Target：ALK

Fields：Cytokine-cytokine receptor interaction

Gene Name：ACVRL1

Protein Name：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

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

Specificity：ALK-1 Polyclonal Antibody detects endogenous levels of ALK-1 protein.

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

Source：Polyclonal, Rabbit,IgG

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

Purification：The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen.

Concentration：1 mg/ml

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

Other Name：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

Observed Band(KD)：56kD

Background： 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],

Function：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

Subcellular Location：Cell membrane ; Single-pass type I membrane protein .

Expression： Brain,Placenta,