IIH6 C4

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$40.00
SKU: IIH6 C4-s
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DSHB Data Sheet

Catalog Fields

Antigen: Dystroglycan, alpha
Depositor: Campbell, K.P.
Antigen Species: Rabbit
Depositors Institution: University of Iowa
Isotype: MIgM, kappa light chain
Depositors Notes: This antibody binds to an extracellular peripheral glycoprotein that is associated with beta-dystroglycan, a transmembrane protein.
Host Species: mouse
Gene: DAG1
Positive Tested Species Reactivity: Canine, Human, Mouse, Rabbit, Rat
Clonality: Monoclonal
Antigen Molecular Weight: Apparent: 156 kDa
Epitope Mapped: Yes
Immunogen: Dystrophin complex isolated from detergent-solubilized skeletal muscle membranes using wheat-germ agglutinin-Sepharose.
Epitope Location or Sequence: N-terminal region (lamiminin-binding glycoepitope)
Alternate Antibody Name: IIH6
Immunogen Sequence: Full length protein
Myeloma Strain: P3X63Ag8.653
Additional Information: IH6 recognizes a glycoconjugate on the LARGE fully glycosylated form of alphaDG. It detects the laminin-binding glycoepitope. Dystroglycan is a central component of the dystrophin-glycoprotein complex that links the extracellular matrix and the cytoskeleton in the skeletal muscle. RRID:AB_2617216
Uniprot ID: Q28685 
Entrez Gene ID: 100009278 
Antibody Registry ID: AB_2617216 
Recommended Applications: FACS, Function Blocking, Immunofluorescence, Immunohistochemistry, Immunoprecipitation, Western Blot
These hybridomas were created by your colleagues. Please acknowledge the hybridoma contributor and the Developmental Studies Hybridoma Bank (DSHB) in the Materials and Methods of your publications. Please email the citation to us.
For your Materials & Methods section:
IIH6 C4 was deposited to the DSHB by Campbell, K.P. (DSHB Hybridoma Product IIH6 C4)
Storage and Handling Recommendations
Although many cell products are maintained at 4°C for years without loss of activity, shelf-life at 4°C is highly variable. To ensure retention of antibody activity, we recommend aliquotting the product into two parts: 1) a volume of antibody stored at 4°C to be used within two weeks. 2) the remaining product diluted with an equal volume of molecular grade glycerol and stored at -20°C.
Usage Recommendations
While optimal Ig concentration for an application will vary, a good starting concentration for immunohistochemistry (IHC), immunofluorescence(IF) and staining is 2-5 µg/ml. For Western blots, the concentration is decreased by one order of magnitude (that is, 0.2-0.5 µg/ml).
All cell products contain the antimicrobial ProClin. Click here for additional information.

35 References

  • Initial Publication
  • IF References
  • WB References
  • IHC References
  • FB References
  • FACS References
  • Epitope Map References
  • All References
  • Initial Publication

    Membrane organization of the dystrophin-glycoprotein complex.
    Campbell KP
    Cell 66.6 (1991 Sep 20): 1121-31.

    IF References

    Disruption of the dystrophin-glycoprotein complex in the cardiomyopathic hamster.
    Campbell KP
    The Journal of biological chemistry 268.16 (1993 Jun 5): 11496-9.

    Ultrastructural localization of adhalin, alpha-dystroglycan and merosin in normal and dystrophic muscle.
    Campbell KP
    Neuropathology and applied neurobiology 22.1 (1996 Feb): 30-7.

    Progressive muscular dystrophy in alpha-sarcoglycan-deficient mice.
    Campbell KP
    The Journal of cell biology 142.6 (1998 Sep 21): 1461-71.

    Molecular pathogenesis of muscle degeneration in the delta-sarcoglycan-deficient hamster.
    Campbell KP
    The American journal of pathology 153.5 (1998 Nov): 1623-30.

    A role for dystroglycan in basement membrane assembly.
    Campbell KP
    Cell 95.6 (1998 Dec 11): 859-70.

    epsilon-sarcoglycan replaces alpha-sarcoglycan in smooth muscle to form a unique dystrophin-glycoprotein complex.
    Campbell KP
    The Journal of biological chemistry 274.39 (1999 Sep 24): 27989-96.

    Sarcospan-deficient mice maintain normal muscle function.
    Campbell KP
    Molecular and cellular biology 20.5 (2000 Mar): 1669-77.

    Disruption of DAG1 in differentiated skeletal muscle reveals a role for dystroglycan in muscle regeneration.
    Campbell KP
    Cell 110.5 (2002 Sep 6): 639-48.

