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Recent advances in understanding disease mechanisms in arrhythmogenic cardiomyopathy implicate the remodeling of intercalated disk proteins and aberrant cell signaling pathways.
This review highlights the role of intercalated disk protein remodeling and aberrant cell signaling in the pathogenesis of arrhythmogenic cardiomyopathy.
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Intercalated disk remodeling may drive early arrhythmias in AC; leaves open mechanistic targets for prospective validation.
Arrhythmogenic cardiomyopathy (AC) is a primary myocardial disorder characterized by a high incidence of ventricular arrhythmias often preceding the onset of ventricular remodeling and dysfunction. Approximately 50% of patients diagnosed with AC have one or more mutations in genes encoding desmosomal proteins, although non-desmosomal genes have also been associated with the disease. Increasing evidence implicates remodeling of intercalated disk proteins reflecting abnormal responses to mechanical load and aberrant cell signaling pathways in the pathogenesis of AC. This review summarizes recent advances in understanding disease mechanisms in AC that have come from studies of human myocardium and experimental models.
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Asimaki et al. (2014) conducted a review in Arrhythmogenic cardiomyopathy. Recent advances in understanding disease mechanisms in arrhythmogenic cardiomyopathy implicate the remodeling of intercalated disk proteins and aberrant cell signaling pathways.
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