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Review
. 2019 Aug 16;8(8):1232.
doi: 10.3390/jcm8081232.

Telomere Abnormalities in the Pathobiology of Idiopathic Pulmonary Fibrosis

Hasancan Bilgili  1 , Adam J Białas  2 , Paweł Górski  1 , Wojciech J Piotrowski  1
Affiliations
Review

Telomere Abnormalities in the Pathobiology of Idiopathic Pulmonary Fibrosis

Hasancan Bilgili et al. J Clin Med. .

Abstract

Idiopathic pulmonary fibrosis (IPF) occurs primarily in older adults and the incidence is clearly associated with aging. This disease seems to be associated with several hallmarks of aging, including telomere attrition and cellular senescence. Increasing evidence suggests that abnormalities involving telomeres and their proteome play a significant role in the pathobiology of IPF. The aim of this study is to summarize present knowledge in the field, as well as to discuss its possible clinical implications. Numerous mutations in genes associated with telomere functioning were studied in the context of IPF, mainly for Telomerase Reverse Transcriptase (TERT) and Telomerase RNA Component (TERC). Such mutations may lead to telomere shortening, which seems to increase the risk of IPF, negatively influence disease progression, and contribute to worse prognosis after lung transplantation. Some evidence indicates the possibility for the use of telomerase activators as potential therapeutic agents in pulmonary fibrosis. To sum up, increasing evidence suggests the role of telomere abnormalities in the pathobiology of IPF, natural history and prognosis of the disease. There are also possibilities for telomerase targeting in the potential development of new treatment agents. However, all these aspects require further research.

Keywords: idiopathic pulmonary fibrosis; lung fibrosis; telomerase; telomere; usual interstitial pneumonia.

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Conflict of interest statement

A.J.B. and W.J.P. have been founded by Roche and Boehringer-Ingelheim companies and received travel grants. W.J.P. also received compensation for lectures preparation. Other authors declare no competing interests.

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