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

05-298
200 µL  
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      Overview

      Replacement Information

      Key Spec Table

      Species ReactivityKey ApplicationsHostFormatAntibody Type
      Ca, Gp, H, M, R, RbIHC, WBMCulture SupernatantMonoclonal Antibody
      Description
      Catalogue Number05-298
      Brand Family Upstate
      Trade Name
      • Upstate
      DescriptionAnti-α-Dystroglycan Antibody, clone VIA4-1
      OverviewNote: 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 InformationDystroglycan 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.
      References
      Product Information
      FormatCulture Supernatant
      Control
      • Mouse, rat and human skeletal muscle tissue extracts or DU 145 (human prostate carcinoma tumor) cell lysate
      PresentationCulture supernatant containing 0.05% sodium azide.
      Applications
      ApplicationDetect α-Dystroglycan using this Anti-α-Dystroglycan Antibody, clone VIA4-1. This antibody has been published and validated for use in IH and WB.
      Key Applications
      • Immunohistochemistry
      • Western Blotting
      Biological Information
      ImmunogenRabbit skeletal muscle membrane preparation
      CloneVIA4-1
      ConcentrationPlease refer to the Certificate of Analysis for the lot-specific concentration.
      HostMouse
      SpecificityRecognizes α-dystroglycan/LARGE-glycan.
      IsotypeIgG1
      Species Reactivity
      • Canine
      • Guinea Pig
      • Human
      • Mouse
      • Rat
      • Rabbit
      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
      Dimensions
      Materials Information
      Toxicological Information
      Safety Information according to GHS
      Safety Information
      Product Usage Statements
      Quality Assuranceroutinely 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 ConditionsMaintain 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 Size200 µL
      Transport Information
      Supplemental Information
      Specifications

      Documentation

      SDS

      Title

      Safety Data Sheet (SDS) 

      Certificates of Analysis

      TitleLot Number
      Anti-#945;-Dystroglycan, clone VIA4-1 - 2150158 2150158
      Anti-#945;-Dystroglycan, clone VIA4-1 - DAM1834934 DAM1834934
      Anti--Dystroglycan, clone VIA4-1 - 1951974 1951974
      Anti--Dystroglycan, clone VIA4-1 - 1993896 1993896
      Anti--Dystroglycan, clone VIA4-1 - 2089509 2089509
      Anti--Dystroglycan, clone VIA4-1 - DAM1408802 DAM1408802
      Anti--Dystroglycan, clone VIA4-1 - DAM1632063 DAM1632063
      Anti--Dystroglycan, clone VIA4-1 - DAM1764385 DAM1764385
      Anti--Dystroglycan, clone VIA4-1 - DAM179797926 DAM179797926
      Anti--Dystroglycan, clone VIA4-1 - JBC1863401 JBC1863401

      References

      Reference overviewApplicationSpeciesPub Med ID
      Absence of glial α-dystrobrevin causes abnormalities of the blood-brain barrier and progressive brain edema.
      Lien, CF; Mohanta, SK; Frontczak-Baniewicz, M; Swinny, JD; Zablocka, B; Górecki, DC
      The Journal of biological chemistry  287  41374-85  2012

      Show Abstract
      23043099 23043099
      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

      Show Abstract
      20660259 20660259
      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

      Show Abstract
      ImmunofluorescenceMouse18723687 18723687
      Worldwide distribution and broader clinical spectrum of muscle-eye-brain disease.
      Taniguchi, Kiyomi, et al.
      Hum. Mol. Genet., 12: 527-34 (2003)  2003

      Show Abstract
      Immunohistochemistry (Tissue)12588800 12588800
      Mechanistic distinctions between agrin and laminin-1 induced aggregation of acetylcholine receptors.
      Lee, LK; Kunkel, DD; Stollberg, J
      BMC neuroscience  3  10  2002

      Show Abstract
      12182759 12182759
      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

      Show Abstract
      Immunocytochemistry, Immunoblotting (Western)11416038 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 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

      9858474 9858474
      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

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

      Show Abstract
      1913804 1913804

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      Categories

      Life Science Research > Antibodies and Assays > Primary Antibodies