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Telomere Abnormalities in the Pathobiology of Idiopathic Pulmonary Fibrosis.


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

SUBMITTER: Bilgili H 

PROVIDER: S-EPMC6723768 | biostudies-literature | 2019 Aug

REPOSITORIES: biostudies-literature