Ion channel impairments in dystrophic cardiomyocytes

Background Muscular dystrophies comprise a heterogeneous group of inherited diseases that are characterized by progressive muscle weakness and degeneration. Severe forms, e.g. Duchenne muscular dystrophy (DMD), which is caused by a mutation in the dystrophin gene, lead to loss of ambulation, respiratory failure, and premature death. In many types of the muscular dystrophies the cardiac muscle is also affected cardiomyopathy and/or cardiac arrhythmias regularly represent life threatening complications. The current understanding of the pathomechanisms underlying these cardiac diseases in various muscular dystrophies is still very limited. Here we tested the hypothesis that dysfunctional ion channels may be critically involved in dystrophy-associated cardiac disease.