This webpage contains legacy information. The product is either no longer available from the supplier or has been delisted at Labome.
product summary
company name :
EMD Millipore
other brands :
Oncogene Research Products, Calbiochem, Novagen, Merck, Upstate Biotechnology, Chemicon, LINCO, Novabiochem, Guava
product type :
antibody
product name :
α-Dystroglycan Antibody, clone IIH6C4
catalog :
05-593
quantity :
200 µL
clonality :
monoclonal
host :
mouse
conjugate :
nonconjugated
clone name :
IIH6C4

The same clone is also sold as:
reactivity :
human, mouse, rat, dogs
application :
western blot, immunohistochemistry, immunocytochemistry, flow cytometry, immunohistochemistry - paraffin section, immunohistochemistry - frozen section, blocking or activating experiments, other
citations: 54
Published Application/Species/Sample/DilutionReference
  • immunohistochemistry - frozen section; mouse; 1:100; fig s3c
McKee K, Crosson S, Meinen S, Reinhard J, Ruegg M, Yurchenco P. Chimeric protein repair of laminin polymerization ameliorates muscular dystrophy phenotype. J Clin Invest. 2017;127:1075-1089 pubmed publisher
  • western blot; mouse; loading ...; fig 3
Hu H, Liu Y, Bampoe K, He Y, Yu M. Postnatal Gene Therapy Improves Spatial Learning Despite the Presence of Neuronal Ectopia in a Model of Neuronal Migration Disorder. Genes (Basel). 2016;7: pubmed
  • immunohistochemistry - paraffin section; mouse; 1:500; loading ...; fig 3 a","b
  • western blot; mouse; 1:2000; loading ...; fig s1 B",:A
Blaeser A, Awano H, Wu B, Lu Q. Progressive Dystrophic Pathology in Diaphragm and Impairment of Cardiac Function in FKRP P448L Mutant Mice. PLoS ONE. 2016;11:e0164187 pubmed publisher
  • western blot; mouse; 1:500; fig 6
Vanhoutte D, Schips T, Kwong J, Davis J, Tjondrokoesoemo A, Brody M, et al. Thrombospondin expression in myofibers stabilizes muscle membranes. elife. 2016;5: pubmed publisher
  • flow cytometry; human; 1:100; fig s4
Gerin I, Ury B, Breloy I, Bouchet Seraphin C, Bolsée J, Halbout M, et al. ISPD produces CDP-ribitol used by FKTN and FKRP to transfer ribitol phosphate onto α-dystroglycan. Nat Commun. 2016;7:11534 pubmed publisher
  • immunohistochemistry - frozen section; human; loading ...; fig 7fs2h
Praissman J, Willer T, Sheikh M, Toi A, Chitayat D, Lin Y, et al. The functional O-mannose glycan on ?-dystroglycan contains a phospho-ribitol primed for matriglycan addition. elife. 2016;5: pubmed publisher
  • blocking or activating experiments; mouse; 10 ug/ml; fig 4a
  • blocking or activating experiments; human; 10 ug/ml; loading ...; fig 4e
Anselmo A, Lauranzano E, Soldani C, Ploia C, Angioni R, D Amico G, et al. Identification of a novel agrin-dependent pathway in cell signaling and adhesion within the erythroid niche. Cell Death Differ. 2016;23:1322-30 pubmed publisher
  • immunohistochemistry; mouse; 1:50; fig 5
  • western blot; mouse; 1:500; fig 5, 6
Tjondrokoesoemo A, Schips T, Kanisicak O, Sargent M, Molkentin J. Genetic overexpression of Serpina3n attenuates muscular dystrophy in mice. Hum Mol Genet. 2016;25:1192-202 pubmed publisher
  • immunocytochemistry; rat; 1:100; fig 2
  • western blot; rat; 1:1500; fig s2
Bijata M, Wlodarczyk J, Figiel I. Dystroglycan controls dendritic morphogenesis of hippocampal neurons in vitro. Front Cell Neurosci. 2015;9:199 pubmed publisher
  • other; rat; loading ...; fig 4a
  • immunocytochemistry; rat; loading ...; fig 1e
  • western blot; rat; loading ...; fig 1j
Colombelli C, Palmisano M, Eshed Eisenbach Y, Zambroni D, Pavoni E, Ferri C, et al. Perlecan is recruited by dystroglycan to nodes of Ranvier and binds the clustering molecule gliomedin. J Cell Biol. 2015;208:313-29 pubmed publisher
