Abstract
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.
Highlights
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
We found that dystrophic cardiomyocytes show reduced sodium current density compared to wild-type cardiomyocytes
Summary
Muscular dystrophies comprise a heterogeneous group of inherited diseases that are characterized by progressive muscle weakness and degeneration. Address: 1Institute of Pharmacology, Center for Biomolecular Medicine and Pharmacology, Medical University of Vienna, 1090 Vienna, Austria and 2Department of Applied Anatomy, Center for Anatomy and Cell Biology, Medical University of Vienna, 1090 Vienna, Austria Email: Karlheinz Hilber* - karlheinz.hilber@meduniwien.ac.at * Corresponding author from 15th Scientific Symposium of the Austrian Pharmacological Society (APHAR) Joint meeting with the Hungarian Society of Experimental and Clinical Pharmacology (MFT) and the Slovenian Pharmacological Society (SDF) Graz, Austria. Published: 12 November 2009 BMC Pharmacology 2009, 9(Suppl 2):A31 doi:10.1186/1471-2210-9-S2-A31
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