Arrhythmogenic Cardiomyopathy: the Guilty Party in Adipogenesis

J Cardiovasc Transl Res. 2017 Dec;10(5-6):446-454. doi: 10.1007/s12265-017-9767-8. Epub 2017 Oct 5.

Abstract

Arrhythmogenic cardiomyopathy (ACM) is a genetic cardiac condition characterized by the replacement of the ventricular myocardium with fibro-fatty tissue, by arrhythmias and sudden death. Adipogenesis in ACM is considered an aberrant remodeling following myocardial loss. Which cell type(s) is (are) responsible for the adipose replacement is still matter of debate. A systematic overview of the different cells that have been, over time, considered as main players in adipose replacement is provided. The comprehension of the cellular component giving rise to arrhythmogenic cardiomyopathy substrate defects may represent both an essential tool for mechanistic studies of disease pathogenesis and a novel possible therapeutic target.

Keywords: ARVC; Adipogenesis; Arrhythmogenic cardiomyopathy; Cardiomyocytes; Mesenchymal cells; Progenitors.

Publication types

  • Review

MeSH terms

  • Adipogenesis*
  • Adipose Tissue / metabolism
  • Adipose Tissue / pathology
  • Adipose Tissue / physiopathology*
  • Animals
  • Arrhythmogenic Right Ventricular Dysplasia / genetics
  • Arrhythmogenic Right Ventricular Dysplasia / metabolism
  • Arrhythmogenic Right Ventricular Dysplasia / pathology
  • Arrhythmogenic Right Ventricular Dysplasia / physiopathology*
  • Cell Transdifferentiation
  • Fibrosis
  • Humans
  • Myocardium* / metabolism
  • Myocardium* / pathology
  • Myocytes, Cardiac / metabolism
  • Myocytes, Cardiac / pathology
  • Phenotype
  • Pluripotent Stem Cells / metabolism
  • Pluripotent Stem Cells / pathology
  • Ventricular Remodeling*