Metabolic Alterations in Cardiomyocytes of Patients with Duchenne and Becker Muscular Dystrophies.

ABSTRACT: Duchenne and Becker muscular dystrophies (DMD/BMD) result in progressive weakness of skeletal and cardiac muscles due to the deficiency of functional dystrophin. Respiratory failure is a leading cause of mortality in DMD patients; however, improved management of the respiratory symptoms have increased patients' life expectancy, thereby also increasing the clinical relevance of heart disease. In fact, the prevalence of cardiomyopathy, which significantly contributes to mortality in DMD patients, increases with age and disease progression, so that over 95% of adult patients has cardiomyopathy signs. We here review the current literature featuring the metabolic alterations observed in the dystrophic heart of the mdx mouse, i.e., the best-studied animal model of the disease, and discuss their

SUBMITTER: Esposito G 

PROVIDER: S-EPMC6947625 | biostudies-literature | 2019 Dec

REPOSITORIES: biostudies-literature