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Lung fibroblasts from idiopathic pulmonary fibrosis patients harbor short and unstable telomeres leading to chromosomal instability

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dc.contributor.authorM'kacher, R.
dc.contributor.authorJaillet, M.
dc.contributor.authorColicchio, B.
dc.contributor.authorVasarmidi, E.
dc.contributor.authorMailleux, A.
dc.contributor.authorDieterlen, A.
dc.contributor.authorKannengiesser, C.
dc.contributor.authorBorie, C.
dc.contributor.authorOudrhiri, N.
dc.contributor.authorJunker, S.
dc.contributor.authorVoisin, P.
dc.contributor.authorJeandidier, E.
dc.contributor.authorCarde, P.
dc.contributor.authorFenech, M.
dc.contributor.authorBennaceur Griscelli, A.
dc.contributor.authorCrestani, B.
dc.contributor.authorBorie, R.
dc.date.issued2022
dc.description.abstractIdiopathic pulmonary fibrosis (IPF) is associated with several hallmarks of aging including telomere shortening, which can result from germline mutations in telomere related genes (TRGs). Here, we assessed the length and stability of telomeres as well as the integrity of chromosomes in primary lung fibroblasts from 13 IPF patients (including seven patients with pathogenic variants in TRGs) and seven controls. Automatized high-throughput detection of telomeric FISH signals highlighted lower signal intensity in lung fibroblasts from IPF patients, suggesting a telomere length defect in these cells. The increased detection of telomere loss and terminal deletion in IPF cells, particularly in TRG-mutated cells (IPF-TRG), supports the notion that these cells have unstable telomeres. Furthermore, fibroblasts from IPF patients with TRGs mutations exhibited dicentric chromosomes and anaphase bridges. Collectively, our study indicates that fibroblasts from IPF patients exhibit telomere and chromosome instability that likely contribute to the physiopathology.
dc.identifier.citationBiomedicines, 2022; 10(2, article no. 310):1-13
dc.identifier.doi10.3390/biomedicines10020310
dc.identifier.issn2227-9059
dc.identifier.issn2227-9059
dc.identifier.orcidFenech, M. [0000-0002-8466-0991]
dc.identifier.urihttps://hdl.handle.net/11541.2/28790
dc.language.isoen
dc.publisherMDPIAG
dc.rightsCopyright 2022 The author(s). This is an open access article distributed under the Creative Commons Attribution License which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited (https://creativecommons.org/licenses/by/4.0/)
dc.source.urihttps://doi.org/10.3390/biomedicines10020310
dc.subjectidiopathic pulmonary fibrosis
dc.subjecttelomere dysfunction
dc.subjectdicentric chromosome
dc.subjectanaphase bridges
dc.subjectmicronuclei
dc.subjectTERT
dc.subjectRTEL1
dc.titleLung fibroblasts from idiopathic pulmonary fibrosis patients harbor short and unstable telomeres leading to chromosomal instability
dc.typeJournal article
pubs.publication-statusPublished
ror.fileinfo12245279900001831 13245279890001831 M'Kacher et al Biomedicines-1563799
ror.mmsid9916608951201831

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