Epigenetic reprogramming to prevent genetic cardiomyopathy

J Clin Invest. 2021 Jan 4;131(1):e143684. doi: 10.1172/JCI143684.

Abstract

Mutations in the gene that codes for lamin A/C (LMNA) are a common cause of adult-onset cardiomyopathy and heart failure. In this issue of the JCI, Guénantin and Jebeniani et al. identify impaired cardiomyocyte development and maturation as a prenatal feature in a model of laminopathy. Cardiomyocytes carrying the Lmna point mutation H222P misexpressed genes involved in the epithelial-mesenchymal transition and showed decreased methylation at the fourth lysine of histone H3 (H3K4). Notably, inhibiting lysine-specific demethylase 1 in the LMNA H222P mouse model treated this congenital form of cardiomyopathy and improved survival in utero. These data highlight early epigenomic modifications in lamin A/C-mediated pathology and indicate a unique therapeutic strategy for cardiomyopathy.

Publication types

  • Research Support, N.I.H., Extramural
  • Comment

MeSH terms

  • Animals
  • Cardiomyopathies* / genetics
  • Epigenesis, Genetic
  • Epigenomics*
  • Lamin Type A / genetics
  • Lamin Type A / metabolism
  • Mice
  • Mutation

Substances

  • Lamin Type A