Cells (Jul 2024)

In Vivo Approaches to Understand Arrhythmogenic Cardiomyopathy: Perspectives on Animal Models

  • Giovanni Risato,
  • Raquel Brañas Casas,
  • Marco Cason,
  • Maria Bueno Marinas,
  • Serena Pinci,
  • Monica De Gaspari,
  • Silvia Visentin,
  • Stefania Rizzo,
  • Gaetano Thiene,
  • Cristina Basso,
  • Kalliopi Pilichou,
  • Natascia Tiso,
  • Rudy Celeghin

DOI
https://doi.org/10.3390/cells13151264
Journal volume & issue
Vol. 13, no. 15
p. 1264

Abstract

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Arrhythmogenic cardiomyopathy (AC) is a hereditary cardiac disorder characterized by the gradual replacement of cardiomyocytes with fibrous and adipose tissue, leading to ventricular wall thinning, chamber dilation, arrhythmias, and sudden cardiac death. Despite advances in treatment, disease management remains challenging. Animal models, particularly mice and zebrafish, have become invaluable tools for understanding AC’s pathophysiology and testing potential therapies. Mice models, although useful for scientific research, cannot fully replicate the complexity of the human AC. However, they have provided valuable insights into gene involvement, signalling pathways, and disease progression. Zebrafish offer a promising alternative to mammalian models, despite the phylogenetic distance, due to their economic and genetic advantages. By combining animal models with in vitro studies, researchers can comprehensively understand AC, paving the way for more effective treatments and interventions for patients and improving their quality of life and prognosis.

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