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 VIA4-1
目录 :
05-298
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 :
VIA4-1

同一抗体克隆可从以下厂商购到:
反应物种 :
人类, 小鼠
应用 :
免疫印迹, 免疫组化, 流式细胞仪, 免疫组化-石蜡切片, 免疫组化-冰冻切片
文章摘录数: 27
出版应用/物种/样本/稀释参考文献
  • 免疫印迹; 小鼠; 1:200; 图 2e
Rubio Fernández M, Uribe M, Vicente Tejedor J, Germain F, Susín Lara C, Quereda C, et al. Impairment of photoreceptor ribbon synapses in a novel Pomt1 conditional knockout mouse model of dystroglycanopathy. Sci Rep. 2018;8:8543 pubmed 出版商
  • 免疫组化-石蜡切片; 人类; 1:50
Rickelt S, Hynes R. Antibodies and methods for immunohistochemistry of extracellular matrix proteins. Matrix Biol. 2018;71-72:10-27 pubmed 出版商
  • 免疫组化-冰冻切片; 人类; 图 7fs2i
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
  • 免疫组化; 小鼠
  • 免疫印迹; 小鼠
Domi T, Porrello E, Velardo D, Capotondo A, Biffi A, Tonlorenzi R, et al. Mesoangioblast delivery of miniagrin ameliorates murine model of merosin-deficient congenital muscular dystrophy type 1A. Skelet Muscle. 2015;5:30 pubmed 出版商
  • 流式细胞仪; 人类
  • 免疫组化; 人类
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
  • 免疫印迹; 小鼠; 图 7
Lien C, Mohanta S, Frontczak Baniewicz M, Swinny J, Zablocka B, Gorecki D. Absence of glial ?-dystrobrevin causes abnormalities of the blood-brain barrier and progressive brain edema. J Biol Chem. 2012;287:41374-85 pubmed 出版商
Balci Hayta B, Talim B, Kale G, Dincer P. LARGE expression in different types of muscular dystrophies other than dystroglycanopathy. BMC Neurol. 2018;18:207 pubmed 出版商
. 15th International Congress on Neuromuscular Diseases, July 6 - 10, 2018 Vienna, Austria. J Neuromuscul Dis. 2018;5:S1-S408 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
Osborn D, Pond H, Mazaheri N, Dejardin J, Munn C, Mushref K, et al. Mutations in INPP5K Cause a Form of Congenital Muscular Dystrophy Overlapping Marinesco-Sjögren Syndrome and Dystroglycanopathy. Am J Hum Genet. 2017;100:537-545 pubmed 出版商
Jensen L, Andersen L, Schrøder H, Frandsen U, Sjøgaard G. Neuronal nitric oxide synthase is dislocated in type I fibers of myalgic muscle but can recover with physical exercise training. Biomed Res Int. 2015;2015:265278 pubmed 出版商
Dong M, Noguchi S, Endo Y, Hayashi Y, Yoshida S, Nonaka I, et al. DAG1 mutations associated with asymptomatic hyperCKemia and hypoglycosylation of α-dystroglycan. Neurology. 2015;84:273-9 pubmed 出版商
Martínez Zárate A, Martínez Vieyra I, Alonso Rangel L, Cisneros B, Winder S, Cerecedo D. Dystroglycan depletion inhibits the functions of differentiated HL-60 cells. Biochem Biophys Res Commun. 2014;448:274-80 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
Hafner P, Bonati U, Fischmann A, Schneider J, Frank S, Morris Rosendahl D, et al. Skeletal muscle MRI of the lower limbs in congenital muscular dystrophy patients with novel POMT1 and POMT2 mutations. Neuromuscul Disord. 2014;24:321-4 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
Yu M, He Y, Wang K, Zhang P, Zhang S, Hu H. Adeno-associated viral-mediated LARGE gene therapy rescues the muscular dystrophic phenotype in mouse models of dystroglycanopathy. Hum Gene Ther. 2013;24:317-30 pubmed 出版商
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
Bangratz M, Sarrazin N, Devaux J, Zambroni D, Echaniz Laguna A, Rene F, et al. A mouse model of Schwartz-Jampel syndrome reveals myelinating Schwann cell dysfunction with persistent axonal depolarization in vitro and distal peripheral nerve hyperexcitability when perlecan is lacking. Am J Pathol. 2012;180:2040-55 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
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
