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DMD polyclonal antibody   

  • カタログ番号 # : PAB8692
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  • サイズ
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  • 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:
  • Rabbit polyclonal antibody raised against synthetic peptide of DMD.
  • Immunogen:
  • A synthetic peptide corresponding to residues surrounding amino acids 3656 of human DMD.
  • Host:
  • Rabbit
  • Theoretical MW (kDa):
  • 60, 130
  • Reactivity:
  • Human, Mouse, Rat
  • Specificity:
  • This antibody detects ~60 and 130 KDa of Dystrophin.
  • Form:
  • Liquid
  • Quality Control Testing:
  • Antibody Reactive Against Synthetic Peptide.
  • Recommend Usage:
  • Western Blot (0.5-4 ug/mL)
    The optimal working dilution should be determined by the end user.
  • Storage Buffer:
  • In PBS, pH 7.2 (30% glycerol, 0.01% thimerosal)
  • Storage Instruction:
  • Store at -20°C. For long term storage store at -80°C.
    Aliquot to avoid repeated freezing and thawing.
  • Note:
  • This product contains thimerosal: a POISONOUS AND HAZARDOUS SUBSTANCE which should be handled by trained staff only.
  • Applications
  • Western Blot
  • Western Blot
  • Western blot analysis of DMD expression in NIH/3T3 cell lysate (Lane 1) and rat kidney tissue lysate (Lane 2) .
  • Application Image
  • 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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