货号：05-298

品牌：Merck Millipore

规格：200UL

目录价：￥6281.00

市场价格：￥5338.85

会员价格：￥5024.80

金山科研平台，产品价格货期咨询微信：jinshanbio Description: Anti-α-Dystroglycan Antibody, clone VIA4-1 | 05-298 View All» Promotional Text: Special Shipping Offer on Antibodies100% Performance Guaranteed View All» Trade Name: Upstate (Millipore) View All» Product Overview: 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. View All» Specificity: Recognizes dystroglycan. View All» Molecular Weight: 156 kDa View All» Immunogen: Rabbit skeletal muscle membrane preparation View All» Clone: VIA4-1 View All» Isotype: IgG1 View All» Background Information: 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. View All» Species Reactivity:

• Canine

• Guinea Pig

• Human

• Mouse

• Rat

• Rabbit
  View All» Control: Mouse, rat and human skeletal muscle tissue extracts or DU 145 (human prostate carcinoma tumor) cell lysate View All» Quality Assurance: routinely evaluated on rabbit skeletal muscle in a western blot. View All» Purification Method: Unpurified View All» Presentation: Culture supernatant containing 0.05% sodium azide. View All» Storage Conditions: 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. View All» UniProt Number: Q14118 View All» Entrez Gene Number: NM_004393 View All» Gene Symbol:
  • DAG1

  • A3a

  • DAG

  • beta-dystroglycan

  • alpha-dystroglycan

  • AGRNR

  • 156DAG
    View All» Usage Statement: 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. View All» Key Applications:
    • Immunohistochemistry

    • Western Blotting
      View All» Entrez Gene Summary: 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. View All» UniProt Summary: 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. View All» Brand Family: Upstate View All» Product Name: Anti-α-Dystroglycan Antibody, clone VIA4-1 | 05-298 View All» Concentration: Variable View All» Antibody Type: Monoclonal Antibody View All» Qty/Pk: 200 μL View All» Format: Culture Supernatant View All» Host: Mouse View All»
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