The Role of Telomerase and Telomeres in Interstitial Lung Diseases: From Molecules to Clinical Implications

Int J Mol Sci. 2019 Jun 19;20(12):2996. doi: 10.3390/ijms20122996.


Telomeres are distal chromosome regions associated with specific protein complexes that protect the chromosome against degradation and aberrations. Telomere maintenance capacity is an essential indication of healthy cell populations, and telomere damage is observed in processes such as malignant transformation, apoptosis, or cell senescence. At a cellular level, telomere damage may result from genotoxic stress, decreased activity of telomerase enzyme complex, dysfunction of shelterin proteins, or changes in expression of telomere-associated RNA such as TERRA. Clinical evidence suggests that mutation of telomerase genes (Tert/Terc) are associated with increased risk of congenital as well as age-related diseases (e.g., pneumonitis, idiopathic pulmonary fibrosis (IPF), dyskeratosis congenita, emphysema, nonspecific interstitial pneumonia, etc.). Thus, telomere length and maintenance can serve as an important prognostic factor as well as a potential target for new strategies of treatment for interstitial lung diseases (ILDs) and associated pulmonary pathologies.

Keywords: idiopathic pulmonary fibrosis; interstitial lung disease; long noncoding RNA; shelterin; telomerase; telomere.

Publication types

  • Review

MeSH terms

  • Animals
  • Cell Transformation, Neoplastic / genetics
  • Cell Transformation, Neoplastic / metabolism
  • Disease Susceptibility
  • Gene Expression Regulation
  • Genetic Association Studies
  • Humans
  • Lung Diseases, Interstitial / genetics*
  • Lung Diseases, Interstitial / metabolism*
  • Lung Diseases, Interstitial / pathology
  • Mutation
  • RNA, Long Noncoding
  • Telomerase / genetics*
  • Telomerase / metabolism*
  • Telomere / genetics*
  • Telomere / metabolism


  • RNA, Long Noncoding
  • TERT protein, human
  • Telomerase