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

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      Key Specifications Table

      Species ReactivityKey ApplicationsHostFormatAntibody Type
      H, R, M, Rb, Ca, Gp WB, Inhibition, IHC, IF M Ascites Monoclonal Antibody
      Catalogue Number05-593
      Brand Family Upstate
      Trade Name
      • Upstate
      DescriptionAnti-α-Dystroglycan Antibody, clone IIH6C4
      OverviewThis 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
      • dystroglycan 1
      • dystroglycan 1 (dystrophin-associated glycoprotein 1)
      • dystrophin-associated glycoprotein-1
      • LARGE-glycan
      • Large glycan
      Background InformationDystroglycans 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.
      Product Information
      • Rabbit skeletal muscle lysate.
      PresentationMouse ascites, in PBS containing 0.05% sodium azide and 30% glycerol.
      Liquid at -20ºC.
      Quality LevelMQ100
      ApplicationThis Anti-α-Dystroglycan Antibody, clone IIH6C4 is validated for use in IH, FUNC, WB for the detection of α-Dystroglycan.
      Key Applications
      • Western Blotting
      • Inhibition
      • Immunohistochemistry
      • Immunofluorescence
      Application NotesInhibition 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
      ImmunogenRabbit skeletal muscle membrane preparation. Clone IIH6C4.
      ConcentrationPlease refer to the Certificate of Analysis for the lot-specific concentration.
      SpecificityThis antibody recognizes α-Dystroglycan/LARGE-glycan, Mr 156 kDa.
      Species Reactivity
      • Human
      • Rat
      • Mouse
      • Rabbit
      • Canine
      • Guinea Pig
      Antibody TypeMonoclonal Antibody
      Entrez Gene Number
      Entrez Gene SummaryDystroglycan 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
      • A3a
      • DAG
      • beta-dystroglycan
      • alpha-dystroglycan
      • AGRNR
      • 156DAG
      Purification MethodUnpurified
      UniProt Number
      UniProt SummaryFUNCTION: 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.
      Molecular Weight156 kDa
      Physicochemical Information
      Materials Information
      Toxicological Information
      Safety Information according to GHS
      Safety Information
      Product Usage Statements
      Quality AssuranceRoutinely 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 ConditionsStable 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 Size200 µL
      Transport Information
      Supplemental Information


      Anti-α-Dystroglycan Antibody, clone IIH6C4 SDS


      Safety Data Sheet (SDS) 

      Anti-α-Dystroglycan Antibody, clone IIH6C4 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


      Reference overviewApplicationSpeciesPub Med ID
      Dystroglycan controls dendritic morphogenesis of hippocampal neurons in vitro.
      Bijata, M; Wlodarczyk, J; Figiel, I
      Frontiers in cellular neuroscience  9  199  2015

      Show Abstract
      26074769 26074769
      Gephyrin clusters are absent from small diameter primary afferent terminals despite the presence of GABA(A) receptors.
      Lorenzo, LE; Godin, AG; Wang, F; St-Louis, M; Carbonetto, S; Wiseman, PW; Ribeiro-da-Silva, A; De Koninck, Y
      The Journal of neuroscience : the official journal of the Society for Neuroscience  34  8300-17  2014

      Show Abstract
      24920633 24920633
      Resistance exercise increases active MMP and β1-integrin protein expression in skeletal muscle.
      Ogasawara, R; Nakazato, K; Sato, K; Boppart, MD; Fujita, S
      Physiological reports  2  2014

      Show Abstract
      25413329 25413329
      ISPD gene mutations are a common cause of congenital and limb-girdle muscular dystrophies.
      Cirak, S; Foley, AR; Herrmann, R; Willer, T; Yau, S; Stevens, E; Torelli, S; Brodd, L; Kamynina, A; Vondracek, P; Roper, H; Longman, C; Korinthenberg, R; Marrosu, G; Nürnberg, P; , ; Michele, DE; Plagnol, V; Hurles, M; Moore, SA; Sewry, CA; Campbell, KP; Voit, T; Muntoni, F
      Brain : a journal of neurology  136  269-81  2013

      Show Abstract
      ImmunohistochemistryHuman23288328 23288328
      Pseudotype-dependent lentiviral transduction of astrocytes or neurons in the rat substantia nigra.
      Cannon, JR; Sew, T; Montero, L; Burton, EA; Greenamyre, JT
      Experimental neurology  228  41-52  2011

      Show Abstract
      21056560 21056560
      Renal collecting system growth and function depend upon embryonic γ1 laminin expression.
      Yang, DH; McKee, KK; Chen, ZL; Mernaugh, G; Strickland, S; Zent, R; Yurchenco, PD
      Development (Cambridge, England)  138  4535-44  2011

      Show Abstract
      21903675 21903675
      Conditional knockout of protein O-mannosyltransferase 2 reveals tissue-specific roles of O-mannosyl glycosylation in brain development.
      Hu, H; Li, J; Gagen, CS; Gray, NW; Zhang, Z; Qi, Y; Zhang, P
      The Journal of comparative neurology  519  1320-37  2011

      Show Abstract
      Mouse21452199 21452199
      Transgenic overexpression of LARGE induces α-dystroglycan hyperglycosylation in skeletal and cardiac muscle.
      Brockington, M; Torelli, S; Sharp, PS; Liu, K; Cirak, S; Brown, SC; Wells, DJ; Muntoni, F
      PloS one  5  e14434  2010

      Show Abstract
      Immunofluorescence21203384 21203384
      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 20019182
      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 19131360

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      Life Science Research > Antibodies and Assays > Primary Antibodies