DMD TREATMENT: ANIMAL MODELS

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Gastrointestinal dysfunction is highly prevalent in Duchenne muscular dystrophy (DMD) with increased symptomatology over time. DMD is caused by a mutation in the gene that encodes for the protein dystrophin. Dp71 is one of the dystrophin isoforms that has been described to be expressed in non-muscle tissue. However, its distribution in the intestine has not been studied in detail. Here we examined the potential aetiology in a physiological model by analysing the expression of Dp71 relative to alpha-smooth muscle actin (aSMA) in six anatomic regions of the intestine, i.e., duodenum, jejunum, ileum, caecum, colon, and rectum from neonatal (24-hours postnatal; n=3), and adult (six months-old; n=5-12) rats.

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