Progressive cardiomyopathy with intercalated disc disorganization in a rat model of Becker dystrophy
Valentina Taglietti,
Kaouthar Kefi,
Busra Mirciloglu
et al.
Abstract:Becker muscular dystrophy (BMD) is an X-linked disorder due to in-frame mutations in the DMD gene, leading to a less abundant and truncated dystrophin. BMD is less common and severe than Duchenne muscular dystrophy (DMD) as well as less investigated. To accelerate the search for innovative treatments, we developed a rat model of BMD by deleting the exons 45–47 of the Dmd gene. Here, we report a functional and histopathological evaluation of these rats during their first year of life, compared to DMD and contro… Show more
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