IIH6 C4

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SKU: IIH6 C4-s

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DSHB Data Sheet

Catalog Fields

Clone ID/Product Name: IIH6 C4
Available to For-Profits: Yes
Alternate Antibody Name: IIH6
Gene Symbol: DAG1
Ab Isotype: MIgM, kappa light chain
Gene Name:
Antibody Registry ID: AB_2617216 
Uniprot ID: Q28685 
RRID:  
Entrez Gene ID: 100009278 
Clonality: Monoclonal
Immunogen: Dystrophin complex isolated from detergent-solubilized skeletal muscle membranes using wheat-germ agglutinin-Sepharose.
Clone:
Immunogen Sequence: Full length protein
Myeloma Strain: P3X63Ag8.653
Epitope Mapped: Yes
Antigen Name: Dystroglycan, alpha
Epitope Location or Sequence: N-terminal region (lamiminin-binding glycoepitope)
Alternate Antigen Name:
Deposit Date: 9/29/2011
Antigen Molecular Weight: Apparent: 156 kDa
Depositor: Campbell, K.P.
Antigen Sequence:
Depositor Institution: University of Iowa
Antigen Species: Rabbit
Depositor Notes: This antibody binds to an extracellular peripheral glycoprotein that is associated with beta-dystroglycan, a transmembrane protein.
Host Species: mouse
Hybridoma Cells Available (Non-Profit): No
Confirmed Species Reactivity: Canine, Human, Mouse, Rabbit, Rat
Additional Information: IIH6 C4 recognizes a glycoconjugate on the LARGE fully glycosylated form of alpha DAG. 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
Predicted Species Reactivity:  
Human Protein Atlas:  
Additional Characterization:  
Recommended Applications: FACS, Function Blocking, Immunofluorescence, Immunohistochemistry, Immunoprecipitation, Western Blot
All cell products contain the antimicrobial ProClin. Click here for additional information.
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. For immediate use, short term storage at 4°C up to two weeks is recommended. For long term storage, divide the solution into volumes of no less than 20 ul for freezing at -20°C or -80°C. The small volume aliquot should provide sufficient reagent for short term use. Freeze-thaw cycles should be avoided. For concentrate or bioreactor products, an equal volume of glycerol, a cryoprotectant, may be added prior to freezing.
Usage Recommendations
The optimal Ig concentration for an application varies by species and antibody affinity. For each product, the antibody titer must be optimized for every application by the end user laboratory. A good starting concentration for immunohistochemistry (IHC), immunofluorescence (IF), and immunocytochemistry (ICC) when using mouse Ig is 2-5 ug/ml. For western blots, the recommended concentration range of mouse Ig 0.2-0.5 ug/ml. In general, rabbit antibodies demonstrate greater affinity and are used at a magnitude lower Ig concentration for initial testing. The recommended concentrations for rabbit Ig are 0.2-0.5 ug/ml (IF, IHC and ICC) and 20-50 ng/ml (WB).

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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