:骨形态发生蛋白受体1B抗体
英文名称:Rabbit Anti-BMPR1B antibody
抗体来源: Rabbit
克隆类型:多克隆
细胞定位:细胞膜
性 状:Liquid
亚 型:IgG
纯化方法:affinity purified by Protein A
保存条件:Shipped at 4℃. Store at -20 °C for one year. Avoid repeated freeze/thaw cycles.
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
SWISS:O00238
Gene ID :658
Human Gene ID:658
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
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 malformat
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.
Important Note:This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.