Idiopathic Pulmonary Fibrosis: Pathogenesis and the Emerging Role of Long Non-Coding RNAs

Int J Mol Sci. 2020 Jan 14;21(2):524. doi: 10.3390/ijms21020524.

Abstract

Idiopathic pulmonary fibrosis (IPF) is a progressive chronic disease characterized by excessing scarring of the lungs leading to irreversible decline in lung function. The aetiology and pathogenesis of the disease are still unclear, although lung fibroblast and epithelial cell activation, as well as the secretion of fibrotic and inflammatory mediators, have been strongly associated with the development and progression of IPF. Significantly, long non-coding RNAs (lncRNAs) are emerging as modulators of multiple biological processes, although their function and mechanism of action in IPF is poorly understood. LncRNAs have been shown to be important regulators of several diseases and their aberrant expression has been linked to the pathophysiology of fibrosis including IPF. This review will provide an overview of this emerging role of lncRNAs in the development of IPF.

Keywords: IPF; fibrosis; lncRNAs; long non-coding RNAs; lung.

Publication types

  • Review

MeSH terms

  • Alveolar Epithelial Cells / metabolism
  • Alveolar Epithelial Cells / pathology
  • Animals
  • Biomarkers
  • Chromatin / genetics
  • Chromatin / metabolism
  • Chromatin Assembly and Disassembly
  • Disease Susceptibility*
  • Extracellular Matrix / metabolism
  • Gene Expression Regulation
  • Genetic Predisposition to Disease
  • Humans
  • Idiopathic Pulmonary Fibrosis / diagnosis
  • Idiopathic Pulmonary Fibrosis / drug therapy
  • Idiopathic Pulmonary Fibrosis / etiology*
  • Idiopathic Pulmonary Fibrosis / metabolism*
  • Pneumonia / complications
  • Pneumonia / etiology
  • Pneumonia / metabolism
  • Pneumonia / pathology
  • RNA Processing, Post-Transcriptional
  • RNA, Long Noncoding / genetics*
  • Respiratory Mucosa / metabolism
  • Respiratory Mucosa / pathology
  • Symptom Assessment
  • Transcription, Genetic

Substances

  • Biomarkers
  • Chromatin
  • RNA, Long Noncoding