Alpha Dystroglycan Antibody (2238)

Immunohistochemistry: Alpha Dystroglycan Antibody (2238) [NBP1-49634] - Endothelial B-catenin GOF does not affect astrocytic endfoot polarization of a-dystroglycan (a-Dag) and Kir4.1 within the subfornical organ (SFO). Striatal BBB-vessel showing a polarized distribution of a-Dag and Kir4.1 in AC endfeet. Lumen is stained by Podxl (asterisk) (A). Coronal overview of the subfornical organ (SFO) (B); rectangular inset demarcates area for higher magnification in (C). Dashed lines outline SFO vessels. Scale bar show 2 um (A), 50 um (B), 10 um (C). Image collected and cropped by CiteAb from the following publication (elifesciences.org/articles/43818), licensed under a CC-BY license.

Endothelial beta -catenin GOF does not affect astrocytic endfoot polarization of alpha -dystroglycan ( alpha -Dag) and Kir4.1 within the subfornical organ (SFO).Striatal BBB-vessel showing a polarized distribution of alpha -Dag and Kir4.1 in AC endfeet. Lumen is stained by Podxl (asterisk) (A). Coronal overview of the subfornical organ (SFO) (B); rectangular inset demarcates area for higher magnification in (C). Dashed lines outline SFO vessels. Scale bar show 2 µm (A), 50 µm (B), 10 µm (C).

Immunocytochemistry/ Immunofluorescence: Alpha Dystroglycan Antibody (2238) [NBP1-49634] - Endothelial beta -catenin GOF does not affect astrocytic endfoot polarization of alpha -dystroglycan ( alpha -Dag) & Kir4.1 within the subfornical organ (SFO).Striatal BBB-vessel showing a polarized distribution of alpha -Dag & Kir4.1 in AC endfeet. Lumen is stained by Podxl (asterisk) (A). Coronal overview of the subfornical organ (SFO) (B); rectangular inset demarcates area for higher magnification in (C). Dashed lines outline SFO vessels. Scale bar show 2 µm (A), 50 µm (B), 10 µm (C). Image collected & cropped by CiteAb from the following publication (//pubmed.ncbi.nlm.nih.gov/30932814), licensed under a CC-BY license. Not internally tested by Novus Biologicals.

• Reactivity: Hu, Mu, Rt, Bv, Rb
• Applications: WB, ELISA, ICC/IF, IHC
• Clone: 2238
• Clonality: Monoclonal
• Host: Mouse
• Conjugate: Unconjugated
• Concentration: 0.1 mg/ml

Alpha Dystroglycan Antibody (2238) Summary

• Immunogen: Monoclonal antibody 2238 recognizes a glycoepitope unique to brain alpha-dystroglycan.
• Specificity: The monoclonal antibody 2238 is specific for a glycoepitope on brain bovine alpha-dystroglycan, which is absent on alpha-dystroglycan expressed in all other tissues.
• Isotype: IgG2b
• Clonality: Monoclonal
• Host: Mouse
• Gene: DAG1
• Purity: Protein A or G purified

Applications/Dilutions

• Dilutions:
  • Immunoassay
  • Immunocytochemistry/ Immunofluorescence
  • Immunohistochemistry 1:10-1:500
  • Immunohistochemistry-Paraffin 1:10-1:500
  • Western Blot 1:100-1:2000
• Application Notes: This antibody works in Western Blot, Immunoassay and Immunohistochemistry-Paraffin WB:it recognizes alpha-DG as protein of ~130 kD, especially after enrichment of the lysates for dystroglycans Use in Immunocytochemistry/immunofluorescence reported in scientific literature (PMID 27015747)

Reactivity Notes

Please note that this antibody is reactive to Mouse and derived from the same host, Mouse. Additional Mouse on Mouse blocking steps may be required for IHC and ICC experiments. Please contact Technical Support for more information.

