MicroRNA regulatory networks in idiopathic pulmonary fibrosis

Biochem Cell Biol. 2015 Apr;93(2):129-37. doi: 10.1139/bcb-2014-0101. Epub 2015 Jan 5.


Idiopathic pulmonary fibrosis (IPF) is a chronic, progressive, and fatal scarring lung disease of unknown etiology, characterized by changes in microRNA expression. Activation of transforming growth factor (TGF-β) is a key event in the development of IPF. Recent reports have also identified epigenetic modification as an important player in the pathogenesis of IPF. In this review, we summarize the main results of studies that address the role of microRNAs in IPF and highlight the synergistic actions of these microRNAs in regulating TGF-β, the primary fibrogenic mediator. We outline epigenetic regulation of microRNAs by methylation. Functional studies identify microRNAs that alter proliferative and migratory properties of fibroblasts, and induce phenotypic changes in epithelial cells consistent with epithelial-mesenchymal transition. Though these studies were performed in isolation, we identify multiple co-operative actions after assembling the results into a network. Construction of such networks will help identify disease-propelling hubs that can be targeted for therapeutic purposes.

Keywords: FPI; IPF; TFG-β; TGF-β; epithelial-mesenchymal transition; fibroblastes; fibroblasts; micro-ARN; microRNA; transition épithélium-mésenchyme.

Publication types

  • Review

MeSH terms

  • Animals
  • Cells, Cultured
  • DNA Methylation
  • Epigenesis, Genetic
  • Epithelial Cells / metabolism*
  • Epithelial-Mesenchymal Transition
  • Fibroblasts / metabolism*
  • Gene Expression Regulation
  • Humans
  • Idiopathic Pulmonary Fibrosis / genetics*
  • Mice
  • MicroRNAs / genetics*
  • Transforming Growth Factor beta / metabolism*


  • MicroRNAs
  • Transforming Growth Factor beta