Application

Anti-Dystrophin antibody, Mouse monoclonal has been used in: immunohistochemistry immunoblotting immunofluorescence enzyme-linked immunosorbent assay (ELISA)

Biochem/physiol Actions

Dystrophin is absent, reduced or altered because of mutation in Duchenne and Becker muscular dystrophies (DMD/BMD) or in its homologue in mouse. Severe DMD is associated with a marked Dystrophin deficiency whereas patients with the milder form of BMD show less pronounced abnormalities of protein expression.

Disclaimer

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General description

Anti-Dystrophin antibody, Mouse monoclonal (mouse IgG1 isotype) is derived from the MANDRA1 hybridoma produced by the fusion of mouse myeloma cells and splenocytes from BALB/c mice. Dystrophin is a rod-shaped cytoskeletal protein located to the periphery (plasma membrane) of normal striated muscle fibers.

Dystrophin is encoded by the gene mapped to human chromosome Xp21.2-p21.1.

Physical form

Solution in 0.01 M phosphate buffered saline, pH 7.4, containing 15 mM sodium azide.

Specificity

Anti-Dystrophin antibody, Mouse monoclonal specifically recognizes an epitope located on the 128 amino acids at the end of the C-terminal domain of the human Dystrophin molecule (residues 3558-3684). The antibody reacts with Dystrophin from human, mouse, rat and zebrafish origin. The antibody is specific to dystrophin and does not react with -actinin or utrophin.

Storage and Stability

For continuous use, store at 2–8 °C for up to one month. For extended storage, freeze in working aliquots. Repeated freezing and thawing is not recommended. If slight turbidity occurs upon prolonged storage, clarify the solution by centrifugation before use. Working dilution samples should be discarded if not used within 12 hours.