This webpage contains legacy information. The product is either no longer available from the supplier or has been delisted at Labome.
产品简要
公司名称 :
默克密理博中国
其他品牌 :
Oncogene Research Products, Calbiochem, Novagen, Merck, Upstate Biotechnology, Chemicon, LINCO, Novabiochem, Guava
产品类型 :
抗体
产品名称 :
α-Dystroglycan Antibody, clone IIH6C4
目录 :
05-593
规格 :
200微升
克隆性 :
单克隆
宿主 :
小鼠
共轭标签 :
未共轭
克隆名称 :
IIH6C4

同一抗体克隆可从以下厂商购到:
反应物种 :
人类, 小鼠, 大鼠,
应用 :
免疫印迹, 免疫组化, 免疫细胞化学, 流式细胞仪, 免疫组化-石蜡切片, 免疫组化-冰冻切片, 抑制或激活实验, 其他
文章摘录数: 54
出版应用/物种/样本/稀释参考文献
  • 免疫组化-冰冻切片; 小鼠; 1:100; 图 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 出版商
  • 免疫印迹; 小鼠; 图 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
  • 免疫组化-石蜡切片; 小鼠; 1:500; 图 3 a","b
  • 免疫印迹; 小鼠; 1:2000; 图 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 出版商
  • 免疫印迹; 小鼠; 1:500; 图 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 出版商
  • 流式细胞仪; 人类; 1:100; 图 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 出版商
  • 免疫组化-冰冻切片; 人类; 图 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 出版商
  • 抑制或激活实验; 人类; 10 ug/ml; 图 4e
  • 抑制或激活实验; 小鼠; 10 ug/ml; 图 4a
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 出版商
  • 免疫组化; 小鼠; 1:50; 图 5
  • 免疫印迹; 小鼠; 1:500; 图 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 出版商
  • 免疫细胞化学; 大鼠; 1:100; 图 2
  • 免疫印迹; 大鼠; 1:1500; 图 s2
Bijata M, Wlodarczyk J, Figiel I. Dystroglycan controls dendritic morphogenesis of hippocampal neurons in vitro. Front Cell Neurosci. 2015;9:199 pubmed 出版商
  • 其他; 大鼠; 图 4a
  • 免疫细胞化学; 大鼠; 图 1e
  • 免疫印迹; 大鼠; 图 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 出版商
  • 免疫印迹; 大鼠
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 出版商
  • 免疫组化-冰冻切片; 小鼠
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 出版商
  • 免疫组化-冰冻切片; 人类
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 出版商
  • 免疫组化-冰冻切片; 小鼠; 1:100
  • 免疫印迹; 小鼠; 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 出版商
  • 免疫印迹; 小鼠; 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 出版商
  • 免疫印迹; 人类
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 出版商
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 出版商
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 出版商
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 出版商
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 出版商
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 出版商
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 出版商
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 出版商
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 出版商
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 出版商
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 出版商
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 出版商
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 出版商
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 出版商
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 出版商
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 出版商
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 出版商
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 出版商
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 出版商
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 出版商
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 出版商
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 出版商
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 出版商
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 出版商
Eyermann C, Czaplinski K, Colognato H. Dystroglycan promotes filopodial formation and process branching in differentiating oligodendroglia. J Neurochem. 2012;120:928-47 pubmed 出版商
Pawlisz A, Feng Y. Three-dimensional regulation of radial glial functions by Lis1-Nde1 and dystrophin glycoprotein complexes. PLoS Biol. 2011;9:e1001172 pubmed 出版商
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 出版商
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 出版商
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 出版商
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 出版商
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 出版商
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 出版商
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 出版商
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 出版商
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 出版商
Santhanakrishnan M, Ray K, Oppenheimer K, Bonney E. Dynamic regulation of alpha-dystroglycan in mouse placenta. Placenta. 2008;29:932-6 pubmed 出版商
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
产品信息
目录代码 :
05-593
亚类别 :
代谢
产品名称 :
Anti-α-Dystroglycan Antibody, clone IIH6C4
产品类型 :
抗体
克隆性 :
单克隆抗体
基因编号 :
Q14118
宿主名称 :
小鼠
抗原 :
α-Dystroglycan
克隆 :
IIH6C4
共轭标签 :
腹水
抗体亚型 :
IgM
产品描述 :
Anti-α-Dystroglycan Antibody, clone IIH6C4
交叉活性 :
人类;大鼠;小鼠;兔;狗;豚鼠
背景 :
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.
别名 :
Dystrophin-associated glycoprotein 1;dystroglycan 1;dystroglycan 1 (dystrophin-associated glycoprotein 1);dystrophin-associated glycoprotein-1;LARGE-glycan;Large glycan
免疫原 :
Rabbit skeletal muscle membrane preparation. Clone IIH6C4.
特异性 :
This antibody recognizes α-Dystroglycan/LARGE-glycan, Mr 156 kDa.
包装尺寸 :
200微升
用途 :
免疫印迹;抑制作用;免疫组化;免疫荧光
储存 :
Stable for 1 year at -20°C from date of receipt. For maximum recovery of product, centrifuge the vial prior to removing the cap.
公司信息
默克密理博中国
上海市浦东张江高科技园区晨晖路88号2号楼2楼
asiatechserv@millipore.com
http://www.millipore.com/china/wwcn/cnhome
400-889-1988
公司总部: 美国
EMD Millipore is the Life Science division of Merck KGaA of Darmstadt, Germany

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View all the new antibodies at www.emdmillipore.com.