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ABN2287-100UG Anti-Dystrophin

Anti-Dystrophin, Cat. No. ABN2287, is a rabbit polyclonal antibody that detects Dystrophin and is tested for use in Western Blotting, ELISA and Immunohistochemistry.

NouveauABN2287-100UG
100 μg  
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      Replacement Information
      Description
      Catalogue NumberABN2287-100UG
      DescriptionAnti-Dystrophin
      Alternate Names
      • Dystrophin
      Background InformationDystrophin (UniProt P11532) is encoded by the DMD (also known as BMD, CMD3B, DXS142, DXS164, DXS206, DXS230, DXS239, DXS268, DXS269, DXS270, DXS272, MRX85) gene (Gene ID 1756) in human. Dystrophin is a cytoplasmic protein and, a vital part of a protein complex that connects the cytoskeleton of a muscle fiber to the surrounding extracellular matrix through the cell membrane. It is expressed in muscle fibers accumulating in the costameres of myoplasm at the sarcolemma. Its expression is also reported in brain, muscle, kidney, lung and testis. Dystrophin is localized to the inner part of the muscle fiber cell membrane (sarcolemma), where it forms the dystrophin-associated glycoprotein complex (DGC) that links the extracellular matrix to the actin cytoskeleton. The N-terminus is the actin-binding domain, and the carboxy-terminal domain interacts with beta-dystroglycan as well as dystrobrevin and the syntrophin. The central rod domain that comprises the major mass of the dystrophin molecule forms a flexible, rod-shaped structure. Dystrophin plays an important role in stabilizing the muscle fiber against the mechanical forces of muscle contraction by providing a shock-absorbing connection between the cytoskeleton and the extracellular matrix. The DMD gene is the largest known gene in humans and mutations in this gene are known to cause Duchenne muscular dystrophy (DMD), a sex-linked recessive disorder that affects males aged 3 to 7 year as proximal muscle weakness with steady progression. The mutations in DMD gene disrupts the open reading frame (ORF) and prevent the full translation of dystrophin. ORF restoration by exon skipping using antisense oligonucleotides is designed to transform the DMD phenotype to that of the milder disorder, Becker muscular dystrophy (BMD), which is typically caused by in-frame dystrophin deletions that allow the production of an internally deleted, but partially functional dystrophin. Since abnormalities in the protein expression occur specifically in patients with these types of muscular dystrophy, dystrophin analysis may be used to distinguish these conditions from other neuromuscular diseases. (Ref.: Lu, QL et al. (2003) Nat Med 9(8) 1009-1014, (PMID 12847521); Lu, QL et al. (2005) PNAS 102(1) 198-203. (PMID 15608067); Wu, B. et al. (2008) PNAS 105 (39) 14814-14819 (PMID 18806224).
      References
      Product Information
      FormatPurified
      PresentationPurified rabbit polyclonal antibody in PBS with 0.1% BSA with 0.05% sodium azide.
      Quality LevelMQ200
      Applications
      ApplicationAnti-Dystrophin, Cat. No. ABN2287, is a rabbit polyclonal antibody that detects Dystrophin and is tested for use in Western Blotting, ELISA and Immunohistochemistry.
      Key Applications
      • Western Blotting
      • Immunohistochemistry
      • ELISA
      Application NotesTested Applications

      Western Blotting Analysis: A 1:1,000 dilution of this antibody detected Dystrophin protein in lysate from human skeletal muscle tissue.

      Immunohistochemistry Applications: A representative lot detected Dystrophin in Immunohistochemistry application (Lu, QL et al. (2005) PNAS 102(1) 198-203).

      ELISA Analysis: A representative lot detected Dystrophin in ELISA application (Lu, QL et al. (2003) Nat Med 9(8) 1009-1014).

      Western Blotting Analysis: A representative lot detected Dystrophin in Western Blotting application (Lu, QL et al. (2003) Nat Med 9(8) 1009-1014).

      Note: Actual optimal working dilutions must be determined by end user as specimens, and experimental conditions may vary with the end user.
      Biological Information
      ImmunogenRecombinant fragment corresponding to 231 amino acids from the C-terminal half of the human Dystrophin protein.
      EpitopeC-terminal half
      Concentration0.5 mg/mL. Please refer to guidance on suggested starting dilutions and/or titers per application and sample type.
      HostRabbit
      SpecificityThis rabbit polyclonal antibody detects Dystrophin. It targets an epitope within 231 amino acids from the C-terminal half of the protein.
      IsotypeIgG
      Species Reactivity
      • Human
      • Mouse
      Species Reactivity NoteMouse, Human.
      Antibody TypePolyclonal Antibody
      Entrez Gene Number
      Gene Symbol
      • DMD
      Purification MethodProtein A purified
      UniProt Number
      Molecular WeightTarget molecular weight ~ 475 kDa observed; 426.75 kDa calculated. Uncharacterized bands may be observed in some lysate(s)
      Physicochemical Information
      Dimensions
      Materials Information
      Toxicological Information
      Safety Information according to GHS
      Safety Information
      Product Usage Statements
      Quality AssuranceEvaluated by Western Blotting in lysate from mouse skeletal muscle tissue.

      Western Blotting Analysis: A 1:1,000 dilution of this antibody detected Dystrophin protein in lysate from mouse skeletal muscle tissue.
      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.
      Storage and Shipping Information
      Storage ConditionsRecommended storage: +2°C to +8°C.
      Packaging Information
      Material Size100 μg
      Transport Information
      Supplemental Information
      Specifications
      Global Trade Item Number
      Référence GTIN
      ABN2287-100UG 04065272074216