    Post-translational disruption of dystroglycan-ligand interactions in congenital muscular dystrophies.
    Campbell KP
    Nature 418.6896 (2002 Jul 25): 417-22.

    Unique role of dystroglycan in peripheral nerve myelination, nodal structure, and sodium channel stabilization.
    Campbell KP
    Neuron 38.5 (2003 Jun 5): 747-58.

    LARGE can functionally bypass alpha-dystroglycan glycosylation defects in distinct congenital muscular dystrophies.
    Campbell KP
    Nature medicine 10.7 (2004 Jul): 696-703.

    Disruption of perlecan binding and matrix assembly by post-translational or genetic disruption of dystroglycan function.
    Campbell KP
    FEBS letters 579.21 (2005 Aug 29): 4792-6.

    Fukutin-related protein associates with the sarcolemmal dystrophin-glycoprotein complex.
    Campbell KP
    The Journal of biological chemistry 282.23 (2007 Jun 8): 16713-7.

    Basal lamina strengthens cell membrane integrity via the laminin G domain-binding motif of alpha-dystroglycan.
    Campbell KP
    Proceedings of the National Academy of Sciences of the United States of America 106.31 (2009 Aug 4): 12573-9.

    Visual impairment in the absence of dystroglycan.
    Campbell KP
    The Journal of neuroscience : the official journal of the Society for Neuroscience 29.42 (2009 Oct 21): 13136-46.

    Dystroglycan matrix receptor function in cardiac myocytes is important for limiting activity-induced myocardial damage.
    Campbell KP
    Circulation research 105.10 (2009 Nov 6): 984-93.

    Distinct functions of glial and neuronal dystroglycan in the developing and adult mouse brain.
    Campbell KP
    The Journal of neuroscience : the official journal of the Society for Neuroscience 30.43 (2010 Oct 27): 14560-72.

    A dystroglycan mutation associated with limb-girdle muscular dystrophy.
    Campbell KP
    The New England journal of medicine 364.10 (2011 Mar 10): 939-46.

    WB References

    Membrane organization of the dystrophin-glycoprotein complex.
    Campbell KP
    Cell 66.6 (1991 Sep 20): 1121-31.

    Disruption of the dystrophin-glycoprotein complex in the cardiomyopathic hamster.
    Campbell KP
    The Journal of biological chemistry 268.16 (1993 Jun 5): 11496-9.

    Functional rescue of the sarcoglycan complex in the BIO 14.6 hamster using delta-sarcoglycan gene transfer.
    Campbell KP
    Molecular cell 1.6 (1998 May): 841-8.

    Progressive muscular dystrophy in alpha-sarcoglycan-deficient mice.
    Campbell KP
    The Journal of cell biology 142.6 (1998 Sep 21): 1461-71.

    Molecular pathogenesis of muscle degeneration in the delta-sarcoglycan-deficient hamster.
    Campbell KP
    The American journal of pathology 153.5 (1998 Nov): 1623-30.

    Biochemical characterization of the epithelial dystroglycan complex.
    Campbell KP
    The Journal of biological chemistry 274.37 (1999 Sep 10): 26609-16.

    epsilon-sarcoglycan replaces alpha-sarcoglycan in smooth muscle to form a unique dystrophin-glycoprotein complex.
    Campbell KP
    The Journal of biological chemistry 274.39 (1999 Sep 24): 27989-96.

    Disruption of the beta-sarcoglycan gene reveals pathogenetic complexity of limb-girdle muscular dystrophy type 2E.
    Campbell KP
    Molecular cell 5.1 (2000 Jan): 141-51.

    Sarcospan-deficient mice maintain normal muscle function.
    Campbell KP
    Molecular and cellular biology 20.5 (2000 Mar): 1669-77.

    Deletion of brain dystroglycan recapitulates aspects of congenital muscular dystrophy.
    Campbell KP
    Nature 418.6896 (2002 Jul 25): 422-5.

    Disruption of DAG1 in differentiated skeletal muscle reveals a role for dystroglycan in muscle regeneration.
    Campbell KP
    Cell 110.5 (2002 Sep 6): 639-48.

    Post-translational disruption of dystroglycan-ligand interactions in congenital muscular dystrophies.
    Campbell KP
    Nature 418.6896 (2002 Jul 25): 417-22.

    Molecular recognition by LARGE is essential for expression of functional dystroglycan.
    Campbell KP
    Cell 117.7 (2004 Jun 25): 953-64.

    LARGE can functionally bypass alpha-dystroglycan glycosylation defects in distinct congenital muscular dystrophies.
    Campbell KP
    Nature medicine 10.7 (2004 Jul): 696-703.

