Epigenetic Regulation of Pulmonary Arterial Hypertension-Induced Vascular and Right Ventricular Remodeling: New Opportunities?

Jordy M. M. Kocken, Paula A. da Costa Martins*

*Corresponding author for this work

Research output: Contribution to journal(Systematic) Review article peer-review

Abstract

Pulmonary artery hypertension (PAH) is a rare chronic disease with high impact on patients' quality of life and currently no available cure. PAH is characterized by constant remodeling of the pulmonary artery by increased proliferation and migration of pulmonary arterial smooth muscle cells (PASMCs), fibroblasts (FBs) and endothelial cells (ECs). This remodeling eventually leads to increased pressure in the right ventricle (RV) and subsequent right ventricle hypertrophy (RVH) which, when left untreated, progresses into right ventricle failure (RVF). PAH can not only originate from heritable mutations, but also develop as a consequence of congenital heart disease, exposure to drugs or toxins, HIV, connective tissue disease or be idiopathic. While much attention was drawn into investigating and developing therapies related to the most well understood signaling pathways in PAH, in the last decade, a shift towards understanding the epigenetic mechanisms driving the disease occurred. In this review, we reflect on the different epigenetic regulatory factors that are associated with the pathology of RV remodeling, and on their relevance towards a better understanding of the disease and subsequently, the development of new and more efficient therapeutic strategies.

Original languageEnglish
Article number8901
Number of pages26
JournalInternational journal of molecular sciences
Volume21
Issue number23
DOIs
Publication statusPublished - Dec 2020

Keywords

  • epigenetics
  • hypertrophy
  • non-coding RNAs
  • pulmonary arterial hypertension
  • right ventricle remodeling
  • CIRCULAR RNA
  • DNA METHYLATION
  • NITRIC-OXIDE SYNTHASE
  • HISTONE H3 PHOSPHORYLATION
  • LONG NONCODING RNA
  • MESSENGER-RNA
  • MITOCHONDRIAL DYSFUNCTION
  • SMOOTH-MUSCLE-CELLS
  • CHROMATIN MODIFICATION
  • MOLECULAR-MECHANISMS

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