  • western blot; rat
Ogasawara R, Nakazato K, Sato K, Boppart M, Fujita S. Resistance exercise increases active MMP and β1-integrin protein expression in skeletal muscle. Physiol Rep. 2014;2: pubmed publisher
  • immunohistochemistry - frozen section; mouse
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
  • immunohistochemistry - frozen section; human
von Renesse A, Petkova M, Lützkendorf S, Heinemeyer J, Gill E, Hübner C, et al. POMK mutation in a family with congenital muscular dystrophy with merosin deficiency, hypomyelination, mild hearing deficit and intellectual disability. J Med Genet. 2014;51:275-82 pubmed publisher
  • immunohistochemistry - frozen section; mouse; 1:100
  • western blot; mouse; 1:1000
Sharpe K, Premsukh M, Townsend D. Alterations of dystrophin-associated glycoproteins in the heart lacking dystrophin or dystrophin and utrophin. J Muscle Res Cell Motil. 2013;34:395-405 pubmed publisher
  • western blot; mouse; 1:5000
Hawkins B, Gu Y, Izawa Y, del Zoppo G. Disruption of dystroglycan-laminin interactions modulates water uptake by astrocytes. Brain Res. 2013;1503:89-96 pubmed publisher
  • western blot; human
Cirak S, Foley A, Herrmann R, Willer T, Yau S, Stevens E, et al. ISPD gene mutations are a common cause of congenital and limb-girdle muscular dystrophies. Brain. 2013;136:269-81 pubmed publisher
Xie Z, Xiao J, Zheng Y, Wang Z, Yuan Y. Magnetic Resonance Imaging Findings in the Muscle Tissue of Patients with Limb Girdle Muscular Dystrophy Type 2I Harboring the Founder Mutation c.545A>G in the FKRP Gene. Biomed Res Int. 2018;2018:3710814 pubmed publisher
Urbiola C, Santer F, Petersson M, van der Pluijm G, Horninger W, Erlmann P, et al. Oncolytic activity of the rhabdovirus VSV-GP against prostate cancer. Int J Cancer. 2018;: pubmed publisher
El Battrawy I, Zhao Z, Lan H, Li X, Yücel G, Lang S, et al. Ion Channel Dysfunctions in Dilated Cardiomyopathy in Limb-Girdle Muscular Dystrophy. Circ Genom Precis Med. 2018;11:e001893 pubmed publisher
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
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
Aranmolate A, Tse N, Colognato H. Myelination is delayed during postnatal brain development in the mdx mouse model of Duchenne muscular dystrophy. BMC Neurosci. 2017;18:63 pubmed publisher
Bassat E, Mutlak Y, Genzelinakh A, Shadrin I, Baruch Umansky K, Yifa O, et al. The extracellular matrix protein agrin promotes heart regeneration in mice. Nature. 2017;547:179-184 pubmed publisher
Zhang C, Hu B, Xiao L, Liu Y, Wang P. Pseudotyping lentiviral vectors with lymphocytic choriomeningitis virus glycoproteins for transduction of dendritic cells and in vivo immunization. Hum Gene Ther Methods. 2014;25:328-38 pubmed publisher
Wu B, Cloer C, Lu P, Milazi S, Shaban M, Shah S, et al. Exon skipping restores dystrophin expression, but fails to prevent disease progression in later stage dystrophic dko mice. Gene Ther. 2014;21:785-93 pubmed publisher
Raphael A, Couthouis J, Sakamuri S, Siskind C, Vogel H, Day J, et al. Congenital muscular dystrophy and generalized epilepsy caused by GMPPB mutations. Brain Res. 2014;1575:66-71 pubmed publisher
Johnson E, Li B, Yoon J, Flanigan K, Martin P, ERVASTI J, et al. Identification of new dystroglycan complexes in skeletal muscle. PLoS ONE. 2013;8:e73224 pubmed publisher
Stevens E, Torelli S, Feng L, Phadke R, Walter M, Schneiderat P, et al. Flow cytometry for the analysis of ?-dystroglycan glycosylation in fibroblasts from patients with dystroglycanopathies. PLoS ONE. 2013;8:e68958 pubmed publisher