    Fukutin-related protein associates with the sarcolemmal dystrophin-glycoprotein complex.
    Campbell KP
    The Journal of biological chemistry 282.23 (2007 Jun 8): 16713-7.

    Dysferlin-mediated membrane repair protects the heart from stress-induced left ventricular injury.
    Campbell KP
    The Journal of clinical investigation 117.7 (2007 Jul): 1805-13.

    Brain and eye malformations resembling Walker-Warburg syndrome are recapitulated in mice by dystroglycan deletion in the epiblast.
    Campbell KP
    The Journal of neuroscience : the official journal of the Society for Neuroscience 28.42 (2008 Oct 15): 10567-75.

    Basal lamina strengthens cell membrane integrity via the laminin G domain-binding motif of alpha-dystroglycan.
    Campbell KP
    Proceedings of the National Academy of Sciences of the United States of America 106.31 (2009 Aug 4): 12573-9.

    Dystroglycan matrix receptor function in cardiac myocytes is important for limiting activity-induced myocardial damage.
    Campbell KP
    Circulation research 105.10 (2009 Nov 6): 984-93.

    O-mannosyl phosphorylation of alpha-dystroglycan is required for laminin binding.
    Campbell KP
    Science (New York, N.Y.) 327.5961 (2010 Jan 1): 88-92.

    Distinct functions of glial and neuronal dystroglycan in the developing and adult mouse brain.
    Campbell KP
    The Journal of neuroscience : the official journal of the Society for Neuroscience 30.43 (2010 Oct 27): 14560-72.

    A dystroglycan mutation associated with limb-girdle muscular dystrophy.
    Campbell KP
    The New England journal of medicine 364.10 (2011 Mar 10): 939-46.

    Endogenous glucuronyltransferase activity of LARGE or LARGE2 required for functional modification of α-dystroglycan in cells and tissues.
    Campbell KP
    The Journal of biological chemistry 289.41 (2014 Oct 10): 28138-48.

    IHC References
    FB References
    FACS References

    Loss of alpha-dystroglycan laminin binding in epithelium-derived cancers is caused by silencing of LARGE.
    Campbell KP
    The Journal of biological chemistry 284.17 (2009 Apr 24): 11279-84.

    Epitope Map References
    All References

    A dystroglycan mutation associated with limb-girdle muscular dystrophy.
    Campbell KP
    The New England journal of medicine 364.10 (2011 Mar 10): 939-46.

    Adeno-associated virus-mediated overexpression of LARGE rescues α-dystroglycan function in dystrophic mice with mutations in the fukutin-related protein.
    Lu QL
    Human gene therapy methods 25.3 (2014 Jun): 187-96.

    Development of rabbit monoclonal antibodies for detection of alpha-dystroglycan in normal and dystrophic tissue.
    Beedle AM
    PloS one 9.5 (2014): e97567.

    Dystroglycan on radial glia end feet is required for pial basement membrane integrity and columnar organization of the developing cerebral cortex.
    Hevner RF
    Journal of neuropathology and experimental neurology 71.12 (2012 Dec): 1047-63.

    High throughput screening for compounds that alter muscle cell glycosylation identifies new role for N-glycans in regulating sarcolemmal protein abundance and laminin binding.
    Baum LG
    The Journal of biological chemistry 287.27 (2012 Jun 29): 22759-70.

    Disruption of the dystrophin-glycoprotein complex in the cardiomyopathic hamster.
    Campbell KP
    The Journal of biological chemistry 268.16 (1993 Jun 5): 11496-9.

    Ultrastructural localization of adhalin, alpha-dystroglycan and merosin in normal and dystrophic muscle.
    Campbell KP
    Neuropathology and applied neurobiology 22.1 (1996 Feb): 30-7.

    Progressive muscular dystrophy in alpha-sarcoglycan-deficient mice.
    Campbell KP
    The Journal of cell biology 142.6 (1998 Sep 21): 1461-71.

    Molecular pathogenesis of muscle degeneration in the delta-sarcoglycan-deficient hamster.
    Campbell KP
    The American journal of pathology 153.5 (1998 Nov): 1623-30.

    A role for dystroglycan in basement membrane assembly.
    Campbell KP
    Cell 95.6 (1998 Dec 11): 859-70.

    epsilon-sarcoglycan replaces alpha-sarcoglycan in smooth muscle to form a unique dystrophin-glycoprotein complex.
    Campbell KP
    The Journal of biological chemistry 274.39 (1999 Sep 24): 27989-96.

    Sarcospan-deficient mice maintain normal muscle function.
    Campbell KP
    Molecular and cellular biology 20.5 (2000 Mar): 1669-77.

