05-593 | Anti-α-Dystroglycan Antibody, clone IIH6C4

WB: Rabbit skeletal muscle lysate was probed with anti-α-Dystroglycan
05-593
200 µL  
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      Overview

      Replacement Information

      Key Spec Table

      Species Reactivity Key Applications Host Format Antibody Type
      H, Gp, Ca, Rb, M, RWB, IF, IHC, InhibitionMAscitesMonoclonal Antibody
      Description
      Catalogue Number 05-593
      Brand Family Upstate
      Trade Name
      • Upstate
      Description Anti-α-Dystroglycan Antibody, clone IIH6C4
      Overview This product may be used for research purposes only. Diagnostic use of this product requires a license from the University of Iowa Research Foundation, 214 Technology Innovation Center, Iowa City, IA 52242
      Alternate Names
      • Dystrophin-associated glycoprotein 1
      • Large glycan
      • LARGE-glycan
      • dystrophin-associated glycoprotein-1
      • dystroglycan 1 (dystrophin-associated glycoprotein 1)
      • dystroglycan 1
      Background Information Dystroglycans are essential elements of the neuromuscular junction (NMJ). The gene for dystroglycan is expressed as a precursor protein that is post translationally cleaved into a 156 kDa extracellular peripheral membrane protein called alpha dystroglycan and a 43 kDa transmembrane protein, beta dystroglycan. The latter protein contains a PPxY motif that promotes binding to WW domain containing proteins, such as utrophin and dystrophin. Phosphorylation at tyrosine 892 within the PPxY motif may regulate c Src interactions with beta dystroglycan, as well as inhibit interactions with WW domain proteins. In skeletal muscle, beta dystroglycan is normally localized to the plasma membrane, however phosphorylation of Tyr892 leads to localization of beta dystroglycan to endosomal compartments along with c Src. Thus, phosphorylation at Tyr892 may have important roles in altering the localization of beta dystroglycan during NMJ formation.
      References
      Product Information
      Format Ascites
      Control
      • Rabbit skeletal muscle lysate.
      Presentation Mouse ascites, in PBS containing 0.05% sodium azide and 30% glycerol.
      Liquid at -20ºC.
      Applications
      Application This Anti-α-Dystroglycan Antibody, clone IIH6C4 is validated for use in IH, FUNC, WB for the detection of α-Dystroglycan.
      Key Applications
      • Western Blotting
      • Immunofluorescence
      • Immunohistochemistry
      • Inhibition
      Application Notes Inhibition of Laminin Binding to Dystroglycan: An independent lab has shown, in a nitrocellulose overlay experiment, that this antibody inhibits binding of 125I-laminin to dystroglycan (Ervasti, J., et al. (1993).

      Western Blot Analysis: A previous lot of this antibody was used on mouse muscle tissue lysate 3 months after shRNA induction, and in littermate controls (ctrl) and LARGE-null negative control (myd) (Goddeeris, M., et al. 2013, Nature).

