Modeling Cardiomyopathy and Arrhythmias in Induced Pluripotent Stem Cell-Derived Cardiomyocytes.

摘要: See Article by El-Battrawy et al Dilated cardiomyopathy (DCM) are genetic heart diseases associated with arrhythmias, which sometimes fatal, and progressive failure leading to transplantation.1 An overlap of DCM muscular dystrophies is well known was first noted in the case dystrophinopathies, Duchenne Becker dystrophies, where Muscular Dystrophy gene ( DMD  ) mutations lead both skeletal cardiac muscle diseases. In some instances, a cardiac-only phenotype sudden death, X-linked DCM, results from mutations.2 The discovery arrhythmias provided connection between lamin A/C LMNA ) DCM. responsible for variety phenotypes including LGMD1B (limb-girdle dystrophy type 1B), Emery–Dreifuss dystrophy, or without conduction disease subclinical involvement.3 caused have been extensively studied, it has shown that can cause particularly malignant terms refractory severe ventricular causing premature death. identification distinct arrhythmogenic laminopathies had an important clinical impact and, new 2017 American Heart Association/American College Cardiology/Heart Rhythm Society guidelines, prompted specific recommendations prevention …