• 靶点：
• ALK

• 简介：
• >>Cytokine-cytokine receptor interaction

• 基因名称：
• ACVRL1

• 蛋白名称：
• Serine/threonine-protein kinase receptor R3

• Human Gene Id：
• 94

• Human Swiss Prot No：
• P37023

• Mouse Gene Id：
• 11482

• Mouse Swiss Prot No：
• Q61288

• Rat Gene Id：
• 25237

• Rat Swiss Prot No：
• P80203

• 免疫原：
• The antiserum was produced against synthesized peptide derived from the N-terminal region of human ACVRL1. AA range:21-70

• 特异性：
• ALK-1 Polyclonal Antibody detects endogenous levels of ALK-1 protein.

• 组成：
• Liquid in PBS containing 50% glycerol, 0.5% BSA and 0.02% sodium azide.

• 来源：
• Polyclonal, Rabbit,IgG

• 稀释：
• WB 1:500 - 1:2000. IHC: 1:100-300 ELISA: 1:20000.. IF 1:50-200

• 纯化工艺：
• The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen.

• 浓度：
• 1 mg/ml

• 储存：
• -15°C to -25°C/1 year(Do not lower than -25°C)

• 其他名称：
• ACVRL1;ACVRLK1;ALK1;Serine/threonine-protein kinase receptor R3;SKR3;Activin receptor-like kinase 1;ALK-1;TGF-B superfamily receptor type I;TSR-I

• 实测条带：
• 56kD

• 背景：
• This gene encodes a type I cell-surface receptor for the TGF-beta superfamily of ligands. It shares with other type I receptors a high degree of similarity in serine-threonine kinase subdomains, a glycine- and serine-rich region (called the GS domain) preceding the kinase domain, and a short C-terminal tail. The encoded protein, sometimes termed ALK1, shares similar domain structures with other closely related ALK or activin receptor-like kinase proteins that form a subfamily of receptor serine/threonine kinases. Mutations in this gene are associated with hemorrhagic telangiectasia type 2, also known as Rendu-Osler-Weber syndrome 2. [provided by RefSeq, Jul 2008],

• 功能：
• catalytic activity:ATP + [receptor-protein] = ADP + [receptor-protein] phosphate.,cofactor:Magnesium or manganese.,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.,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.,simi

• 细胞定位：
• Cell membrane ; Single-pass type I membrane protein .

• 组织表达：
• Brain,Placenta,

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