Dystroglycan, alpha | Developmental Studies Hybridoma Bank IIH6 C4 product information

product summary

company name :

Developmental Studies Hybridoma Bank

product type :

antibody

product name :

Dystroglycan, alpha

catalog :

IIH6 C4

clonality :

monoclonal

host :

mouse

conjugate :

nonconjugated

clone name :

IIH6 C4

reactivity :

human, mouse, rabbit

application :

western blot, immunohistochemistry, immunocytochemistry, immunoprecipitation, FACS, immunohistochemistry - frozen section

more info or order :

Developmental Studies Hybridoma Bank product webpage

citations: 46

Published Application/Species/Sample/Dilution	Reference
immunohistochemistry - frozen section; mouse; 1:500; loading ...; fig 5 western blot; mouse; loading ...; fig 10a	Blaeser A, Awano H, Lu P, Lu Q. Distinct expression of functionally glycosylated alpha-dystroglycan in muscle and non-muscle tissues of FKRP mutant mice. PLoS ONE. 2018;13:e0191016 pubmed publisher
western blot; human; loading ...; fig 1e	Raaben M, Jae L, Herbert A, Kuehne A, Stubbs S, Chou Y, et al. NRP2 and CD63 Are Host Factors for Lujo Virus Cell Entry. Cell Host Microbe. 2017;22:688-696.e5 pubmed publisher
immunocytochemistry; mouse; 1:50; loading ...; fig 7o western blot; mouse; 1:50; loading ...; fig 7a	Van Ry P, Wuebbles R, Key M, Burkin D. Galectin-1 Protein Therapy Prevents Pathology and Improves Muscle Function in the mdx Mouse Model of Duchenne Muscular Dystrophy. Mol Ther. 2015;23:1285-1297 pubmed publisher
	Cohen Dvashi H, Cohen N, Israeli H, Diskin R. Molecular Mechanism for LAMP1 Recognition by Lassa Virus. J Virol. 2015;89:7584-92 pubmed publisher
	Inamori K, Willer T, Hara Y, Venzke D, Anderson M, Clarke N, et al. Endogenous glucuronyltransferase activity of LARGE or LARGE2 required for functional modification of ?-dystroglycan in cells and tissues. J Biol Chem. 2014;289:28138-48 pubmed publisher
	Fortunato M, Ball C, Hollinger K, Patel N, Modi J, Rajasekaran V, et al. Development of rabbit monoclonal antibodies for detection of alpha-dystroglycan in normal and dystrophic tissue. PLoS ONE. 2014;9:e97567 pubmed publisher
	Vannoy C, Xu L, Keramaris E, Lu P, Xiao X, Lu Q. Adeno-associated virus-mediated overexpression of LARGE rescues ?-dystroglycan function in dystrophic mice with mutations in the fukutin-related protein. Hum Gene Ther Methods. 2014;25:187-96 pubmed publisher
	Myshrall T, Moore S, Ostendorf A, Satz J, Kowalczyk T, Nguyen H, et al. Dystroglycan on radial glia end feet is required for pial basement membrane integrity and columnar organization of the developing cerebral cortex. J Neuropathol Exp Neurol. 2012;71:1047-63 pubmed publisher
	Johnson E, Zhang L, Adams M, Phillips A, Freitas M, Froehner S, et al. Proteomic analysis reveals new cardiac-specific dystrophin-associated proteins. PLoS ONE. 2012;7:e43515 pubmed publisher
	Cabrera P, Pang M, Marshall J, Kung R, Nelson S, Stalnaker S, et al. High throughput screening for compounds that alter muscle cell glycosylation identifies new role for N-glycans in regulating sarcolemmal protein abundance and laminin binding. J Biol Chem. 2012;287:22759-70 pubmed publisher
	Zhou Y, Munoz J, Jiang D, Jarrett H. Laminin-?1 LG4-5 domain binding to dystroglycan mediates muscle cell survival, growth, and the AP-1 and NF-?B transcription factors but also has adverse effects. Am J Physiol Cell Physiol. 2012;302:C902-14 pubmed publisher
