Age- and training-dependent development of arrhythmogenic right ventricular cardiomyopathy in heterozygous plakoglobin-deficient mice

  • Kirchhof P
  • Fabritz L
  • Zwiener M
 et al. 
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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

Author-supplied keywords

  • Adherens junctions
  • Arrhythmia
  • Cardiomyopathy
  • Conduction
  • Death, sudden
  • Desmosomes
  • Exercise

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  • Paulus Kirchhof

  • Larissa Fabritz

  • Melanie Zwiener

  • Henning Witt

  • Michael Schäfers

  • Stephan Zellerhoff

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