Intercalated disc abnormalities, reduced Na(+) current density, and conduction slowing in desmoglein-2 mutant mice prior to cardiomyopathic changes.

@article{Rizzo2012IntercalatedDA,
  title={Intercalated disc abnormalities, reduced Na(+) current density, and conduction slowing in desmoglein-2 mutant mice prior to cardiomyopathic changes.},
  author={Stefania Rizzo and Elisabeth M Lodder and Arie O. Verkerk and Rianne Wolswinkel and Leander Beekman and Kalliopi Pilichou and Cristina Basso and Carol Ann Remme and Gaetano Thiene and Connie R. Bezzina},
  journal={Cardiovascular research},
  year={2012},
  volume={95 4},
  pages={409-18}
}
AIMS Mutations in genes encoding desmosomal proteins have been implicated in the pathogenesis of arrhythmogenic right ventricular cardiomyopathy (ARVC). However, the consequences of these mutations in early disease stages are unknown. We investigated whether mutation-induced intercalated disc remodelling impacts on electrophysiological properties before the onset of cell death and replacement fibrosis. METHODS AND RESULTS Transgenic mice with cardiac overexpression of mutant Desmoglein2 (Dsg2… CONTINUE READING