Key result
Heterozygous plakoglobin deficiency in mice increased right ventricular volume, reduced function, and caused spontaneous ventricular ectopy (all P<0.05), which was accelerated by endurance training.
Why the study?
Reduced desmosomal protein expression, specifically heterozygous plakoglobin deficiency, may cause ARVC and its development may be influenced by endurance training.
Does heterozygous plakoglobin deficiency and endurance training provoke arrhythmogenic right ventricular cardiomyopathy in mice?
Does heterozygous plakoglobin deficiency and endurance training provoke arrhythmogenic right ventricular cardiomyopathy in mice?
Answers are grounded in this paper and cite their sources.
BACKGROUND: Arrhythmogenic right ventricular cardiomyopathy (ARVC) is an inherited disorder that causes sudden death and right ventricular heart failure in the young. Clinical data suggest that competitive sports may provoke ARVC in susceptible persons. Genetically, loss-of-function mutations in desmosomal proteins (plakophilin, desmoplakin, or plakoglobin) have been associated with ARVC. To test the hypothesis that reduced desmosomal protein expression causes ARVC, we studied the cardiac effects of heterozygous plakoglobin deficiency in mice. METHODS AND RESULTS: Ten-month-old heterozygous plakoglobin-deficient mice (plakoglobin+/-) had increased right ventricular volume, reduced right ventricular function, and spontaneous ventricular ectopy (all P<0.05). Left ventricular size and function were not altered. Isolated, perfused plakoglobin+/- hearts had spontaneous ventricular tachycardia of right ventricular origin and prolonged right ventricular conduction times compared with wild-type hearts. Endurance training accelerated the development of right ventricular dysfunction and arrhythmias in plakoglobin+/- mice. Histology and electron microscopy did not identify right ventricular abnormalities in affected animals. CONCLUSIONS: Heterozygous plakoglobin deficiency provokes ARVC. Manifestation of the phenotype is accelerated by endurance training. This suggests a functional role for plakoglobin and training in the development of ARVC.
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Kirchhof et al. (2006) studied Arrhythmogenic right ventricular cardiomyopathy. Heterozygous plakoglobin deficiency and endurance training vs. Wild-type mice was evaluated on Right ventricular volume, right ventricular function, and spontaneous ventricular ectopy (p=<0.05). Heterozygous plakoglobin deficiency in mice increased right ventricular volume, reduced function, and caused spontaneous ventricular ectopy (all P<0.05), which was accelerated by endurance training.
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