Dystrophin Antibody

Images

 
Orthogonal Strategies: Immunohistochemistry-Paraffin: Dystrophin Antibody [NBP1-89953] - Staining in human skeletal muscle and tonsil tissues . Corresponding DMD RNA-seq data are presented for the same tissues.
Immunohistochemistry-Paraffin: Dystrophin Antibody [NBP1-89953] - Staining of human heart muscle shows strong membranous positivity in myocytes.
Immunohistochemistry-Paraffin: Dystrophin Antibody [NBP1-89953] - Staining of human skeletal muscle shows strong membranous positivity in myocytes.
Immunohistochemistry-Paraffin: Dystrophin Antibody [NBP1-89953] - Staining of human tonsil shows no positivity as expected.

Product Details

Summary
Reactivity Hu, Mu, RtSpecies Glossary
Applications ICC/IF, IHC
Clonality
Polyclonal
Host
Rabbit
Conjugate
Unconjugated
Validated by:
       

Orthogonal Strategies

 

Order Details

Dystrophin Antibody Summary

Immunogen
This antibody was developed against Recombinant Protein corresponding to amino acids: KQNDVHRAFKRELKTKEPVIMSTLETVRIFLTEQPLEGLEKLYQEPRELPPEERAQNVTRLLRKQAEEVNTEWEKLNLHSADWQRKIDETLERLQELQEATDELDLKLRQAEVIKGSWQPVGDLLIDSLQDHLEKVKALRGEIAPLKENV
Predicted Species
Rat (98%). Backed by our 100% Guarantee.
Isotype
IgG
Clonality
Polyclonal
Host
Rabbit
Gene
DMD
Purity
Immunogen affinity purified
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Applications/Dilutions

Dilutions
  • Immunocytochemistry/ Immunofluorescence -Reported in scientific literature (PMID 27374118).
  • Immunohistochemistry 1:2500 - 1:5000
  • Immunohistochemistry-Paraffin 1:2500 - 1:5000
Application Notes
For IHC-Paraffin, HIER pH 6 retrieval is recommended.
Control Peptide
Dystrophin Protein (NBP1-89953PEP)
Publications
Read Publications using
NBP1-89953 in the following applications:

Reactivity Notes

Mouse reactivity reported in scientific literature (PMID: 26721911).

Packaging, Storage & Formulations

Storage
Store at 4C short term. Aliquot and store at -20C long term. Avoid freeze-thaw cycles.
Buffer
PBS (pH 7.2) and 40% Glycerol
Preservative
0.02% Sodium Azide
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

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.

Publications for Dystrophin Antibody (NBP1-89953)(4)

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Product General Protocols

Find general support by application which include: protocols, troubleshooting, illustrated assays, videos and webinars.

Video Protocols

ICC/IF Video Protocol

FAQs for Dystrophin Antibody (NBP1-89953). (Showing 1 - 1 of 1 FAQs).

  1. Do you know if this antibody has been used in paraffin or frozens section using immunofluorescence? Also, is there any cross-reactivity with mouse?
    • Yes this antibody has been validated for use in IHC-P in Human but we predict there will be cross reactivity to mouse based on the 96% homology. The human immunogen sequence homology is 96% homologous with the mouse protein.

Secondary Antibodies

 

Isotype Controls

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Research Areas for Dystrophin Antibody (NBP1-89953)

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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