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

WB: Rabbit skeletal muscle lysate was probed with anti-α-Dystroglycan
05-593
200  µL
Retrieving price...
Price could not be retrieved
Upon Order Completion More Information
You Saved ()
 
Request Pricing
Limited Availability Limited Availability
Discontinued
There is a newer version of this product
Limited Quantities Available
Availability to be confirmed
      Will advise

      Special Offers

       

      Contact Customer Service

         CompareClick To Print This Page

      Overview

      Key Spec Table

      Species ReactivityKey ApplicationsHostFormatAntibody Type
      Gp, H, R, M, Rb, CaIF, WB, Inhibition, IHCMAscitesMonoclonal Antibody
      Description
      Catalogue Number 05-593
      Brand Family Upstate
      Synonyms Large glycan LARGE-glycan dystrophin-associated glycoprotein-1 dystroglycan 1 (dystrophin-associated glycoprotein 1) dystroglycan 1 Dystrophin-associated glycoprotein 1
      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
      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
      Specificity This antibody recognizes α-Dystroglycan/LARGE-glycan, Mr 156 kDa.
      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
      • Immunofluorescence
      • Western Blotting
      • Inhibition
      • Immunohistochemistry
      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 species 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 Guinea Pig Human Rat Mouse Rabbit Canine
      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
      • 156DAG
      • DAG1
      • A3a
      • DAG
      • beta-dystroglycan
      • alpha-dystroglycan
      • AGRNR
      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
      Qty/Pck 200 µL
      Material Size 200
      Material Package µL
      Transport Information
      Supplemental Information
      Specifications

      Documentation

      Certificates of Analysis

      TitleLot Number
      Anti--Dystroglycan, clone IIH6C4 - 2322511 2322511
      Anti-α-Dystroglycan, clone IIH6C4 - 2393917 2393917
      Millipore - COA - Anti-#945;-Dystroglycan, clone IIH6C4 - 1970371 1970371
      Millipore - COA - Anti-#945;-Dystroglycan, clone IIH6C4 - DAM1754429 DAM1754429
      Millipore - COA - Anti--Dystroglycan, clone IIH6C4 - 1992447 1992447
      Millipore - COA - Anti--Dystroglycan, clone IIH6C4 - 2064510 2064510
      Millipore - COA - Anti--Dystroglycan, clone IIH6C4 - 2200926 2200926
      Millipore - COA - Anti--Dystroglycan, clone IIH6C4 - DAM1636068 DAM1636068
      Millipore - COA - Anti--Dystroglycan, clone IIH6C4 - DAM1687511 DAM1687511
      Millipore - COA - Anti--Dystroglycan, clone IIH6C4 - DAM1718044 DAM1718044
      Millipore - COA - Anti--Dystroglycan, clone IIH6C4 - DAM1792483 DAM1792483
      Millipore - COA - Anti--Dystroglycan, clone IIH6C4 - JBC1878057 JBC1878057
      Millipore - COA - Anti-a-Dystroglycan, clone IIH6C4 (mouse ascites IgM) - DAM1412899 DAM1412899
      Millipore - COA - Anti-a-Dystroglycan, clone IIH6C4 - DAM1442519 DAM1442519
      Millipore - COA - Anti-alpha-Dystroglycan, clone IIH6C4 - DAM1588207 DAM1588207
      Millipore - COA - Anti-alpha;-Dystroglycan, clone IIH6C4 - JBC1362765 JBC1362765
      Millipore - COAs - Anti-alpha;-Dystroglycan, clone IIH6C4 - 0612047637 0612047637
      Millipore - COAs - Anti-alpha;-Dystroglycan, clone IIH6C4 - 20274 20274
      Millipore - COAs - Anti-alpha;-Dystroglycan, clone IIH6C4 - 22991 22991
      Millipore - COAs - Anti-alpha;-Dystroglycan, clone IIH6C4 - 27702 27702
      Millipore - COAs - Anti-alpha;-Dystroglycan, clone IIH6C4 - 32529 32529

      References

      Reference overviewSpeciesPub 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
      Reduced expression of fukutin related protein in mice results in a model for fukutin related protein associated muscular dystrophies.
      Ackroyd, M R, et al.
      Brain, 132: 439-51 (2009) 2009

      Show Abstract
      Mouse19155270
      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

      Alternative Packsize

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

      Categories

      Life Science Research > Antibodies and Assays > Primary Antibodies