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. Arrhythmogenic cardiomyopathy (AC) is a heart muscle disease characterized by a progressive replacement of the ventricular myocardium with adipose and fibrous tissue. This disease is often associated with mutations in genes encoding desmosomal proteins in the majority of patients. Based on results...
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Published in | Future cardiology Vol. 9; no. 4; pp. 467 - 470 |
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Main Authors | , , |
Format | Journal Article |
Language | English |
Published |
Future Medicine Ltd
01.07.2013
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Subjects | |
Online Access | Get full text |
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Summary: | . Arrhythmogenic cardiomyopathy (AC) is a heart muscle disease characterized by a progressive replacement of the ventricular myocardium with adipose and fibrous tissue. This disease is often associated with mutations in genes encoding desmosomal proteins in the majority of patients. Based on results obtained from recent experimental models, a disturbed distribution of gap junction proteins and cardiac sodium channels may also be observed in AC phenotypes, secondary to desmosomal dysfunction. The study from Noorman et al. examined heart sections from patients diagnosed with AC and performed immunohistochemical analyses of N-cadherin, PKP2, PKG, Cx43 and the cardiac sodium channel Na
1.5. Altered expression/distribution of Cx43, PKG and Na
1.5 was found in most cases of patients with AC. The altered expression and/or distribution of Na
1.5 channels in AC hearts may play a mechanistic role in the arrhythmias leading to sudden cardiac death in AC patients. Thus, Na
1.5 should be considered as a supplemental element in the evaluation of risk stratification and management strategies. However, additional experiments are required to clearly understand the mechanisms leading to AC phenotypes. |
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ISSN: | 1479-6678 1744-8298 |
DOI: | 10.2217/fca.13.38 |