Anti-ACVRL1 antibody (ab10179)
- Product nameAnti-ACVRL1 antibodySee all ACVRL1 primary antibodies ...
- DescriptionGoat polyclonal to ACVRL1
- Tested applicationsWB more details
- Species reactivityReacts with: Human
Synthetic peptide: KISNSPEKPKVIQ, corresponding to C terminal amino acids 491-503 of Human ACVRL1.
- Positive controlLung and liver extracts.
- General notes
Activin A receptor, type II-like 1 is a type I cell-surface receptor for the TGF-beta superfamily of ligands. ACVRL1 is most highly expressed in human placenta and lung. ACVRL1 deficiency causes hemorrhagic telangiectasia type 2 (HHT2; MIM 600376 ) also known as Rendu-Osler-Weber syndrome 2 (ORW2). Note: ACVRL1 is also known as ALK1 - not to be confused with the similarly named ALK (anaplastic lymphoma kinase).
- Storage instructionsShipped at 4°C. Upon delivery aliquot and store at -20°C or -80°C. Avoid repeated freeze / thaw cycles.
- Storage bufferPreservative: 0.02% Sodium Azide
Constituents: 0.5% BSA, Tris-saline. pH 7.3
- Concentration information loading...
- PurityImmunogen affinity purified
- Purification notesPurified from goat serum by ammonium sulphate precipitation followed by antigen affinity chromatography using the immunizing peptide.
- Primary antibody notes Activin A receptor, type II-like 1 is a type I cell-surface receptor for the TGF-beta superfamily of ligands. ACVRL1 is most highly expressed in human placenta and lung. ACVRL1 deficiency causes hemorrhagic telangiectasia type 2 (HHT2; MIM 600376 ) also known as Rendu-Osler-Weber syndrome 2 (ORW2). Note: ACVRL1 is also known as ALK1 - not to be confused with the similarly named ALK (anaplastic lymphoma kinase).
- Clonality Polyclonal
Our Abpromise guarantee covers the use of ab10179 in the following tested applications.
The application notes include recommended starting dilutions; optimal dilutions/concentrations should be determined by the end user.
|WB||WB: Use a concentration of 1 - 3 µg/ml. Detects a band of approximately 60 kDa (predicted molecular weight: 56 kDa).Can be blocked with ACVRL1 peptide (491-503) (ab23196).|
- FunctionOn 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.
- Involvement in diseaseDefects 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.
- Sequence similaritiesBelongs to the protein kinase superfamily. TKL Ser/Thr protein kinase family. TGFB receptor subfamily.
Contains 1 GS domain.
Contains 1 protein kinase domain.
- Cellular localizationMembrane.
- Activin A receptor antibodyActivin A receptor type II like 1 antibodyActivin A receptor, type II like kinase 1 antibody
- Activin receptor like kinase 1 antibodyActivin receptor-like kinase 1 antibodyACVL1_HUMAN antibodyACVRL1 antibodyACVRLK1 antibodyALK-1 antibodyALK1 antibodyHHT antibodyHHT2 antibodyORW2 antibodyOsler Rendu Weber syndrome 2 antibodySerine/threonine protein kinase receptor R3 antibodySerine/threonine-protein kinase receptor R3 antibodySKR3 antibodyTGF B superfamily receptor type I antibodyTGF-B superfamily receptor type I antibodyTSR-I antibodyTSR1 antibody
Anti-ACVRL1 antibody images
Anti-ACVRL1 antibody (ab10179) at 2 µg/ml + MCF7 lysate at 35 µg
developed using the ECL technique
Predicted band size : 56 kDa
Observed band size : 60 kDa (why is the actual band size different from the predicted?)
References for Anti-ACVRL1 antibody (ab10179)
ab10179 has not yet been referenced specifically in any publications.