Dystrophin Antibody (MANDRA1)

Immunohistochemistry-Frozen: Dystrophin Antibody (MANDRA1) [NB120-7164] - Staining of frozen human tongue sections with 1:100 Monoclonal Anti-Dystrophin, Clone: MANDRA1 using Goat Anti-Mouse IgG, FITC-conjugate.

Product Discontinued

Dystrophin Antibody (MANDRA1) Summary

• Immunogen: Fusion protein, corresponding to amino acids 3200-3684 of Human Dystrophin.
• Localization: Cell Membrane
• Specificity: The antibody recognizes an epitope located on the 128 amino acids at the end of the C-terminal domain of the human dystrophin molecule (amino acid residues 3558-3684).
• Isotype: IgG1
• Clonality: Monoclonal
• Host: Mouse
• Gene: DMD
• Purity: Unpurified

Applications/Dilutions

• Dilutions:
  • ELISA 1:100-1:2000
  • Immunocytochemistry/ Immunofluorescence 1:100
  • Immunohistochemistry
  • Immunohistochemistry-Frozen 1:10-1:500
  • Microarray
• Application Notes: ELISA: See Nyugen thi Man et al (1992) and Morris G.E et al (1993) IF: Use freshly dissected and frozen human or animal muscle tissue. Immunohistochemical staining of muscle tissue results in a clear labeling confined to the periphery (plasma membrane) of normal striated muscle fibers.

Reactivity Notes

Please note that this antibody is reactive to Mouse and derived from the same host, Mouse. Additional Mouse on Mouse blocking steps may be required for IHC and ICC experiments. Please contact Technical Support for more information.

Packaging, Storage & Formulations

• Storage: Store at 4C short term. Aliquot and store at -20C long term. Avoid freeze-thaw cycles.
• Buffer: Ascites
• Preservative: 0.09% Sodium Azide
• Purity: Unpurified

Alternate Names for Dystrophin Antibody (MANDRA1)

• 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 (NB120-7164)(4)

Publications using NB120-7164

• Stay TL, Miterko LN, Arancillo M et al. In vivo cerebellar circuit function is disrupted in an mdx mouse model of Duchenne muscular dystrophy Dis Model Mech 2019-11-08 [PMID: 31704708] (IF/IHC, Mouse)
• Annese T, Corsi P, Ruggieri S et al. Isolation and characterization of neural stem cells from dystrophic mdx mouse. Exp. Cell Res. 2016-03-22 [PMID: 27015747] (ICC/IF, Mouse)
• Morris GE, Ellis JM, Nguyen TM et al. A quantitative ELISA for dystrophin. J Immunol Methods. 1993-05-01 [PMID: 8486926]
• Nguyen TM, Ginjaar IB, van Ommen GJ et al. Monoclonal antibodies for dystrophin analysis. Epitope mapping and improved binding to SDS-treated muscle sections. Biochem J. 1992-12-01 [PMID: 1281410]

Bioinformatics

• Gene Symbol: DMD
• Entrez:
  • 1756(Human)
  • 13405(Mouse)
  • 24907(Rat)