Monoclonal Anti-Dystrophin (mouse IgG2b isotype) is derived from the MANDYS8 hybridoma produced by the fusion of mouse myeloma cells and splenocytes from an immunized mouse. Dystrophin is 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.
In striated muscle cells, DMD (dystrophin) acts as a component of the multimeric protein dystrophin-associated glycoprotein (DGC) complex. It helps DGC to adhere with the extracellular matrix for various signalling pathways. It helps to maintain structural assembly of plasma membrane, regulates ion homeostasis and transmembrane signaling cascade. It stabilzes muscle fiber cells. It is associated with various muscular dystrophies, such as Duchenne/Becker muscular dystrophy, congenital muscular dystrophy and different forms of limb-girdle muscular dystrophy.
