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Anti-ACVRL1 antibody (ab10179)

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Overview

Product name

Anti-ACVRL1 antibody
See all ACVRL1 products (7) ...

Description

Goat polyclonal to ACVRL1

Tested applications

WBmore details

Cross reactivity

Reacts with

Human

Immunogen

Synthetic peptide: KISNSPEKPKVIQ, corresponding to C terminal amino acids 491-503 of Human ACVRL1.

C-KISNSPEK PKVIQ

Positive control

Lung and liver extracts.

Properties

Form

Liquid

Storage instructions

Shipped at 4°C. Upon delivery aliquot and store at -20°C or -80°C. Avoid repeated freeze / thaw cycles.

Storage buffer

Preservative: 0.02% Sodium Azide
Constituents: 0.5% BSA, Tris-saline. pH 7.3

Concentration

Concentration information loading...

Purity

Immunogen affinity purified

Purification notes

Purified 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

Isotype

IgG

  • Western blot - Anti-ACVRL1 antibody (ab10179)Western blot - Anti-ACVRL1 antibody (ab10179) image (enlarge)

Applications

Show applications key

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.

  • ShowHide1 Image

    WB

     WB: Use a concentration ...Read more →

    WB: Use a concentration of 1 - 3 µg/ml.Detects a band of approximately 60 kDa (predicted molecular weight: 56 kDa).

Target

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.

Involvement in 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.

Sequence similarities

Belongs to the protein kinase superfamily. TKL Ser/Thr protein kinase family. TGFB receptor subfamily.
Contains 1 GS domain.
Contains 1 protein kinase domain.

Cellular localization

Membrane.

Target information above from: UniProt accessionP37023 The UniProt Consortium
The Universal Protein Resource (UniProt) in 2010
Nucleic Acids Res. 38:D142-D148 (2010).

Information by UniProt

Alternative names

  • Activin A receptor antibody
  • Activin A receptor type II like 1 antibody
  • Activin receptor like kinase 1 antibody
  • Activin receptor-like kinase 1 antibody
  • ACVL1_HUMAN antibody
  • ACVRL1 antibody
  • ACVRLK1 antibody
  • ALK-1 antibody
  • ALK1 antibody
  • HHT antibody
  • HHT2 antibody
  • ORW2 antibody
  • Osler Rendu Weber syndrome 2 antibody
  • Serine/threonine protein kinase receptor R3 antibody
  • Serine/threonine-protein kinase receptor R3 antibody
  • SKR3 antibody
  • TGF B superfamily receptor type I antibody
  • TGF-B superfamily receptor type I antibody
  • TSR-I antibody
  • TSR1 antibody
see all

Anti-ACVRL1 antibody images:

  Western blot - Anti-ACVRL1 antibody (ab10179)

Western blot - Anti-ACVRL1 antibody (ab10179)

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.

Publishing research using ab10179? Please let us know so that we can cite the reference in this datasheet

Please note: All products are "FOR RESEARCH USE ONLY AND ARE NOT INTENDED FOR DIAGNOSTIC OR THERAPEUTIC USE"