Preload-induced cardiomyocyte calcium overload and ventricular damage in isolated hearts from young Duchenne muscular dystrophy (Dmdmdx-4CV) mice

Abstract

Duchenne muscular dystrophy (DMD) is caused by mutations in the dystrophin gene, which leads to structural and functional defects of the muscle cell sarcolemma. Patients with DMD progress to dilated cardiomyopathy leading to sudden cardiac death. Dystrophin deficiency associates with stretch-induced membrane instability and/or stretch-activated ion channel activation leading to cellular calcium overload. In this study we test the hypothesis that dystrophin deficiency leads to calcium handling abnormalities and cardiomyocyte damage following ventricular preload challenge in ex vivo perfused hearts.