    Disruption of DAG1 in differentiated skeletal muscle reveals a role for dystroglycan in muscle regeneration.
    Campbell KP
    Cell 110.5 (2002 Sep 6): 639-48.

    Post-translational disruption of dystroglycan-ligand interactions in congenital muscular dystrophies.
    Campbell KP
    Nature 418.6896 (2002 Jul 25): 417-22.

    Unique role of dystroglycan in peripheral nerve myelination, nodal structure, and sodium channel stabilization.
    Campbell KP
    Neuron 38.5 (2003 Jun 5): 747-58.

    LARGE can functionally bypass alpha-dystroglycan glycosylation defects in distinct congenital muscular dystrophies.
    Campbell KP
    Nature medicine 10.7 (2004 Jul): 696-703.

    Disruption of perlecan binding and matrix assembly by post-translational or genetic disruption of dystroglycan function.
    Campbell KP
    FEBS letters 579.21 (2005 Aug 29): 4792-6.

    Fukutin-related protein associates with the sarcolemmal dystrophin-glycoprotein complex.
    Campbell KP
    The Journal of biological chemistry 282.23 (2007 Jun 8): 16713-7.

    Basal lamina strengthens cell membrane integrity via the laminin G domain-binding motif of alpha-dystroglycan.
    Campbell KP
    Proceedings of the National Academy of Sciences of the United States of America 106.31 (2009 Aug 4): 12573-9.

    Visual impairment in the absence of dystroglycan.
    Campbell KP
    The Journal of neuroscience : the official journal of the Society for Neuroscience 29.42 (2009 Oct 21): 13136-46.

    Dystroglycan matrix receptor function in cardiac myocytes is important for limiting activity-induced myocardial damage.
    Campbell KP
    Circulation research 105.10 (2009 Nov 6): 984-93.

    Distinct functions of glial and neuronal dystroglycan in the developing and adult mouse brain.
    Campbell KP
    The Journal of neuroscience : the official journal of the Society for Neuroscience 30.43 (2010 Oct 27): 14560-72.

    Membrane organization of the dystrophin-glycoprotein complex.
    Campbell KP
    Cell 66.6 (1991 Sep 20): 1121-31.

    Functional rescue of the sarcoglycan complex in the BIO 14.6 hamster using delta-sarcoglycan gene transfer.
    Campbell KP
    Molecular cell 1.6 (1998 May): 841-8.

    Biochemical characterization of the epithelial dystroglycan complex.
    Campbell KP
    The Journal of biological chemistry 274.37 (1999 Sep 10): 26609-16.

    Disruption of the beta-sarcoglycan gene reveals pathogenetic complexity of limb-girdle muscular dystrophy type 2E.
    Campbell KP
    Molecular cell 5.1 (2000 Jan): 141-51.

    Deletion of brain dystroglycan recapitulates aspects of congenital muscular dystrophy.
    Campbell KP
    Nature 418.6896 (2002 Jul 25): 422-5.

    Molecular recognition by LARGE is essential for expression of functional dystroglycan.
    Campbell KP
    Cell 117.7 (2004 Jun 25): 953-64.

    Dysferlin-mediated membrane repair protects the heart from stress-induced left ventricular injury.
    Campbell KP
    The Journal of clinical investigation 117.7 (2007 Jul): 1805-13.

    Brain and eye malformations resembling Walker-Warburg syndrome are recapitulated in mice by dystroglycan deletion in the epiblast.
    Campbell KP
    The Journal of neuroscience : the official journal of the Society for Neuroscience 28.42 (2008 Oct 15): 10567-75.

    O-mannosyl phosphorylation of alpha-dystroglycan is required for laminin binding.
    Campbell KP
    Science (New York, N.Y.) 327.5961 (2010 Jan 1): 88-92.

    Endogenous glucuronyltransferase activity of LARGE or LARGE2 required for functional modification of α-dystroglycan in cells and tissues.
    Campbell KP
    The Journal of biological chemistry 289.41 (2014 Oct 10): 28138-48.

    A role for the dystrophin-glycoprotein complex as a transmembrane linker between laminin and actin.
    Campbell KP
    The Journal of cell biology 122.4 (1993 Aug): 809-23.

    Laminin-α1 LG4-5 domain binding to dystroglycan mediates muscle cell survival, growth, and the AP-1 and NF-κB transcription factors but also has adverse effects.
    Jarrett HW
    American journal of physiology. Cell physiology 302.6 (2012 Mar 15): C902-14.

    Loss of alpha-dystroglycan laminin binding in epithelium-derived cancers is caused by silencing of LARGE.
    Campbell KP
    The Journal of biological chemistry 284.17 (2009 Apr 24): 11279-84.

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