Packaging, Storage & Formulations

• Storage: Store at 4C.
• Buffer: 0.2 um filtered solution in PBS and 0.1% BSA
• Preservative: 0.02% Sodium Azide
• Concentration: 0.1 mg/ml
• Purity: Protein A or G purified

Alternate Names for Alpha Dystroglycan Antibody (2238)

• A3a
• AGRNR
• DAG156DAG
• dystroglycan 1 (dystrophin-associated glycoprotein 1)
• dystroglycan
• Dystrophin-associated glycoprotein 1

Background

Monoclonal antibody 2238 recognizes a glycoepitope unique to brain alpha-dystroglycan. Alpha-dystroglycan (alpha-DG), also known as dystrophin-associated glycoprotein, is a laminin-binding protein of ~156 kDa (including glyco-groups). Alpha-DG is a component of the dystroglycan complex, which is involved in early development, morphogenesis and in the pathogenesis of muscular dystrophies. Alpha- and beta-DG are encoded by a single gene and are derived from a precursor polypeptide by posttranslational cleavage. Beta-DG is an integral membrane protein, whereas alpha-DG is membrane-associated through its noncovalent interaction with the extracellular domain of beta-DG. The alpha- and beta-DGs provide important physical linkages between components of basement membranes and cytoplasmic proteins that bind to the actin cytoskeleton. Alpha-DG is a heavily glycosylated, mucin-like protein anchored on the extracellular surface of the myotube, where it may provide linkage between the sarcolemma and extracellular matrix (ECM). Alpha-DG is expressed in a variety of fetal and adult tissues. Tissue-specific glycosylation modifies the laminin specificity of alpha-DG. The muscle and nonmuscle isoforms of dystroglycan differ by carbohydrate moieties but not protein sequence. Alpha-DG has been shown to colocalize with laminin in skeletal and cardiac muscle and a number of other cells including peripheral nerve, astrocytes, Purkinje neurons and kidney epithelium. Laminin-10/11 was shown to bind preferentially to brain alpha-DG. In Duchenne muscular dystrophy, the expression of alpha-DG is dramatically reduced leading to a loss of linkage between the sarcolemma and extracellular matrix, rendering muscle fibers more susceptible to necrosis. In the central nervous system, dystroglycan functions as a dual receptor for agrin and laminin-2 for instance in the Schwann cell membrane. Furthermore, defects in dystroglycan are central to the pathogenesis of structural and functional brain abnormalities seen in congenital muscular dystrophies (CMD).

Limitations

This product is for research use only and is not approved for use in humans or in clinical diagnosis. Primary Antibodies are guaranteed for 1 year from date of receipt.

Publications for Alpha Dystroglycan Antibody (NBP1-49634)(4)

Publications using NBP1-49634

• Hathazi D, Cox D, D'Amico A et al. INPP5K and SIL1 associated pathologies with overlapping clinical phenotypes converge through dysregulation of PHGDH Brain : a journal of neurology 2021-04-01 [PMID: 33792664] (IF/IHC, Human)
• Benz, F;Wichitnaowarat, V;Lehmann, M;Germano, RF;Mihova, D;Macas, J;Adams, RH;Taketo, MM;Plate, KH;Guerit, S;Vanhollebeke, B;Liebner, S; Low wnt/beta-catenin signaling determines leaky vessels in the subfornical organ and affects water homeostasis in mice Elife 2019-04-01 [PMID: 30932814] (ICC/IF, Mouse)
• Koeppen AH, Becker AB, Qian J et al. Friedreich Ataxia: Developmental Failure of the Dorsal Root Entry Zone. J. Neuropathol. Exp. Neurol. 2017-11-01 [PMID: 29044418] (Human)
• Annese T, Corsi P, Ruggieri S et al. Isolation and characterization of neural stem cells from dystrophic mdx mouse. Exp. Cell Res. 2016-03-22 [PMID: 27015747] (WB, ICC/IF, Mouse)

FAQs for Alpha Dystroglycan Antibody (NBP1-49634). (Showing 1 - 1 of 1 FAQs).

1. Is it applicable in frozen section muscle tissue for immunohistochemistry staining?
   • Our Alpha Dystroglycan antibody with catalogue number NBP1-49634 has been validated for IHC with paraffin-embedded tissue, however we have not yet tested it with frozen samples.

Bioinformatics

• Gene Symbol: DAG1
• Entrez:
  • 281439(Bovine)
  • 1605(Human)
  • 13138(Mouse)
  • 100009278(Rabbit)
  • 114489(Rat)
• Uniprot:
  • O18738(Bovine)