05-298 | Anti-α-Dystroglycan Antibody, clone VIA4-1

WB: Rabbit skeletal muscle lysate was probed with anti-α Dystroglycan (1:2000).
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      Tableau de caractéristiques principal

      Species Reactivity Key Applications Host Format Antibody Type
      Ca, Rb, R, M, H, Gp IHC, WB M Culture Supernatant Monoclonal Antibody
      Catalogue Number 05-298
      Brand Family Upstate
      Trade Name
      • Upstate
      Description Anti-α-Dystroglycan Antibody, clone VIA4-1
      Overview Note: 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 Dystroglycan is one of the dystrophin-associated glycoproteins, which is encoded by a 5.5 kb transcript in Homo sapiens by chromosome 3. There are two exons that are separated by a large intron. The spliced exons codes for a protein product is finally cleaved into two non-covalently associated subunits, α (N-terminal) and β (C-terminal). In skeletal muscle the dystroglycan complex works as a transmembrane linkage between the extracellular matrix and the cytoskeleton. α-dystroglycan is extracellular and binds to merosin (α-2 laminin) in the basement membrane, while β-dystroglycan is a transmembrane protein and binds to dystrophin, which is a large rod-like cytoskeletal protein, absent in Duchenne muscular dystrophy patients. Dystrophin binds to intracellular actin cables. In this way, the dystroglycan complex, which links the extracellular matrix to the intracellular actin cables, is thought to provide structural integrity in muscle tissues. The dystroglycan complex is also known to serve as an agrin receptor in muscle, where it may regulate agrin-induced acetylcholine receptor clustering at the neuromuscular junction. There is also evidence that suggests the function of dystroglycan as a part of the signal transduction pathway because it is shown that Grb2, a mediator of the Ras-related signal pathway, can interact with the cytoplasmic domain of dystroglycan. In general, aberrant expression of dystrophin-associated protein complex underlies the pathogenesis of Duchenne muscular dystrophy, Becker muscular dystrophy and severe childhood autosomal recessive muscular dystrophy. Interestingly, no genetic disease has been described for either α- or β-dystroglycan.
      Dystroglycan is widely distributed in non-muscle tissues as well as in muscle tissues. During epithelial morphogenesis of kidney, the dystroglycan complex is shown to act as a receptor for the basement membrane. Dystroglycan expression in Mus musculus brain and neural retina has also been reported. However, the physiological role of dystroglycan in non-muscle tissues has remained unclear.
      Product Information
      Format Culture Supernatant
      • Mouse, rat and human skeletal muscle tissue extracts or DU 145 (human prostate carcinoma tumor) cell lysate
      Presentation Culture supernatant containing 0.05% sodium azide.
      Application Detect α-Dystroglycan using this Anti-α-Dystroglycan Antibody, clone VIA4-1 validated for use in IH & WB.
      Key Applications
      • Immunohistochemistry
      • Western Blotting
      Biological Information
      Immunogen Rabbit skeletal muscle membrane preparation
      Clone VIA4-1
      Concentration Please refer to the Certificate of Analysis for the lot-specific concentration.
      Host Mouse
      Specificity Recognizes α-dystroglycan/LARGE-glycan.
      Isotype IgG1
      Species Reactivity
      • Canine
      • Rabbit
      • Rat
      • Mouse
      • Human
      • 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
      Materials Information
      Toxicological Information
      Safety Information according to GHS
      Safety Information
      Product Usage Statements
      Quality Assurance routinely evaluated on rabbit skeletal muscle in a western blot.
      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 Maintain for 2 years at -20°C from date of shipment. Aliquot to avoid repeated freezing and thawing. For maximum recovery of product, centrifuge the original vial after thawing and prior to removing the cap.
      Packaging Information
      Material Size 200 µL
      Transport Information
      Supplemental Information




