Modeling of arrhythmogenic right ventricular cardiomyopathy with human induced pluripotent stem cells.

@article{Caspi2013ModelingOA,
  title={Modeling of arrhythmogenic right ventricular cardiomyopathy with human induced pluripotent stem cells.},
  author={Oren Caspi and Irit Huber and Amira Gepstein and Gil Arbel and Leonid Maizels and Monther Boulos and Lior Gepstein},
  journal={Circulation. Cardiovascular genetics},
  year={2013},
  volume={6 6},
  pages={557-68}
}
BACKGROUND Arrhythmogenic right ventricular cardiomyopathy (ARVC) is a primary heart muscle disorder resulting from desmosomal protein mutations. ARVC is characterized pathologically by fibrofatty infiltration and clinically by arrhythmias and sudden cardiac death. We aimed to establish a patient-/disease-specific human induced pluripotent stem cell (hiPSC) model of ARVC. METHODS AND RESULTS Dermal fibroblasts were obtained from 2 patients with ARVC with plakophilin-2 (PKP2) mutations… CONTINUE READING
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