      Immunofluorescence: A previous lot of this antibody was used to detect α-Dystroglycan /LARGE-glycan in mouse muscle tissue (Goddeeris, M., et al. 2013, Nature).
      Biological Information
      Immunogen Rabbit skeletal muscle membrane preparation. Clone IIH6C4.
      Clone IIH6C4
      Concentration Please refer to the Certificate of Analysis for the lot-specific concentration.
      Host Mouse
      Specificity This antibody recognizes α-Dystroglycan/LARGE-glycan, Mr 156 kDa.
      Isotype IgM
      Species Reactivity Human Guinea Pig Canine Rabbit Mouse Rat
      Species Reactivity Note Rabbit, human, mouse, rat, dog, and guinea pig.
      Antibody Type Monoclonal Antibody
      Entrez Gene Number
      Entrez Gene Summary Dystroglycan is a laminin binding component of the dystrophin-glycoprotein complex which provides a linkage between the subsarcolemmal cytoskeleton and the extracellular matrix. Dystroglycan 1 is a candidate gene for the site of the mutation in autosomal recessive muscular dystrophies. The dramatic reduction of dystroglycan 1 in Duchenne muscular dystrophy leads to a loss of linkage between the sarcolemma and extracellular matrix, rendering muscle fibers more susceptible to necrosis. Dystroglycan also functions as dual receptor for agrin and laminin-2 in the Schwann cell membrane. The muscle and nonmuscle isoforms of dystroglycan differ by carbohydrate moieties but not protein sequence.
      Gene Symbol
      • DAG1
      • 156DAG
      • AGRNR
      • alpha-dystroglycan
      • beta-dystroglycan
      • DAG
      • A3a
      Purification Method Unpurified
      UniProt Number
      UniProt Summary FUNCTION: SwissProt: Q14118 # Forms part of the dystrophin-associated protein complex (DAPC) which may link the cytoskeleton to the extracellular matrix. Alpha-dystroglycan functions as a laminin receptor. Binds to several types of arenaviruses. Is a target for the entry of Mycobacterium leprae into peripheral nerve Schwann cells.
      SIZE: 895 amino acids; 97581 Da
      SUBUNIT: Interacts with SGCD (By similarity). Interacts with AGR2 and AGR3.
      SUBCELLULAR LOCATION: Alpha-dystroglycan: Secreted, extracellular space. & Beta-dystroglycan: Cell membrane; Single- pass type I membrane protein.
      TISSUE SPECIFICITY: Expressed in a variety of fetal and adult tissues.
      Physicochemical Information
      Dimensions
      Materials Information
      Toxicological Information
      Safety Information according to GHS
      Safety Information
      Product Usage Statements
      Quality Assurance Routinely evaluated by western blot on rabbit skeletal muscle.

      Western Blot Analysis:
      A 1:1000-1:2000 dilution of this lot detected α-Dystroglycan/LARGE-glycan in rabbit skeletal muscle.
      Note: The use of WGA purified protein results in significantly cleaner blots and immunoprecipitates.
      Post-translational modification of dystroglycan causes band broadening.
      Usage Statement
      • Unless otherwise stated in our catalog or other company documentation accompanying the product(s), our products are intended for research use only and are not to be used for any other purpose, which includes but is not limited to, unauthorized commercial uses, in vitro diagnostic uses, ex vivo or in vivo therapeutic uses or any type of consumption or application to humans or animals.
      Storage and Shipping Information
      Storage Conditions Stable for 1 year at -20°C from date of receipt. For maximum recovery of product, centrifuge the vial prior to removing the cap.
      Packaging Information
      Material Size 200 µL
      Transport Information
      Supplemental Information
      Specifications

      Documentation

      MSDS

      Languages
      Deutschland

      Certificates of Analysis

      TitleLot Number
      Anti-#945;-Dystroglycan, clone IIH6C4 - 19703711970371
      Anti-#945;-Dystroglycan, clone IIH6C4 - DAM1754429DAM1754429
      Anti--Dystroglycan, clone IIH6C4 - 19924471992447
      Anti--Dystroglycan, clone IIH6C4 - 20645102064510
      Anti--Dystroglycan, clone IIH6C4 - 22009262200926
      Anti--Dystroglycan, clone IIH6C4 - 23225112322511
      Anti--Dystroglycan, clone IIH6C4 - DAM1636068DAM1636068
      Anti--Dystroglycan, clone IIH6C4 - DAM1687511DAM1687511
      Anti--Dystroglycan, clone IIH6C4 - DAM1718044DAM1718044
      Anti--Dystroglycan, clone IIH6C4 - DAM1792483DAM1792483
      Anti--Dystroglycan, clone IIH6C4 - JBC1878057JBC1878057
      Anti-a-Dystroglycan, clone IIH6C4 (mouse ascites IgM) - DAM1412899DAM1412899
      Anti-a-Dystroglycan, clone IIH6C4 - DAM1442519DAM1442519
      Anti-alpha-Dystroglycan, clone IIH6C4 - DAM1588207DAM1588207
      Anti-alpha;-Dystroglycan, clone IIH6C4 - 06120476370612047637
      Anti-alpha;-Dystroglycan, clone IIH6C4 - 2027420274
      Anti-alpha;-Dystroglycan, clone IIH6C4 - 2299122991
      Anti-alpha;-Dystroglycan, clone IIH6C4 - 2770227702
      Anti-alpha;-Dystroglycan, clone IIH6C4 - 3252932529
      Anti-alpha;-Dystroglycan, clone IIH6C4 - JBC1362765JBC1362765
      Anti-α-Dystroglycan, clone IIH6C4 - 23939172393917
      Anti-α-Dystroglycan, clone IIH6C4 - 24940182494018
      Anti-α-Dystroglycan, clone IIH6C4 -25246712524671
      Anti-α-Dystroglycan, clone IIH6C4 -25806832580683
      Anti-α-Dystroglycan, clone IIH6C4 -26072752607275

