Dystrophin Antibody

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Formalin fixed paraffin embedded human skeletal muscle stained with Dystrophin antibody

Product Details

Summary
Product Discontinued
View other related Dystrophin Primary Antibodies

Order Details


    • Catalog Number
      NB120-15277
    • Availability
      Product Discontinued

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Dystrophin Antibody Summary

Immunogen
A synthetic peptide corresponding to C-terminal of human Dystrophin.
Localization
Cell Membrane
Specificity
Dystrophin
Clonality
Polyclonal
Host
Rabbit
Gene
DMD
Purity
Immunogen affinity purified
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Applications/Dilutions

Dilutions
  • Western Blot
  • Immunohistochemistry
  • Immunohistochemistry-Paraffin
Application Notes
IHC-P: Use at a dilution of 1/100 for 10 minutes at RT. Staining of formalin-fixed tissues requires boiling tissue sections in 1mM EDTA, pH 8.0 for 10 minutes followed by cooling at RT for 20 minutes.WB: Use at a concentration of 1 ug/ml for 1 hour. Predicted molecular weight: 427 kDa.
Positive Control
Skeletal Muscle Lysate (NB820-59253)

Reactivity Notes

Cross-reacts with Human and Rat. Not yet tested in other species.

Packaging, Storage & Formulations

Storage
Store at 4C short term. Aliquot and store at -20C long term. Avoid freeze-thaw cycles.
Buffer
10mM PBS (pH 7.4) and BSA
Preservative
Sodium Azide
Concentration
0.2 mg/ml
Purity
Immunogen affinity purified

Alternate Names for Dystrophin Antibody

  • BMDDXS272
  • CMD3B
  • DXS142
  • DXS164
  • DXS164, DXS206, DXS230, DXS239, DXS268, DXS269, DXS270, DXS272
  • DXS206
  • DXS230
  • DXS239
  • DXS268
  • DXS269
  • DXS270
  • dystrophin (muscular dystrophy, Duchenne and Becker types), includes DXS142
  • dystrophin

Background

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 extracellular matrix.

Limitations

This product is for research use only and is not approved for use in humans or in clinical diagnosis. Primary Antibodies are guaranteed for 1 year from date of receipt.

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Positive Control Lysate(s)

Secondary Antibodies

 

Isotype Controls

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Blogs on Dystrophin.

Could Laminin be Used to Treat Duchenne Muscular Dystrophy?
Duchenne muscular dystrophy (DMD) is a severe muscle wasting condition, causing disability and early death. There is currently no cure or adequate treatment for DMD, but pioneering research indicates that injection of a laminin protein may prevent (or...  Read full blog post.

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Bioinformatics

Gene Symbol DMD
Entrez