indirect ELISA: suitable, indirect immunofluorescence: 1:400 using frozen human or animal muscle tissue sections., microarray: suitable, western blot: suitable
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.
The rod domain of the human dystrophin molecule is present in normal muscle tissue and in nearly all Becker muscular dystrophies. It is absent in the cases of Duchenne muscular dystrophies and in the dystrophic mouse (mdx).
Immunogen
recombinant human dystrophin fragment.
Application
Monoclonal Anti-Dystrophin antibody produced in mouse has been used in
Monoclonal anti-dystrophin antibody can be used for localization of dystrophin using immunochemical assays like ELISA and immunohistochemistry. The antibody can also be used in immunoblotting for brain dystrophin. Further, it can be used in western blotting and double immunofluorescent labelling (diluted 1: 500) of dystrophin.
Biochem/physiol Actions
Dystrophin deficiency is associated with severe Duchenne muscular dystrophy (DMD). Becker muscular dystrophy (BMD) show less pronounced abnormalities of dystrophin protein expression. Since abnormalities in the protein expression occur specifically in patients with these types of muscular dystrophy, dystrophin analysis may be used to distinguish these conditions from other neuromuscular diseases.
Other Notes
This product can be found as purified product that was produced using cell culture hybridoma product. SAB4200764 Anti-Dystrophin antibody, Mouse monoclonal clone MANDYS8, purified from hybridoma cell culture
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