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DMD monoclonal antibody, clone DYS-48   

  • カタログ番号 # : MAB1453
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  • サイズ
  • 価格
  • 在庫あ
  • 入手可能
  • カート
  • 100 ug
  • JPY ¥ 39,000
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    1. この価格は、 Japanでのみ有効です。国を選択して下さい。
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  • Specification
  • Product Description:
  • Mouse monoclonal antibody raised against full length recombinant DMD.
  • Immunogen:
  • Recombinant protein corresponding to full length human DMD.
  • Host:
  • Mouse
  • Theoretical MW (kDa):
  • 426.6
  • Reactivity:
  • Chicken, Human, Mouse, Rat
  • Form:
  • Lyophilized
  • Purification:
  • Affinity purification
  • Isotype:
  • IgG2b
  • Quality Control Testing:
  • Antibody Reactive Against Recombinant Protein.
  • Recommend Usage:
  • Western Blot (1-2 ug/mL)
    Immunohistochemistry (Formalin/PFA-fixed paraffin-embedded sections) (2-4 ug/mL)
    The optimal working dilution should be determined by the end user.
  • Storage Buffer:
  • Lyophilized from 1.2% sodium acetate (2 mg BSA, 0.01 mg sodium azide)
  • Storage Instruction:
  • Store at -20°C on dry atmosphere.
    After reconstitution with 1 mL of 1.2% sodium acetate or neutral PBS and concentration will be 100 ug/mL, store at -20°C or lower.
    Aliquot to avoid repeated freezing and thawing.
  • Note:
  • This product contains sodium azide: a POISONOUS AND HAZARDOUS SUBSTANCE which should be handled by trained staff only.
  • Publication Reference
  • Applications
  • Western Blot
  • Immunohistochemistry (Frozen sections)
  • Application Image
  • Western Blot
  • Immunohistochemistry (Frozen sections)
  • Gene Information
  • Entrez GeneID:
  • 1756
  • Gene Name:
  • DMD
  • Gene Alias:
  • BMD,CMD3B,DXS142,DXS164,DXS206,DXS230,DXS239,DXS268,DXS269,DXS270,DXS272
  • Gene Description:
  • dystrophin
  • Gene Summary:
  • The dystrophin gene is the largest gene found in nature, measuring 2.4 Mb. The gene was identified through a positional cloning approach, targeted at the isolation of the gene responsible for Duchenne (DMD) and Becker (BMD) Muscular Dystrophies. DMD is a recessive, fatal, X-linked disorder occurring at a frequency of about 1 in 3,500 new-born males. BMD is a milder allelic form. In general, DMD patients carry mutations which cause premature translation termination (nonsense or frame shift mutations), while in BMD patients dystrophin is reduced either in molecular weight (derived from in-frame deletions) or in expression level. The dystrophin gene is highly complex, containing at least eight independent, tissue-specific promoters and two polyA-addition sites. Furthermore, dystrophin RNA is differentially spliced, producing a range of different transcripts, encoding a large set of protein isoforms. Dystrophin (as encoded by the Dp427 transcripts) is a large, rod-like cytoskeletal protein which is found at the inner surface of muscle fibers. Dystrophin is part of the dystrophin-glycoprotein complex (DGC), which bridges the inner cytoskeleton (F-actin) and the extra-cellular matrix. [provided by RefSeq
  • Other Designations:
  • Duchenne muscular dystrophy protein,OTTHUMP00000023117,OTTHUMP00000023124,OTTHUMP00000023125,OTTHUMP00000023126,muscular dystrophy, Duchenne and Becker types
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