Clarke N, Maugenre S, Vandebrouck A, Urtizberea J, Willer T, Peat R, et al. Congenital muscular dystrophy type 1D (MDC1D) due to a large intragenic insertion/deletion, involving intron 10 of the LARGE gene. Eur J Hum Genet. 2011;19:452-7 pubmed 出版商
Edwards M, Mammadova Bach E, Alpy F, Klein A, Hicks W, Roux M, et al. Mutations in Lama1 disrupt retinal vascular development and inner limiting membrane formation. J Biol Chem. 2010;285:7697-711 pubmed 出版商
Lathia J, Patton B, Eckley D, Magnus T, Mughal M, Sasaki T, et al. Patterns of laminins and integrins in the embryonic ventricular zone of the CNS. J Comp Neurol. 2007;505:630-43 pubmed
Yang D, Bierman J, Tarumi Y, Zhong Y, Rangwala R, Proctor T, et al. Coordinate control of axon defasciculation and myelination by laminin-2 and -8. J Cell Biol. 2005;168:655-66 pubmed
Sugita S, Saito F, Tang J, Satz J, Campbell K, Sudhof T. A stoichiometric complex of neurexins and dystroglycan in brain. J Cell Biol. 2001;154:435-45 pubmed
产品信息
目录代码 :
05-298
亚类别 :
Cell Structure
产品名称 :
Anti-α-Dystroglycan Antibody, clone VIA4-1
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 :
VIA4-1
共轭标签 :
Culture Supernatant
抗体亚型 :
IgG1
产品描述 :
Anti-α-Dystroglycan Antibody, clone VIA4-1
交叉活性 :
狗;豚鼠;人类;小鼠;大鼠;兔
背景 :
Dystroglycan is one of the dystrophin-associated glycoproteins, which is encoded by a 5.5 kb transcript in Homo sapiens by chromosome 3. There are two exons that are separated by a large intron. The spliced exons codes for a protein product is finally cleaved into two non-covalently associated subunits, α (N-terminal) and β (C-terminal). In skeletal muscle the dystroglycan complex works as a transmembrane linkage between the extracellular matrix and the cytoskeleton. α-dystroglycan is extracellular and binds to merosin (α-2 laminin) in the basement membrane, while β-dystroglycan is a transmembrane protein and binds to dystrophin, which is a large rod-like cytoskeletal protein, absent in Duchenne muscular dystrophy patients. Dystrophin binds to intracellular actin cables. In this way, the dystroglycan complex, which links the extracellular matrix to the intracellular actin cables, is thought to provide structural integrity in muscle tissues. The dystroglycan complex is also known to serve as an agrin receptor in muscle, where it may regulate agrin-induced acetylcholine receptor clustering at the neuromuscular junction. There is also evidence that suggests the function of dystroglycan as a part of the signal transduction pathway because it is shown that Grb2, a mediator of the Ras-related signal pathway, can interact with the cytoplasmic domain of dystroglycan. In general, aberrant expression of dystrophin-associated protein complex underlies the pathogenesis of Duchenne muscular dystrophy, Becker muscular dystrophy and severe childhood autosomal recessive muscular dystrophy. Interestingly, no genetic disease has been described for either α- or β-dystroglycan. Dystroglycan is widely distributed in non-muscle tissues as well as in muscle tissues. During epithelial morphogenesis of kidney, the dystroglycan complex is shown to act as a receptor for the basement membrane. Dystroglycan expression in Mus musculus brain and neural retina has also been reported. However, the physiological role of dystroglycan in non-muscle tissues has remained unclear.
免疫原 :
Rabbit skeletal muscle membrane preparation
特异性 :
Recognizes α-dystroglycan/LARGE-glycan.
包装尺寸 :
200微升
用途 :
免疫组化;免疫印迹
储存 :
Maintain for 2 years at -20°C from date of shipment. Aliquot to avoid repeated freezing and thawing. For maximum recovery of product, centrifuge the original vial after thawing and 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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