Neurological Disorders – Duchenne Muscular Dystrophy
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Duchenne muscular dystrophy (DMD) is a lethal genetic degenerative disease, which primarily affects voluntary muscles and begins in early childhood. It is an ad X-linked disease, characterized by a rapidly progressive weakness in muscles. Boys with DMD show early signs of muscle weakness, start walking relatively late in childhood, have waddling gait and difficulty climbing stairs. Their muscles show a false or pseudo-hypertrophy, most notably in the calf muscles, where the muscles appear large but are actually replaced by fat or fibrous tissue. The mdx mouse is used as a model for DMD. These mice develop muscle necrosis at 3 weeks old and show less severe myopathology compared to the human disease course. The comparatively mild phenotype of the mdx mice can, in part, be attributed to the compensatory function of the dystrophin-related protein utrophin, which is highly upregulated in regenerating muscle fibers in adult mdx mutants. PsychoGenics has developed new ways to behaviorally phenotype such muscular dystrophy animal models to demonstrate muscle weakness early on in age which can be used to screen acute, sub-chronic or chronic novel therapeutic entities.
The total number of errors (including reference memory and working memory errors) decreases over 4 days of training.