      Certificats d'analyse

      TitreNuméro de lot
      Anti-#945;-Dystroglycan, clone VIA4-1 - 21501582150158
      Anti-#945;-Dystroglycan, clone VIA4-1 - DAM1834934DAM1834934
      Anti--Dystroglycan, clone VIA4-1 - 19519741951974
      Anti--Dystroglycan, clone VIA4-1 - 19938961993896
      Anti--Dystroglycan, clone VIA4-1 - 20895092089509
      Anti--Dystroglycan, clone VIA4-1 - DAM1408802DAM1408802
      Anti--Dystroglycan, clone VIA4-1 - DAM1632063DAM1632063
      Anti--Dystroglycan, clone VIA4-1 - DAM1764385DAM1764385
      Anti--Dystroglycan, clone VIA4-1 - DAM179797926DAM179797926
      Anti--Dystroglycan, clone VIA4-1 - JBC1863401JBC1863401
      Anti--Dystroglycan, clone VIA4-1 - JBC1925515JBC1925515
      Anti-a-Dystroglycan, clone VIA4-1 - DAM1467543DAM1467543
      Anti-alpha-Dystroglycan, clone VIA4-1 - DAM1543693DAM1543693
      Anti-alpha;-Dystroglycan, clone VIA4-1 - 1557115571
      Anti-alpha;-Dystroglycan, clone VIA4-1 - 1675316753
      Anti-alpha;-Dystroglycan, clone VIA4-1 - 1842818428
      Anti-alpha;-Dystroglycan, clone VIA4-1 - 2082420824
      Anti-alpha;-Dystroglycan, clone VIA4-1 - 2270522705
      Anti-alpha;-Dystroglycan, clone VIA4-1 - 2414724147
      Anti-alpha;-Dystroglycan, clone VIA4-1 - 2513325133
      Anti-alpha;-Dystroglycan, clone VIA4-1 - 2949029490
      Anti-alpha;-Dystroglycan, clone VIA4-1 - 3000230002
      Anti-alpha;-Dystroglycan, clone VIA4-1 - 3171831718
      Anti-alpha;-Dystroglycan, clone VIA4-1 - 3253332533
      Anti-alpha;-Dystroglycan, clone VIA4-1 - 3268532685
      Anti-α-Dystroglycan/LARGE-glycan, clone VIA4-12470899
      Anti-α-Dystroglycan/LARGE-glycan, clone VIA4-1 - 23847242384724
      Anti-α-Dystroglycan/LARGE-glycan, clone VIA4-1 -25006242500624
      Anti-α-Dystroglycan/LARGE-glycan, clone VIA4-1 -25522752552275
      Anti-α-Dystroglycan/LARGE-glycan, clone VIA4-1 -26150242615024

      Références bibliographiques

      Aperçu de la référence bibliographiqueApplicationEspèceNº PubMed
      Quiescence and activation of stem and precursor cell populations in the subependymal zone of the mammalian brain are associated with distinct cellular and extracellular matrix signals.
      Kazanis, Ilias, et al.
      J. Neurosci., 30: 9771-81 (2010) 2010

      Afficher le résumé
      Synapse formation and clustering of neuroligin-2 in the absence of GABAA receptors.
      Patrizi, Annarita, et al.
      Proc. Natl. Acad. Sci. U.S.A., 105: 13151-6 (2008) 2008

      Afficher le résumé
      Worldwide distribution and broader clinical spectrum of muscle-eye-brain disease.
      Taniguchi, Kiyomi, et al.
      Hum. Mol. Genet., 12: 527-34 (2003) 2003

      Afficher le résumé
      Immunohistochemistry (Tissue)12588800
      Dystroglycan is present in rat thyroid and rat thyroid cells and responds to thyrotropin.
      Collins, B J, et al.
      Endocrinology, 142: 3152-62 (2001) 2001

      Afficher le résumé
      Immunocytochemistry, Immunoblotting (Western)11416038
      A stoichiometric complex of neurexins and dystroglycan in brain
      Sugita, S., et al
      J Cell Biol, 154:435-45 (2001) 2001

      Immunocytochemistry, Immunoblotting (Western)11470830
      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

      MEK kinase 1, a substrate for DEVD-directed caspases, is involved in genotoxin-induced apoptosis.
      Widmann, C, et al.
      Mol. Cell. Biol., 18: 2416-29 (1998) 1998

      Afficher le résumé
      Immunoblotting (Western)9528810
      Membrane organization of the dystrophin-glycoprotein complex.
      Ervasti, J M and Campbell, K P
      Cell, 66: 1121-31 (1991) 1991

      Afficher le résumé
      Dystrophin constitutes 5% of membrane cytoskeleton in skeletal muscle.
      Ohlendieck, K and Campbell, K P
      FEBS Lett., 283: 230-4 (1991) 1991

      Afficher le résumé
      Deficiency of a glycoprotein component of the dystrophin complex in dystrophic muscle.
      Ervasti, J M, et al.
      Nature, 345: 315-9 (1990) 1990

      Afficher le résumé