	Street R, Mucowski S, Urrabaz Garza R, O Boyle K, Snyder R, Theiler R. Dystroglycan expression in human placenta: basement membrane localization and subunit distribution change between the first and third trimester. Reprod Sci. 2012;19:282-9 pubmed publisher
	Pawlisz A, Feng Y. Three-dimensional regulation of radial glial functions by Lis1-Nde1 and dystrophin glycoprotein complexes. PLoS Biol. 2011;9:e1001172 pubmed publisher
	Townsend D, Daly M, Chamberlain J, Metzger J. Age-dependent dystrophin loss and genetic reconstitution establish a molecular link between dystrophin and heart performance during aging. Mol Ther. 2011;19:1821-5 pubmed publisher
	Hara Y, Balci Hayta B, Yoshida Moriguchi T, Kanagawa M, Beltran Valero de Bernabe D, Gundesli H, et al. A dystroglycan mutation associated with limb-girdle muscular dystrophy. N Engl J Med. 2011;364:939-46 pubmed publisher
	Satz J, Ostendorf A, Hou S, Turner A, Kusano H, Lee J, et al. Distinct functions of glial and neuronal dystroglycan in the developing and adult mouse brain. J Neurosci. 2010;30:14560-72 pubmed publisher
	Yoshida Moriguchi T, Yu L, Stalnaker S, Davis S, Kunz S, Madson M, et al. O-mannosyl phosphorylation of alpha-dystroglycan is required for laminin binding. Science. 2010;327:88-92 pubmed publisher
	Satz J, Philp A, Nguyen H, Kusano H, Lee J, Turk R, et al. Visual impairment in the absence of dystroglycan. J Neurosci. 2009;29:13136-46 pubmed publisher
	Michele D, Kabaeva Z, Davis S, Weiss R, Campbell K. Dystroglycan matrix receptor function in cardiac myocytes is important for limiting activity-induced myocardial damage. Circ Res. 2009;105:984-93 pubmed publisher
	Han R, Kanagawa M, Yoshida Moriguchi T, Rader E, Ng R, Michele D, et al. Basal lamina strengthens cell membrane integrity via the laminin G domain-binding motif of alpha-dystroglycan. Proc Natl Acad Sci U S A. 2009;106:12573-9 pubmed publisher
	de Bernabe D, Inamori K, Yoshida Moriguchi T, Weydert C, Harper H, Willer T, et al. Loss of alpha-dystroglycan laminin binding in epithelium-derived cancers is caused by silencing of LARGE. J Biol Chem. 2009;284:11279-84 pubmed publisher
	Peter A, Marshall J, Crosbie R. Sarcospan reduces dystrophic pathology: stabilization of the utrophin-glycoprotein complex. J Cell Biol. 2008;183:419-27 pubmed publisher
	Kobayashi Y, Rader E, Crawford R, Iyengar N, Thedens D, Faulkner J, et al. Sarcolemma-localized nNOS is required to maintain activity after mild exercise. Nature. 2008;456:511-5 pubmed publisher
	Satz J, Barresi R, Durbeej M, Willer T, Turner A, Moore S, et al. Brain and eye malformations resembling Walker-Warburg syndrome are recapitulated in mice by dystroglycan deletion in the epiblast. J Neurosci. 2008;28:10567-75 pubmed publisher
	Han R, Bansal D, Miyake K, Muniz V, Weiss R, McNeil P, et al. Dysferlin-mediated membrane repair protects the heart from stress-induced left ventricular injury. J Clin Invest. 2007;117:1805-13 pubmed
	Beedle A, Nienaber P, Campbell K. Fukutin-related protein associates with the sarcolemmal dystrophin-glycoprotein complex. J Biol Chem. 2007;282:16713-7 pubmed
	Barresi R, Campbell K. Dystroglycan: from biosynthesis to pathogenesis of human disease. J Cell Sci. 2006;119:199-207 pubmed
	Kanagawa M, Michele D, Satz J, Barresi R, Kusano H, Sasaki T, et al. Disruption of perlecan binding and matrix assembly by post-translational or genetic disruption of dystroglycan function. FEBS Lett. 2005;579:4792-6 pubmed
