Characterization of a Dmd EGFP reporter mouse as a tool to investigate dystrophin expression

Background Dystrophinis a rod-shaped cytoplasmic protein that provides sarcolemmal stability as a structural link between the cytoskeleton and the extracellular matrix via the dystrophin-associated proteincomplex (DAPC). Mutations in the dystrophin-encoding DMD gene cause X-linked dystrophinopathies with variable phenotypes, the most severe being Duchenne muscular dystrophy(DMD) characterized by progressive muscle wasting and fibrosis. However, dystrophindeficiency does not only impair the function of skeletal and heart muscle but may also affect other organ systems such as the brain, eye, and gastrointestinal tract. The generation of a dystrophinreporter mouse would facilitate research into dystrophinmuscular and extramuscular pathophysiology without the need for immunostaining.