Cardiac phenotype of Duchenne Muscular Dystrophy: Insights from cellular studies

Shirokova, Natalia; Niggli, Ernst (2013). Cardiac phenotype of Duchenne Muscular Dystrophy: Insights from cellular studies. Journal of molecular and cellular cardiology, 58, pp. 217-224. Elsevier 10.1016/j.yjmcc.2012.12.009

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Text JMCC7037R1.pdf - Accepted Version Available under License Publisher holds Copyright. This is the author's version of a work that was accepted for publication in Journal of Molecular and Cellular Cardiology. Changes may have been made to this work since it was submitted for publication. A definitive version was subsequently published see http://dx.doi.org/10.1016/j.yjmcc.2012.12.009. Download (4MB) | Preview

Dilated cardiomyopathy is a serious and almost inevitable complication of Duchenne Muscular Dystrophy, a devastating and fatal disease of skeletal muscle resulting from the lack of functional dystrophin, a protein linking the cytoskeleton to the extracellular matrix. Ultimately, it leads to congestive heart failure and arrhythmias resulting from both cardiac muscle fibrosis and impaired function of the remaining cardiomyocytes. Here we summarize findings obtained in several laboratories, focusing on cellular mechanisms that result in degradation of cardiac functions in dystrophy. This article is part of a Special Issue entitled "Calcium Signaling in Heart".

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