Dystrophin Antibody / DMD

• Catalog Number: orb1822602
• Category: Antibodies
• 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.
• Species/Host: Rabbit
• Clonality: Recombinant
• Clone Number: DMD/8773R
• Tested applications: IHC-P
• Reactivity: Human
• Isotype: Rabbit IgG, kappa
• Immunogen: A recombinant partial protein sequence (within amino acids 1700-2300) from the human protein was used as the immunogen for the Dystrophin antibody.
• Dilution range: Immunohistochemistry (FFPE): 1-2ug/ml for 30 min at RT
• Conjugation: Unconjugated
• Formula: 0.2 mg/ml in 1X PBS with 0.1 mg/ml BSA (US sourced), 0.05% sodium azide
• Hazard Information: This Dystrophin antibody is available for research use only.
• UniProt ID: P11532
• Storage: Aliquot the Dystrophin antibody and store frozen at -20°C or colder. Avoid repeated freeze-thaw cycles.
• Note: For research use only
• Expiration Date: 12 months from date of receipt.

IHC staining of FFPE human skeletal muscle tissue with Dystrophin antibody (clone DMD/8773R). HIER: boil tissue sections in pH 9 10mM Tris with 1mM EDTA for 20 min and allow to cool before testing.

SDS-PAGE analysis of purified, BSA-free Dystrophin antibody (clone DMD/8773R) as confirmation of integrity and purity.