Disease relevance of DMD

• Dystrophin-related protein (DRP or utrophin) is an autosomal homologue of dystrophin, a membrane cytoskeletal protein encoded by the Duchenne muscular dystrophy gene [1].

High impact information on DMD

• These confocal microscopic observations suggest that dystrophin may be localized in a nonuniform, discontinuous pattern along the sarcolemma and in some relationship with the underlying myofibrils [2].
• Confocal laser microscopy of dystrophin localization in guinea pig skeletal muscle fibers [2].
• When longitudinal and transverse sections of muscle fibers were examined, the immunostaining of dystrophin was seen as linearly aligned fluorescent dots or intermittent lines along the sarcolemma [2].
• Double staining for dystrophin and vinculin showed that the two proteins were not exactly colocalized [2].
• In analogy to dystrophin of muscle cells, DRP could be playing an important role in the mechanical stress mechanisms of endothelial cells [1].

Anatomical context of DMD

• Purification of dystrophin-related protein (utrophin) from lung and its identification in pulmonary artery endothelial cells [1].
• This distinct and complementary distribution in various domains of spermatozoa may reveal a specific function for each short dystrophin family product, in the stabilization of the domains where they are located [3].

Analytical, diagnostic and therapeutic context of DMD

• In this study, the presence and cellular distribution of dystrophin family products (i.e. Dp71d, Dp71f-like protein and dystrobrevin) was examined by indirect immunofluorescence and Western blotting in guinea pig spermatozoa [3].

References