	Kanagawa M, Saito F, Kunz S, Yoshida Moriguchi T, Barresi R, Kobayashi Y, et al. Molecular recognition by LARGE is essential for expression of functional dystroglycan. Cell. 2004;117:953-64 pubmed
	Barresi R, Michele D, Kanagawa M, Harper H, Dovico S, Satz J, et al. LARGE can functionally bypass alpha-dystroglycan glycosylation defects in distinct congenital muscular dystrophies. Nat Med. 2004;10:696-703 pubmed
	Saito F, Moore S, Barresi R, Henry M, Messing A, Ross Barta S, et al. Unique role of dystroglycan in peripheral nerve myelination, nodal structure, and sodium channel stabilization. Neuron. 2003;38:747-58 pubmed
	Cohn R, Henry M, Michele D, Barresi R, Saito F, Moore S, et al. Disruption of DAG1 in differentiated skeletal muscle reveals a role for dystroglycan in muscle regeneration. Cell. 2002;110:639-48 pubmed
	Moore S, Saito F, Chen J, Michele D, Henry M, Messing A, et al. Deletion of brain dystroglycan recapitulates aspects of congenital muscular dystrophy. Nature. 2002;418:422-5 pubmed
	Michele D, Barresi R, Kanagawa M, Saito F, Cohn R, Satz J, et al. Post-translational disruption of dystroglycan-ligand interactions in congenital muscular dystrophies. Nature. 2002;418:417-22 pubmed
	Durbeej M, Cohn R, Hrstka R, Moore S, Allamand V, Davidson B, et al. Disruption of the beta-sarcoglycan gene reveals pathogenetic complexity of limb-girdle muscular dystrophy type 2E. Mol Cell. 2000;5:141-51 pubmed
	Lebakken C, Venzke D, Hrstka R, Consolino C, Faulkner J, Williamson R, et al. Sarcospan-deficient mice maintain normal muscle function. Mol Cell Biol. 2000;20:1669-77 pubmed
	Straub V, Ettinger A, Durbeej M, Venzke D, Cutshall S, Sanes J, et al. epsilon-sarcoglycan replaces alpha-sarcoglycan in smooth muscle to form a unique dystrophin-glycoprotein complex. J Biol Chem. 1999;274:27989-96 pubmed
	Durbeej M, Campbell K. Biochemical characterization of the epithelial dystroglycan complex. J Biol Chem. 1999;274:26609-16 pubmed
	Henry M, Campbell K. A role for dystroglycan in basement membrane assembly. Cell. 1998;95:859-70 pubmed
	Straub V, Duclos F, Venzke D, Lee J, Cutshall S, Leveille C, et al. Molecular pathogenesis of muscle degeneration in the delta-sarcoglycan-deficient hamster. Am J Pathol. 1998;153:1623-30 pubmed
	Duclos F, Straub V, Moore S, Venzke D, Hrstka R, Crosbie R, et al. Progressive muscular dystrophy in alpha-sarcoglycan-deficient mice. J Cell Biol. 1998;142:1461-71 pubmed
	Holt K, Lim L, Straub V, Venzke D, Duclos F, Anderson R, et al. Functional rescue of the sarcoglycan complex in the BIO 14.6 hamster using delta-sarcoglycan gene transfer. Mol Cell. 1998;1:841-8 pubmed
	Cullen M, Walsh J, Roberds S, Campbell K. Ultrastructural localization of adhalin, alpha-dystroglycan and merosin in normal and dystrophic muscle. Neuropathol Appl Neurobiol. 1996;22:30-7 pubmed
	Roberds S, Ervasti J, Anderson R, Ohlendieck K, Kahl S, Zoloto D, et al. Disruption of the dystrophin-glycoprotein complex in the cardiomyopathic hamster. J Biol Chem. 1993;268:11496-9 pubmed
	Ervasti J, Campbell K. A role for the dystrophin-glycoprotein complex as a transmembrane linker between laminin and actin. J Cell Biol. 1993;122:809-23 pubmed
	Ervasti J, Campbell K. Membrane organization of the dystrophin-glycoprotein complex. Cell. 1991;66:1121-31 pubmed

