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Upingbio

SKU:YP-Ab-13719-100UL

ALK-1 Polyclonal Antibody

ALK-1 Polyclonal Antibody

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  • Gene Name: ACVRL1
  • 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.
  • Composition: Liquid in PBS containing 50% glycerol, 0.5% BSA and 0.02% sodium azide.
  • Source: Polyclonal, Rabbit,IgG
  • Dilution ratio: WB: 1/500 - 1/2000. IHC-p: 1:100-300 ELISA: 1/20000.. IF 1:50-200
  • Purification process: The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen.
  • Concentration: 1 mg/ml
  • Storage: -20°C/1 year
  • Other Names: 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
  • 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
  • Species: Human;Mouse;Rat
  • Range: WB;IHC;IF;ELISA
  • Protein: Serine/threonine-protein kinase receptor R3
  • DA: 56kD
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