Description
Dystrophin Antibody / DMD | V7540SAF-100UG | Gentaur US, UK & Europe Disrtribition
Family: Primary antibody
Formulation: 1 mg/ml in 1X PBS; BSA free, sodium azide free
Format: Purified
Clone: DMD/3245
Host Animal: Mouse
Clonality: Monoclonal (mouse origin)
Species Reactivity: Human
Application: ELISA, IHC-P
Buffer: N/A
Limitation: This Dystrophin antibody is available for research use only.
Purity: Protein G affinity chromatography
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.
Immunogen: Amino acids 114-263 from the human protein were used as the immunogen for the Dystrophin antibody.
Storage: Store the Dystrophin antibody at 2-8oC (with azide) or aliquot and store at -20 °C or colder (without azide).