Walko G, Wögenstein K, Winter L, Fischer I, Feltri M, Wiche G. Stabilization of the dystroglycan complex in Cajal bands of myelinating Schwann cells through plectin-mediated anchorage to vimentin filaments. Glia. 2013;61:1274-87 pubmed publisher
Riisager M, Duno M, Hansen F, Krag T, Vissing C, Vissing J. A new mutation of the fukutin gene causing late-onset limb girdle muscular dystrophy. Neuromuscul Disord. 2013;23:562-7 pubmed publisher
Blaeser A, Keramaris E, Chan Y, Sparks S, Cowley D, Xiao X, et al. Mouse models of fukutin-related protein mutations show a wide range of disease phenotypes. Hum Genet. 2013;132:923-34 pubmed publisher
Stevens E, Carss K, Cirak S, Foley A, Torelli S, Willer T, et al. Mutations in B3GALNT2 cause congenital muscular dystrophy and hypoglycosylation of ?-dystroglycan. Am J Hum Genet. 2013;92:354-65 pubmed publisher
Schreckenbach T, Henn W, Kress W, Roos A, Maschke M, Feiden W, et al. Novel FHL1 mutation in a family with reducing body myopathy. Muscle Nerve. 2013;47:127-34 pubmed publisher
Yoon J, Johnson E, Xu R, Martin L, Martin P, Montanaro F. Comparative proteomic profiling of dystroglycan-associated proteins in wild type, mdx, and Galgt2 transgenic mouse skeletal muscle. J Proteome Res. 2012;11:4413-24 pubmed publisher
Mazzon C, Anselmo A, Soldani C, Cibella J, Ploia C, Moalli F, et al. Agrin is required for survival and function of monocytic cells. Blood. 2012;119:5502-11 pubmed publisher
Raducu M, Baets J, Fano O, Van Coster R, Cruces J. Promoter alteration causes transcriptional repression of the POMGNT1 gene in limb-girdle muscular dystrophy type 2O. Eur J Hum Genet. 2012;20:945-52 pubmed publisher
Tachikawa M, Kanagawa M, Yu C, Kobayashi K, Toda T. Mislocalization of fukutin protein by disease-causing missense mutations can be rescued with treatments directed at folding amelioration. J Biol Chem. 2012;287:8398-406 pubmed publisher
Shimojima M, Stroher U, Ebihara H, Feldmann H, Kawaoka Y. Identification of cell surface molecules involved in dystroglycan-independent Lassa virus cell entry. J Virol. 2012;86:2067-78 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
Eyermann C, Czaplinski K, Colognato H. Dystroglycan promotes filopodial formation and process branching in differentiating oligodendroglia. J Neurochem. 2012;120:928-47 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
Alvarado C, Maruyama S, Cheng J, Ida Yonemochi H, Kobayashi T, Yamazaki M, et al. Nuclear translocation of ?-catenin synchronized with loss of E-cadherin in oral epithelial dysplasia with a characteristic two-phase appearance. Histopathology. 2011;59:283-91 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
Zhang Z, Zhang P, Hu H. LARGE expression augments the glycosylation of glycoproteins in addition to ?-dystroglycan conferring laminin binding. PLoS ONE. 2011;6:e19080 pubmed publisher
Hu H, Li J, Gagen C, Gray N, Zhang Z, Qi Y, et al. Conditional knockout of protein O-mannosyltransferase 2 reveals tissue-specific roles of O-mannosyl glycosylation in brain development. J Comp Neurol. 2011;519:1320-37 pubmed publisher
Brockington M, Torelli S, Sharp P, Liu K, Cirak S, Brown S, et al. Transgenic overexpression of LARGE induces α-dystroglycan hyperglycosylation in skeletal and cardiac muscle. PLoS ONE. 2010;5:e14434 pubmed publisher
Hu H, Li J, Zhang Z, Yu M. Pikachurin interaction with dystroglycan is diminished by defective O-mannosyl glycosylation in congenital muscular dystrophy models and rescued by LARGE overexpression. Neurosci Lett. 2011;489:10-5 pubmed publisher
