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

WB: Rabbit skeletal muscle lysate was probed with anti-α-Dystroglycan
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      Tabla espec. clave

      Reactividad según especies Aplicaciones clave Hospedador Formato Tipo de anticuerpo
      Gp,H,R,M,Rb,Ca IF,WB,Inhibition,IHC M Ascites Monoclonal Antibody
      Número de catálogo 05-593
      Marca de familia Upstate
      Sinónimos Large glycan Dystrophin-associated glycoprotein 1 dystroglycan 1 dystroglycan 1 (dystrophin-associated glycoprotein 1) dystrophin-associated glycoprotein-1 LARGE-glycan
      Nombre comercial
      • Upstate
      Descripción Anti-α-Dystroglycan Antibody, clone IIH6C4
      Visión de conjunto 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
      Información preliminar 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.
      Referencias bibliográficas
      Información del producto
      Formato Ascitos
      Especificidad This antibody recognizes α-Dystroglycan/LARGE-glycan, Mr 156 kDa.
      • Rabbit skeletal muscle lysate.
      Presentación Mouse ascites, in PBS containing 0.05% sodium azide and 30% glycerol.
      Liquid at -20ºC.
      Aplicación This Anti-α-Dystroglycan Antibody, clone IIH6C4 is validated for use in IH, FUNC, WB for the detection of α-Dystroglycan.
      Aplicaciones clave
      • Immunofluorescencia
      • Western Blotting
      • Inmunohistoquímica
      Notas de aplicación 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).
      Información biológica
      Especies inmunógenas Rabbit skeletal muscle membrane preparation. Clone IIH6C4.
      Clon IIH6C4
      Concentración Please refer to the Certificate of Analysis for the lot-specific concentration.
      Hospedador Ratón
      Especificidad This antibody recognizes α-Dystroglycan/LARGE-glycan, Mr 156 kDa.
      Isotipo IgM
      Reactividad según especies Conejillo de Indias Ser humano Rata Ratón Conejo Canina
      Species Reactivity Note Rabbit, human, mouse, rat, dog, and guinea pig.
      Tipo de anticuerpo Anticuerpo monoclonal
      Número de Entrez Gene
      Resumen de Entrez Gene 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.
      Símbolo de gen
      • 156DAG
      • DAG1
      • A3a
      • DAG
      • beta-dystroglycan
      • alpha-dystroglycan
      • AGRNR
      Método de purificación Unpurified
      Número UniProt
      Resumen UniProt 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.
      Información fisicoquímica
      Información de materiales
      Información toxicológica
      Información de seguridad según el GHS
      Información de seguridad
      Declaraciones de uso del producto
      Aseguramiento de calidad 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.
      Declaración de uso
      • 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.
      Información de almacenamiento y transporte
      Condiciones de almacenamiento Stable for 1 year at -20°C from date of receipt. For maximum recovery of product, centrifuge the vial prior to removing the cap.
      Información sobre embalaje
      Tamaño material 200 µL
      Información de transporte
      Información complementaria


      Ficha datos de seguridad (MSDS)


      Certificados de análisis

      CargoNúmero de lote
      Anti-#945;-Dystroglycan, clone IIH6C4 - 1970371 1970371
      Anti-#945;-Dystroglycan, clone IIH6C4 - DAM1754429 DAM1754429
      Anti--Dystroglycan, clone IIH6C4 - 1992447 1992447
      Anti--Dystroglycan, clone IIH6C4 - 2064510 2064510
      Anti--Dystroglycan, clone IIH6C4 - 2200926 2200926
      Anti--Dystroglycan, clone IIH6C4 - 2322511 2322511
      Anti--Dystroglycan, clone IIH6C4 - DAM1636068 DAM1636068
      Anti--Dystroglycan, clone IIH6C4 - DAM1687511 DAM1687511
      Anti--Dystroglycan, clone IIH6C4 - DAM1718044 DAM1718044
      Anti--Dystroglycan, clone IIH6C4 - DAM1792483 DAM1792483
      Anti--Dystroglycan, clone IIH6C4 - JBC1878057 JBC1878057
      Anti-a-Dystroglycan, clone IIH6C4 (mouse ascites IgM) - DAM1412899 DAM1412899
      Anti-a-Dystroglycan, clone IIH6C4 - DAM1442519 DAM1442519
      Anti-alpha-Dystroglycan, clone IIH6C4 - DAM1588207 DAM1588207
      Anti-alpha;-Dystroglycan, clone IIH6C4 - 0612047637 0612047637
      Anti-alpha;-Dystroglycan, clone IIH6C4 - 20274 20274
      Anti-alpha;-Dystroglycan, clone IIH6C4 - 22991 22991
      Anti-alpha;-Dystroglycan, clone IIH6C4 - 27702 27702
      Anti-alpha;-Dystroglycan, clone IIH6C4 - 32529 32529
      Anti-alpha;-Dystroglycan, clone IIH6C4 - JBC1362765 JBC1362765
      Anti-α-Dystroglycan, clone IIH6C4 - 2393917 2393917
      Anti-α-Dystroglycan, clone IIH6C4 - 2494018 2494018
      Anti-α-Dystroglycan, clone IIH6C4 -2524671 2524671

      Referencias bibliográficas

      Visión general referenciasPub 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

      Mostrar resumen
      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

      Mostrar resumen Artículo Texto completo
      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

      Mostrar resumen
      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

      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

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

      Mostrar resumen
      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

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

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

      Mostrar resumen

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