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Review
. 2019 Jun 19;20(12):2996.
doi: 10.3390/ijms20122996.

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

Nissim Arish  1 , Dmytro Petukhov  2 , Shulamit B Wallach-Dayan  3
Affiliations
Review

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

Nissim Arish et al. Int J Mol Sci. .

Abstract

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.

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Conflict of interest statement

The authors declare no conflict of interest.

Figures

Figure 1
Figure 1
Principal factors involved in the regulation of telomere maintenance in the fibrotic lung. External or internal factors (such as mutations and oxidative stress, indicated by dashed blue ovals) may positively (pointed arrow) or negatively (block arrow) affect the telomere maintenance machinery through telomerase complex (green pentagon) or TERRA and shelterin complexes (blue pentagons), leading to structural and functional damage of telomeric and chromosomal DNA (solid blue ovals). The results of these disruptions depend on the affected cell type and vary from apoptosis in epithelial cells to cellular proliferation in fibroblasts (dashed violet ovals), thus causing imbalances in cell populations of the organ in question. In lungs, in particular, this may produce a proliferation of fibrotic tissue that manifests itself as an ILD, such as IPF or familial pulmonary fibrosis (red squares).
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References

    1. Prasad K.N., Wu M., Bondy S.C. Telomere shortening during aging: Attenuation by antioxidants and anti-inflammatory agents. Mech. Ageing Dev. 2017;164:61–66. doi: 10.1016/j.mad.2017.04.004. - DOI - PubMed
    1. Bettin N., Oss Pegorar C., Cusanelli E. The Emerging Roles of TERRA in Telomere Maintenance and Genome Stability. Cells. 2019;8:246. doi: 10.3390/cells8030246. - DOI - PMC - PubMed
    1. Cusanelli E., Chartrand P. Telomeric noncoding RNA: Telomeric repeat-containing RNA in telomere biology. Wiley Interdiscip. Rev. RNA. 2014;5:407–419. doi: 10.1002/wrna.1220. - DOI - PubMed
    1. Snetselaar R., van Moorsel C.H.M., Kazemier K.M., van der Vis J.J., Zanen P., van Oosterhout M.F.M., Grutters J.C. Telomere length in interstitial lung diseases. Chest. 2015;148:1011–1018. doi: 10.1378/chest.14-3078. - DOI - PubMed
    1. Raghu G., Remy-Jardin M., Myers J.L., Richeldi L., Ryerson C.J., Lederer D.J., Behr J., Cottin V., Danoff S.K., Morell F., et al. Diagnosis of Idiopathic Pulmonary Fibrosis. An Official ATS/ERS/JRS/ALAT Clinical Practice Guideline. Am. J. Respir. Crit. Care Med. 2018;198:e44–e68. doi: 10.1164/rccm.201807-1255ST. - DOI - PubMed