product information

Internal ID :

7770

Name :

IIH6 C4

Depositor Name :

Campbell, K.P.

Depositor Institution :

University of Iowa

Date Deposited :

9/29/11

Allow Hybridoma Distribution :

Cells Available (legacy) :

Antigen :

Dystroglycan, alpha

Antigen Species :

Rabbit

Host Species :

mouse

Isotype :

MIgM, kappa light chain

Isotype for catalog (legacy) :

IgM, kappa light chain

Positive Tested Species Reactivity :

Canine,Human,Mouse,Rabbit,Rat

Species Tested (legacy) :

rabbit, mouse, human

Initial Publication Pubmed ID :

1913804

Depositor Notes (Special Instructions) :

This antibody binds to an extracellular peripheral glycoprotein that is associated with beta-dystroglycan, a transmembrane protein.

Collections :

Human,Muscular dystrophy,Skeletal muscle

Search Keywords :

Campbell, Kevin P., Dystroglycan, alpha, Rabbit, MIgM, kappa light chain, Human/Mouse/Rabbit/Canine/Rat, DAG1, , Monoclonal, AB_2617216, IgM, Canine,Human,Mouse,Rabbit,Rat, FACS,Function Blocking,Immunofluorescence,Immunohistochemistry,Immunoprecipitation,Western Blot

Antigen Molecular Weight :

Apparent: 156 kDa

Gene :

DAG1

Uniprot ID :

Q28685

Antibody Registry ID :

AB_2617216

Synonyms (Alternate Clone Names) :

IIH6

Immunogen :

Dystrophin complex isolated from detergent-solubilized skeletal muscle membranes using wheat-germ agglutinin-Sepharose.

Immunogen Sequence :

Full length protein

Clonality :

Monoclonal

Myeloma Strain :

P3X63Ag8.653

Epitope Mapped :

Yes

Epitope Location or Sequence :

N-terminal region (lamiminin-binding glycoepitope)

Epitope Map PubMed ID :

15210115

Recommended Applications :

FACS,Function Blocking,Immunofluorescence,Immunohistochemistry,Immunoprecipitation,Western Blot

Immunoblotting (legacy) :

yes

Immunohistochemistry Pubmed IDs :

21388311 24635668 24824861 23147502 22570487

Immunofluorescence Pubmed IDs :

8505286 8866780 9744877 9811355 9865703 10488149 10669744 12230980 12140558 12797959 15184894 16098969 17452335 19633189 19846701 19797173 20980614 21388311

Western Blot Pubmed IDs :

1913804 8505286 9660967 9744877 9811355 10473626 10488149 10678176 10669744 12140559 12230980 12140558 15210115 15184894 17452335 17607357 18923033 19633189 19797173 20044576 20980614 21388311 25138275

Function Blocking Pubmed IDs :

12140558 16098969 18923033 8349731 22159078

FACS Pubmed IDs :

19244252

Pubmed IDs :

25138275

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

DSHB Growth Medium :

RPMI

References (legacy) :

Nature. 345, 315-319.; J. Biol. Chem. 268, 11496-11499.; Neuropathol. Appl. Neurobiol. 22, 30-37.; Mol. Cell. 1, 841-848.; J. Cell Biol. 142, 1461-1471.; Amer. J. Path. 153, 1623-1630.; Cell 95, 859-870.; J. Cell Biol. 145, 153-165.; J. Biol. Chem. 274, 26609-26616.; J. Biol. Chem. 274, 27989-28002.; Mol. Cell. 5, 141-151.; Mol. Cell. Biol. 20, 1669-1677.; Nature 418, 422-425.; Cell 110, 639-648.; Nature 418(6896), 417-422.; J. Biol. Chem. 278(18), 15457-15460.; Neuron 38(5), 747-758.; PNAS 100(15), 8910-8915.; Cell 117(7), 953-964.; Nature Med. 10(7), 696-703.; FEBS Lett. 579, 4792-4796.; J. Cell Sci. 119, 199-207.; J. Biol. Chem. 282(23), 16713-16717.; J. Clin. Invest. 117(7),1805-1813.; J. Neurosci. 28(42),10567-10575.; Nature 456, 511-515.; J. Biol. Chem. 284, 11279-11284.; Proc. Natl. Acad. Sci. USA. 31, 12573-12579.; J. Neurosci. 29, 13136-13146.; Cir. Res. 105, 984-993.; Science 327, 88-92.; J. Neurosci. 30, 14560-14572.; N. Eng. J. Med. 364, 939-946.

more info or order :

Developmental Studies Hybridoma Bank product webpage