Dystrophin Antibody (DMD/3241) [DyLight 405]

• Reactivity: Hu
• Applications: ELISA, Endotoxin, IHC
• Clone: DMD/3241
• Clonality: Monoclonal
• Host: Mouse
• Conjugate: DyLight 405

Dystrophin Antibody (DMD/3241) [DyLight 405] Summary

• Description: This conjugate is made on demand and is not held in inventory. Each order represents a unique lot. All conjugates are generated using validated conjugation protocols and meet strict degree of labeling (F/P) specifications; additional information is available upon request. The antibody concentration is provided on the individual product label.
  
  For specialized conjugation needs, like large quantities, specific concentrations, and defined F/P ratios, bulk and custom antibody conjugation services are available.
• Immunogen: A recombinant fragment (around aa 114-263) of human DMD protein (exact sequence is proprietary) (Uniprot: P11532)
• Localization: Cell Surface, Cytoplasm
• Marker: Marker of Duchenne and Becker Muscular Dystrophy
• Isotype: IgG1 Kappa
• Clonality: Monoclonal
• Host: Mouse
• Gene: DMD
• Purity: Protein A or G purified

Applications/Dilutions

• Dilutions:
  • ELISA
  • Immunohistochemistry
  • Immunohistochemistry-Paraffin
  • Protein Array
• Application Notes: Recommended applications are based on validated applications from the unconjugated base product NBP2-79910. This conjugated antibody is not kept in inventory and is made to order using validated conjugation protocols.

Packaging, Storage & Formulations

• Storage: Store at 4C in the dark.
• Buffer: 50mM Sodium Borate
• Preservative: 0.05% Sodium Azide
• Purity: Protein A or G purified

Notes

DyLight (R) is a trademark of Thermo Fisher Scientific Inc. and its subsidiaries.

Alternate Names for Dystrophin Antibody (DMD/3241) [DyLight 405]

• 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.

Bioinformatics

• Gene Symbol: DMD