规格 | 价格 | 库存 |
---|---|---|
50ul | ¥ 1200 | 9 |
100ul | ¥ 1900 | 5 |
200ul | ¥ 2900 | 2 |
产品编号 | Ys-21783R |
英文名称 | ACVRL1 |
中文名称 | 激活素受体样激酶1抗体 |
别 名 | Activin A receptor; Activin A receptor type II like 1; Activin receptor like kinase 1; Activin receptor-like kinase 1; ACVL1_HUMAN; ACVRL1; ACVRLK1; ALK-1; ALK1; HHT; HHT2; ORW2; Osler Rendu Weber syndrome 2; Serine/threonine protein kinase receptor R3; Serine/threonine-protein kinase receptor R3; SKR3; TGF B superfamily receptor type I; TGF-B superfamily receptor type I; TSR-I; TSR1. |
抗体来源 | Rabbit |
克隆类型 | Polyclonal |
交叉反应 | Mouse, (predicted: Human, Rat, ) |
产品应用 | WB=1:500-2000 IHC-P=1:100-500 IHC-F=1:100-500 ICC=1:100-500 IF=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. |
理论分子量 | 53kDa |
细胞定位 | 细胞膜 |
性 状 | Liquid |
浓 度 | 1mg/ml |
免 疫 原 | KLH conjugated synthetic peptide derived from human ACVRL1 : 22-118/503 |
亚 型 | IgG |
纯化方法 | affinity purified by Protein A |
缓 冲 液 | 0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol. |
保存条件 | Shipped at 4℃. Store at -20 °C for one year. Avoid repeated freeze/thaw cycles. |
注意事项 | This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications. |
PubMed | PubMed |
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 TGF-beta. May bind activin as well. 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 TGF-beta. May bind activin as well. Subcellular Location: Membrane. DISEASE: Defects in ACVRL1 are the cause of hereditary hemorrhagic telangiectasia type 2 (HHT2) [MIM:600376]; also known as Osler-Rendu-Weber syndrome 2 (ORW2). HHT2 is an autosomal dominant multisystemic vascular dysplasia, characterized by recurrent epistaxis, muco-cutaneous telangiectases, gastro-intestinal hemorrhage, and pulmonary, cerebral and hepatic arteriovenous malformations; all secondary manifestations of the underlying vascular dysplasia. 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. |