Dystrophin Antibody / DMD

SKU: orb1822601

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: Immunohistochemistry (FFPE): 1-2ug/ml for 30 min at RT
• Reactivity: Human
• Application Notes:
  Optimal dilution of the Dystrophin antibody should be determined by the researcher.

Key Properties

• Antibody Type: Primary Antibody
• Host: Rabbit
• Clonality: Recombinant
• Isotype: Rabbit IgG, kappa
• Clone No.: DMD/8773R
• Immunogen: A recombinant partial protein sequence (within amino acids 1700-2300) from the human protein was used as the immunogen for the Dystrophin antibody.
• Purification: Protein A/G affinity
• 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 human skeletal muscle tissue with Dystrophin antibody (clone DMD/8773R). HIER: boil tissue sections in pH9 10 mM Tris with 1 mM 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.

Quick Database Links

UniProt

P11532