Overview

  • Product name

    Anti-Alpha Dystroglycan antibody
    See all Alpha Dystroglycan primary antibodies
  • Description

    Rabbit polyclonal to Alpha Dystroglycan
  • Host species

    Rabbit
  • Tested applications

    Suitable for: WB, ICC/IFmore details
  • Species reactivity

    Reacts with: Human
  • Immunogen

    Recombinant fragment, corresponding to a region within amino acids 471-752 of Human Alpha Dystroglycan (UniProt: Q14118).

  • Positive control

    • U-87 MG whole cell lysate; A431 cells.

Properties

Applications

Our Abpromise guarantee covers the use of ab151979 in the following tested applications.

The application notes include recommended starting dilutions; optimal dilutions/concentrations should be determined by the end user.

Application Abreviews Notes
WB 1/500 - 1/3000. Predicted molecular weight: 97 kDa.
ICC/IF 1/100 - 1/1000.

Target

  • Function

    The dystroglycan complex is involved in a number of processes including laminin and basement membrane assembly, sarcolemmal stability, cell survival, peripheral nerve myelination, nodal structure, cell migration, and epithelial polarization.
    Alpha-dystroglycan is an extracellular peripheral glycoprotein that acts as a receptor for both extracellular matrix proteins containing laminin-G domains, and for certain adenoviruses. Receptor for laminin-2 (LAMA2) and agrin in peripheral nerve Schwann cells. Also acts as a receptor for M.leprae in peripheral nerve Schwann cells but only in the presence of the G-domain of LAMA2, and for lymphocytic choriomeningitis virus, Old World Lassa fever virus, and clade C New World arenaviruses.
    Beta-dystroglycan is a transmembrane protein that plays important roles in connecting the extracellular matrix to the cytoskeleton. Acts as a cell adhesion receptor in both muscle and non-muscle tissues. Receptor for both DMD and UTRN and, through these interactions, scaffolds axin to the cytoskeleton. Also functions in cell adhesion-mediated signaling and implicated in cell polarity.
  • Tissue specificity

    Expressed in a variety of fetal and adult tissues. In epidermal tissue, located to the basement membrane. Also expressed in keratinocytes and fibroblasts.
  • Involvement in disease

    Defects in DAG1 are the cause of muscular dystrophy-dystroglycanopathy limb-girdle type C7 (MDDGC7) [MIM:613818]. An autosomal recessive muscular dystrophy showing onset in early childhood, and associated with mental retardation without structural brain anomalies. Note=MDDGC7 is caused by DAG1 mutations that interfere with normal post-translational processing, resulting in defective DAG1 glycosylation and impaired interactions with extracellular-matrix components. Other muscular dystrophy-dystroglycanopathies are caused by defects in enzymes involved in protein O-glycosylation.
  • Sequence similarities

    Contains 1 peptidase S72 domain.
  • Post-translational
    modifications

    O- and N-glycosylated. Alpha-dystroglycan is heavily O-glycosylated comprising of up to two thirds of its mass and the carbohydrate composition differs depending on tissue type. Mucin-type O-glycosylation is important for ligand binding activity. O-mannosylation of alpha-DAG1 is found in high abundance in both brain and muscle where the most abundant glycan is Sia-alpha-2-3-Gal-beta-1-4-Glc-NAc-beta-1-2-Man. In muscle, glycosylation on Thr-379 by a phosphorylated O-mannosyl glycan with the structure 2-(N-acetylamido)-2-deoxygalactosyl-beta-1,3-2-(N-acetylamido)-2-deoxyglucosyl-beta-1,4-6-phosphomannose is mediated by like-acetylglucosaminyltransferase (LARGE) protein and is required for laminin binding. O-mannosylation is also required for binding lymphocytic choriomeningitis virus, Old World Lassa fever virus, and clade C New World arenaviruses. The O-glycosyl hexose on Thr-367, Thr-369, Thr-372, Thr-381 and Thr-388 is probably mannose. O-glycosylated in the N-terminal region with a core 1 or possibly core 8 glycan. The beta subunit is N-glycosylated.
    Autolytic cleavage produces the alpha and beta subunits. In cutaneous cells, as well as in certain pathological conditions, shedding of beta-dystroglcan can occur releasing a peptide of about 30 kDa.
    SRC-mediated phosphorylation of the PPXY motif of the beta subunit recruits SH2 domain-containing proteins, but inhibits binding to WWW domain-containing proteins, DMD and UTRN. This phosphorylation also inhibits nuclear entry.
  • Cellular localization

    Secreted > extracellular space and Cell membrane. Cytoplasm > cytoskeleton. Nucleus > nucleoplasm. The monomeric form translocates to the nucleus via the action of importins and depends on RAN. Nuclear transport is inhibited by Tyr-892 phosphorylation. In skeletal muscle, this phosphorylated form locates to a vesicular internal membrane compartment. In peripheral nerves, localizes to the Schwann cell membrane. Colocalizes with ERM proteins in Schwann-cell microvilli.
  • Information by UniProt
  • Database links

  • Alternative names

    • 156DAG antibody
    • A3a antibody
    • AGRNR antibody
    • Alpha dystroglycan antibody
    • Alpha-DG antibody
    • Beta-DG antibody
    • Beta-dystroglycan antibody
    • DAG antibody
    • Dag1 antibody
    • DAG1_HUMAN antibody
    • Dystroglycan 1 (dystrophin associated glycoprotein 1) antibody
    • Dystroglycan antibody
    • Dystrophin associated glycoprotein 1 antibody
    • Dystrophin-associated glycoprotein 1 antibody
    • OTTHUMP00000210857 antibody
    • OTTHUMP00000210858 antibody
    see all

Images

  • Anti-Alpha Dystroglycan antibody (ab151979) at 1/500 dilution + U-87 MG whole cell lysate at 30 µg

    Predicted band size: 97 kDa



    7.5% SDS PAGE
  • Immunofluorescent analysis of paraformaldehyde-fixed A431 cells, labeling Alpha Dystroglycan with ab151979 at 1/500 dilution. Lower image shows cells co-stained with Hoechst 33342.

References

This product has been referenced in:

  • Krag TO & Vissing J A New Mouse Model of Limb-Girdle Muscular Dystrophy Type 2I Homozygous for the Common L276I Mutation Mimicking the Mild Phenotype in Humans. J Neuropathol Exp Neurol 74:1137-46 (2015). WB ; Mouse . Read more (PubMed: 26574668) »
See 1 Publication for this product

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