merck millipore,默克密理愽
  • merck millipore,默克密理博,05-298,Anti-α-Dystroglycan Antibody, clone VIA4-1

  • merck millipore,默克密理博,05-298,Anti-α-Dystroglycan Antibody, clone VIA4-1

    产品名称：Anti-α-Dystroglycan Antibody, clone VIA4-1
    产品型号：05-298
    Detect α-Dystroglycan using this Anti-α-Dystroglycan Antibody, clone VIA4-1 validated for use in IH & WB.

    merck millipore,默克密理博,05-298,Anti-α-Dystroglycan Antibody, clone VIA4-1

  • 产品介绍
  • merck millipore,默克密理博,05-298,Anti-α-Dystroglycan Antibody, clone VIA4-1

    重要规格表

    品种反应性 主要应用 宿主 格式 抗体类型
    Rb, Ca, Gp, H, M, RWB, IHCMCulture SupernatantMonoclonal Antibody
    描述
    产品目录编号 05-298
    品牌系列 Upstate
    商名
    • Upstate
    描述 Anti-α-Dystroglycan Antibody, clone VIA4-1
    概述 Note: This product may be used for research purposes only. Diagnostic use of this product requires a license from the University of Iowa Research Foundation, 214 Technology Innovation Center, Iowa City, IA 52242.
    背景信息 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.
    产品信息
    格式 Culture Supernatant
    控制
    • Mouse, rat and human skeletal muscle tissue extracts or DU 145 (human prostate carcinoma tumor) cell lysate
    演示 Culture supernatant containing 0.05% sodium azide.
    应用
    应用 Detect α-Dystroglycan using this Anti-α-Dystroglycan Antibody, clone VIA4-1 validated for use in IH & WB.
    主要应用
    • Western Blotting
    • Immunohistochemistry
    生物信息
    免疫原品种 Rabbit skeletal muscle membrane preparation
    克隆 VIA4-1
    浓缩 Please refer to the Certificate of Analysis for the lot-specific concentration.
    宿主 Mouse
    特异性 Recognizes α-dystroglycan/LARGE-glycan.
    同种型 IgG1
    品种反应性 RabbitCanineGuinea PigHumanMouseRat
    抗体类型 Monoclonal Antibody
    Entrez基因编号
    • NM_004393
    Entrez基因汇总 Dystroglycan is a laminin binding component of the dystrophin-glycoprotein complex which provides a linkage between the subsarcolemmal cytoskeleton and the extracellular matrix. Dystroglycan 1 is a candidate gene for the site of the mutation in autosomal recessive muscular dystrophies. The dramatic reduction of dystroglycan 1 in Duchenne muscular dystrophy leads to a loss of linkage between the sarcolemma and extracellular matrix, rendering muscle fibers more susceptible to necrosis. Dystroglycan also functions as dual receptor for agrin and laminin-2 in the Schwann cell membrane. The muscle and nonmuscle isoforms of dystroglycan differ by carbohydrate moieties but not protein sequence.
    基因符号
    • 156DAG
    • DAG1
    • A3a
    • DAG
    • beta-dystroglycan
    • alpha-dystroglycan
    • AGRNR
    纯化方法 Unpurified
    UniProt编号
    • Q14118
    UniProt汇总 FUNCTION: SwissProt: Q14118 # Forms part of the dystrophin-associated protein complex (DAPC) which may link the cytoskeleton to the extracellular matrix. Alpha-dystroglycan functions as a laminin receptor. Binds to several types of arenaviruses. Is a target for the entry of Mycobacterium leprae into peripheral nerve Schwann cells.
    SIZE: 895 amino acids; 97581 Da
    SUBUNIT: Interacts with SGCD (By similarity). Interacts with AGR2 and AGR3.
    SUBCELLULAR LOCATION: Alpha-dystroglycan: Secreted, extracellular space. & Beta-dystroglycan: Cell membrane; Single- pass type I membrane protein.
    TISSUE SPECIFICITY: Expressed in a variety of fetal and adult tissues.
    产品使用声明
    质量保证 routinely evaluated on rabbit skeletal muscle in a western blot.
    使用声明
    • Unless otherwise stated in our catalog or other company documentation accompanying the product(s), our products are intended for research use only and are not to be used for any other purpose, which includes but is not limited to, unauthorized commercial uses, in vitro diagnostic uses, ex vivo or in vivo therapeutic uses or any type of consumption or application to humans or animals.
    储存和货运信息
    存储条件 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.
    包装信息
    数量 200 µL

    merck millipore,默克密理博,05-298,Anti-α-Dystroglycan Antibody, clone VIA4-1

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