Title of article :
Arrhythmogenic right ventricular cardiomyopathy: new insights into mechanisms of disease
Author/Authors :
Saffitz، نويسنده , , Jeffrey E. and Asimaki، نويسنده , , Angeliki and Huang، نويسنده , , Hayden، نويسنده ,
Issue Information :
روزنامه با شماره پیاپی سال 2010
Pages :
5
From page :
166
To page :
170
Abstract :
Arrhythmogenic right ventricular cardiomyopathy is a primary heart muscle disorder characterized by the early occurrence of arrhythmias often out of proportion to the extent of structural remodeling and contractile derangement. Approximately 40% of patients with arrhythmogenic right ventricular cardiomyopathy have one or more mutations in genes encoding proteins in desmosomes, intercellular adhesion junctions which, in cardiac myocytes, reside within intercalated disks. Some desmosomal proteins fulfill roles both as structural proteins in cell–cell adhesion junctions and as signaling molecules in pathways mediated by Wnt ligands. Evidence is increasing that mutations in desmosomal proteins can perturb the normal balance of critical proteins in junctions and the cytosol which, in turn, could alter gene expression by circumventing normal Wnt signaling pathways. This review highlights recent advances in understanding the pathogenesis of arrhythmogenic right ventricular cardiomyopathy and presents evidence suggesting that the disease is caused by a combination of altered cellular biomechanical behavior and altered signaling.
Keywords :
Biomechanical behavior , desmosome , plakoglobin , GAP JUNCTIONS , arrhythmias
Journal title :
Cardiovascular Pathology
Serial Year :
2010
Journal title :
Cardiovascular Pathology
Record number :
1845655
Link To Document :
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