Dystrophin Antibody / DMD

SKU: orb2642783

Description

Dystrophin-glycoprotein complex (DGC) connects the F-Actin cytoskeleton on the inner surface of muscle fibers to the surrounding extracellular matrix, through the cell membrane interface. A deficiency in this protein contributes to Duchenne (DMD) and Becker (BMD) muscular dystrophies. The human dystrophin gene measures 2.4 megabases, has more than 80 exons, produces a 14 kb mRNA and contains at least 8 independent tissue-specific promoters and 2 poly A sites. The dystrophin mRNA can undergo differential splicing and produce a range of transcripts that encode a large set of proteins. Dystrophin represents approximately 0.002% of total striated muscle protein and localizes to triadic junctions in skeletal muscle, where it is thought to influence calcium ion homeostasis and force transmission.

Images & Validation

• Tested Applications: IHC-P
• Dilution range: The prediluted format is supplied in a dropper bottle and is optimized for use in IHC. After epitope retrieval step (if required), drip mAb solution onto the tissue section and incubate at RT for 30 min.
• Reactivity: Human
• Application Notes:
  Optimal dilution of the Dystrophin antibody should be determined by the researcher.1. The prediluted format is supplied in a dropper bottle and is optimized for use in IHC. After epitope retrieval step (if required), drip mAb solution onto the tissue section and incubate at RT for 30 min.

Key Properties

• Antibody Type: Primary Antibody
• Host: Mouse
• Clonality: Monoclonal
• Isotype: Mouse IgG2a, kappa
• Clone No.: DMD/3245
• Immunogen: Amino acids 114-263 from the human protein were used as the immunogen for the Dystrophin antibody.
• Purification: Protein G affinity chromatography
• Conjugation: Unconjugated

Storage & Handling

• Storage: Maintain refrigerated at 2-8°C for up to 2 weeks. For long term storage store at -20°C in small aliquots to prevent freeze-thaw cycles.
• Disclaimer: For research use only

IHC staining of FFPE skeletal muscle with Dystrophin antibody (clone DMD/3245). HIER: boil tissue sections in pH6, 10mM citrate buffer, for 10-20 min and allow to cool before testing.

SDS-PAGE analysis of purified, BSA-free Dystrophin antibody (clone DMD/3245) as confirmation of integrity and purity.

Quick Database Links

UniProt

P11532