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Dystrophin Antibody (DMD/3244) [FITC]

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Product Details

Summary
Reactivity HuSpecies Glossary
Applications ELISA, IHC, MA
Clone
DMD/3244
Clonality
Monoclonal
Host
Mouse
Conjugate
FITC

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Dystrophin Antibody (DMD/3244) [FITC] Summary

Immunogen
A recombinant fragment (around aa 114-263) of human DMD protein (exact sequence is proprietary) (Uniprot: P11532)
Localization
Cell Surface and Cytoplasmic
Marker
Marker of Duchenne and Becker Muscular Dystrophy
Isotype
IgG1 Kappa
Clonality
Monoclonal
Host
Mouse
Gene
DMD
Purity
Protein A or G purified
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Applications/Dilutions

Dilutions
  • ELISA
  • Immunohistochemistry-Paraffin
  • Protein Array
Application Notes
Optimal dilution of this antibody should be experimentally determined.

Packaging, Storage & Formulations

Storage
Store at 4C in the dark.
Buffer
PBS
Preservative
0.05% Sodium Azide
Purity
Protein A or G purified

Alternate Names for Dystrophin Antibody (DMD/3244) [FITC]

  • 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

Dystrophin is a muscle membrane protein (427 kDa) which is absent, reduced or altered as a result of mutation in Duchenne and Becker muscular dystrophies (DMD/BMD) or its homologue in the mouse.8 Severe DMD is associated with a marked dystrophin deficiency whereas patients with the milder form of BMD show less pronounced abnormalities of protein expression. Because 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. Predictions from the sequence suggest a structural protein on the inner face of the membrane, consisting of a 25-repeat, rod-like triple-helical domain separating an N-terminal actin-binding domain from two C-terminal domains, one of which is rich in cysteine.9 The large size of dystrophin and its low abundance (<0.01% of the total muscle protein) are a hindrance to the isolation of intact, native protein for structure/function studies. Monoclonal antibodies against defined regions10 of dystrophin provide a means for studying its structure and function, interactions with other proteins and the nature of the partial gene products produced in some patients carrying deletions in the dystrophin gene. The antibodies are useful in the prenatal or post-abortion diagnosis of muscular dystrophy carriers by immunohistological analyses.11

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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Secondary Antibodies

 

Isotype Controls

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Research Areas for Dystrophin Antibody (NBP3-08702F)

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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