      References

      Reference overviewPub Med ID
      Pseudotype-dependent lentiviral transduction of astrocytes or neurons in the rat substantia nigra.
      Jason R Cannon,Thomas Sew,Laura Montero,Edward A Burton,J Timothy Greenamyre
      Experimental neurology 228 2011

      Show Abstract
      21056560
      Sub-physiological sarcoglycan expression contributes to compensatory muscle protection in mdx mice.
      Dejia Li, Chun Long, Yongping Yue, Dongsheng Duan, Dejia Li, Chun Long, Yongping Yue, Dongsheng Duan, Dejia Li, Chun Long, Yongping Yue, Dongsheng Duan, Dejia Li, Chun Long, Yongping Yue, Dongsheng Duan
      Human molecular genetics 18 1209-20 2009

      Show Abstract Full Text Article
      19131360
      Mice Lacking Dystrophin or {alpha} Sarcoglycan Spontaneously Develop Embryonal Rhabdomyosarcoma with Cancer-Associated p53 Mutations and Alternatively Spliced or Mutant Mdm2 Transcripts.
      Fernandez K, Serinagaoglu Y, Hammond S, Martin LT, Martin PT
      The American journal of pathology 176 416-34 2009

      Show Abstract
      20019182
      Dystrophic phenotype induced in vitro by antibody blockade of muscle alpha-dystroglycan-laminin interaction
      Brown, S. C., et al
      J Cell Sci, 112 ( Pt 2):209-16 (1999) 1999

      9858474
      Dystroglycan in the cerebellum is a laminin alpha 2-chain binding protein at the glial-vascular interface and is expressed in Purkinje cells.
      Tian, M, et al.
      Eur. J. Neurosci., 8: 2739-47 (1996) 1996

      Show Abstract
      8996823
      Dystroglycan is a binding protein of laminin and merosin in peripheral nerve.
      Yamada, H, et al.
      FEBS Lett., 352: 49-53 (1994) 1994

      Show Abstract
      7925941
      A role for the dystrophin-glycoprotein complex as a transmembrane linker between laminin and actin.
      Ervasti, J M and Campbell, K P
      J. Cell Biol., 122: 809-23 (1993) 1993

      Show Abstract
      8349731
      Primary structure of dystrophin-associated glycoproteins linking dystrophin to the extracellular matrix.
      Ibraghimov-Beskrovnaya, O, et al.
      Nature, 355: 696-702 (1992) 1992

      1741056
      Membrane organization of the dystrophin-glycoprotein complex.
      Ervasti, J M and Campbell, K P
      Cell, 66: 1121-31 (1991) 1991

      Show Abstract
      1913804

      Related Products & Applications

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       Catalogue Number Description  
      05-593-KC Anti-α-Dystroglycan, clone IIH6C4 (1mL) KC Show Pricing & Availability

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      Categories

      Life Science Research > Antibodies and Assays > Primary Antibodies