Cannon J, Sew T, Montero L, Burton E, Greenamyre J. Pseudotype-dependent lentiviral transduction of astrocytes or neurons in the rat substantia nigra. Exp Neurol. 2011;228:41-52 pubmed publisher
Chandrasekharan K, Yoon J, Xu Y, DeVries S, Camboni M, Janssen P, et al. A human-specific deletion in mouse Cmah increases disease severity in the mdx model of Duchenne muscular dystrophy. Sci Transl Med. 2010;2:42ra54 pubmed publisher
Song Y, Aglipay J, Bernstein J, Goswami S, Stanley P. The bisecting GlcNAc on N-glycans inhibits growth factor signaling and retards mammary tumor progression. Cancer Res. 2010;70:3361-71 pubmed publisher
Santhanakrishnan M, Ray K, Oppenheimer K, Bonney E. Dynamic regulation of alpha-dystroglycan in mouse placenta. Placenta. 2008;29:932-6 pubmed publisher
Akhavan A, Crivelli S, Singh M, Lingappa V, Muschler J. SEA domain proteolysis determines the functional composition of dystroglycan. FASEB J. 2008;22:612-21 pubmed
Sparks S, Rakocevic G, Joe G, Manoli I, Shrader J, Harris Love M, et al. Intravenous immune globulin in hereditary inclusion body myopathy: a pilot study. BMC Neurol. 2007;7:3 pubmed
Chiyonobu T, Sasaki J, Nagai Y, Takeda S, Funakoshi H, Nakamura T, et al. Effects of fukutin deficiency in the developing mouse brain. Neuromuscul Disord. 2005;15:416-26 pubmed
product information
Catalog Number :
05-593
Subcategory :
Metabolism
Product Name :
Anti-α-Dystroglycan Antibody, clone IIH6C4
Product Type :
Antibodies
Clonality :
Monoclonal Antibody
Gene ID :
Q14118
Host Name :
Mouse
Antigen :
α-Dystroglycan
Clone :
IIH6C4
Conjugate :
Ascites
Isotype :
IgM
Product Description :
Anti-α-Dystroglycan Antibody, clone IIH6C4
Cross Reactivity :
Human;Rat;Mouse;Rabbit;Canine;Guinea Pig
Background :
Dystroglycans are essential elements of the neuromuscular junction (NMJ). The gene for dystroglycan is expressed as a precursor protein that is post translationally cleaved into a 156 kDa extracellular peripheral membrane protein called alpha dystroglycan and a 43 kDa transmembrane protein, beta dystroglycan. The latter protein contains a PPxY motif that promotes binding to WW domain containing proteins, such as utrophin and dystrophin. Phosphorylation at tyrosine 892 within the PPxY motif may regulate c Src interactions with beta dystroglycan, as well as inhibit interactions with WW domain proteins. In skeletal muscle, beta dystroglycan is normally localized to the plasma membrane, however phosphorylation of Tyr892 leads to localization of beta dystroglycan to endosomal compartments along with c Src. Thus, phosphorylation at Tyr892 may have important roles in altering the localization of beta dystroglycan during NMJ formation.
ALT Names :
Dystrophin-associated glycoprotein 1;dystroglycan 1;dystroglycan 1 (dystrophin-associated glycoprotein 1);dystrophin-associated glycoprotein-1;LARGE-glycan;Large glycan
Immunogen :
Rabbit skeletal muscle membrane preparation. Clone IIH6C4.
Specificity :
This antibody recognizes α-Dystroglycan/LARGE-glycan, Mr 156 kDa.
Package Size :
200 µL
Uses :
Western Blotting;Inhibition;Immunohistochemistry;Immunofluorescence
Storage :
Stable for 1 year at -20°C from date of receipt. For maximum recovery of product, centrifuge the vial prior to removing the cap.
company information
EMD Millipore
290 Concord Road
Billerica, Massachusetts 01821
bioscienceshelp@emdchemical.com
https://www.emdmillipore.com
888-854-3417
headquarters: United States
EMD Millipore is the Life Science division of Merck KGaA of Darmstadt, Germany

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