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Lung Fibroblasts from Idiopathic Pulmonary Fibrosis Patients Harbor Short and Unstable Telomeres Leading to Chromosomal Instability

Research output: Contribution to journal/Conference contribution in journal/Contribution to newspaper › Journal article › Research › peer-review

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Lung Fibroblasts from Idiopathic Pulmonary Fibrosis Patients Harbor Short and Unstable Telomeres Leading to Chromosomal Instability. / M’kacher, Radhia; Jaillet, Madeleine; Colicchio, Bruno et al.

In: Biomedicines, Vol. 10, No. 2, 310, 02.2022.

Research output: Contribution to journal/Conference contribution in journal/Contribution to newspaper › Journal article › Research › peer-review

Harvard

M’kacher, R, Jaillet, M, Colicchio, B, Vasarmidi, E, Mailleux, A, Dieterlen, A, Kannengiesser, C, Borie, C, Oudrhiri, N, Junker, S, Voisin, P, Jeandidier, E, Carde, P, Fenech, M, Bennaceur-Griscelli, A, Crestani, B & Borie, R 2022, 'Lung Fibroblasts from Idiopathic Pulmonary Fibrosis Patients Harbor Short and Unstable Telomeres Leading to Chromosomal Instability', Biomedicines, vol. 10, no. 2, 310. https://doi.org/10.3390/biomedicines10020310

APA

M’kacher, R., Jaillet, M., Colicchio, B., Vasarmidi, E., Mailleux, A., Dieterlen, A., Kannengiesser, C., Borie, C., Oudrhiri, N., Junker, S., Voisin, P., Jeandidier, E., Carde, P., Fenech, M., Bennaceur-Griscelli, A., Crestani, B., & Borie, R. (2022). Lung Fibroblasts from Idiopathic Pulmonary Fibrosis Patients Harbor Short and Unstable Telomeres Leading to Chromosomal Instability. Biomedicines, 10(2), [310]. https://doi.org/10.3390/biomedicines10020310

CBE

M’kacher R, Jaillet M, Colicchio B, Vasarmidi E, Mailleux A, Dieterlen A, Kannengiesser C, Borie C, Oudrhiri N, Junker S, et al. 2022. Lung Fibroblasts from Idiopathic Pulmonary Fibrosis Patients Harbor Short and Unstable Telomeres Leading to Chromosomal Instability. Biomedicines. 10(2):Article 310. https://doi.org/10.3390/biomedicines10020310

MLA

M’kacher, Radhia et al. "Lung Fibroblasts from Idiopathic Pulmonary Fibrosis Patients Harbor Short and Unstable Telomeres Leading to Chromosomal Instability". Biomedicines. 2022. 10(2). https://doi.org/10.3390/biomedicines10020310

Vancouver

M’kacher R, Jaillet M, Colicchio B, Vasarmidi E, Mailleux A, Dieterlen A et al. Lung Fibroblasts from Idiopathic Pulmonary Fibrosis Patients Harbor Short and Unstable Telomeres Leading to Chromosomal Instability. Biomedicines. 2022 Feb;10(2):310. doi: 10.3390/biomedicines10020310

Author

M’kacher, Radhia ; Jaillet, Madeleine ; Colicchio, Bruno et al. / Lung Fibroblasts from Idiopathic Pulmonary Fibrosis Patients Harbor Short and Unstable Telomeres Leading to Chromosomal Instability. In: Biomedicines. 2022 ; Vol. 10, No. 2.

Bibtex

@article{8c75fb994ad2453ba03321dc700518b2,
title = "Lung Fibroblasts from Idiopathic Pulmonary Fibrosis Patients Harbor Short and Unstable Telomeres Leading to Chromosomal Instability",
abstract = "Idiopathic 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.",
keywords = "Anaphase bridges, Dicentric chromosome, Idiopathic pulmonary fibrosis, Micronuclei, RTEL1, Telomere dysfunction, TERT",
author = "Radhia M{\textquoteright}kacher and Madeleine Jaillet and Bruno Colicchio and Eirini Vasarmidi and Arnaud Mailleux and Alain Dieterlen and Caroline Kannengiesser and Claire Borie and Noufissa Oudrhiri and Steffen Junker and Philippe Voisin and Eric Jeandidier and Patrice Carde and Michael Fenech and Annelise Bennaceur-Griscelli and Bruno Crestani and Raphael Borie",
note = "Publisher Copyright: {\textcopyright} 2022 by the authors. Licensee MDPI, Basel, Switzerland.",
year = "2022",
month = feb,
doi = "10.3390/biomedicines10020310",
language = "English",
volume = "10",
journal = "Biomedicines",
issn = "2227-9059",
publisher = "MDPI",
number = "2",

}

RIS

TY - JOUR

T1 - Lung Fibroblasts from Idiopathic Pulmonary Fibrosis Patients Harbor Short and Unstable Telomeres Leading to Chromosomal Instability

AU - M’kacher, Radhia

AU - Jaillet, Madeleine

AU - Colicchio, Bruno

AU - Vasarmidi, Eirini

AU - Mailleux, Arnaud

AU - Dieterlen, Alain

AU - Kannengiesser, Caroline

AU - Borie, Claire

AU - Oudrhiri, Noufissa

AU - Junker, Steffen

AU - Voisin, Philippe

AU - Jeandidier, Eric

AU - Carde, Patrice

AU - Fenech, Michael

AU - Bennaceur-Griscelli, Annelise

AU - Crestani, Bruno

AU - Borie, Raphael

N1 - Publisher Copyright: © 2022 by the authors. Licensee MDPI, Basel, Switzerland.

PY - 2022/2

Y1 - 2022/2

N2 - Idiopathic 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.

AB - Idiopathic 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.

KW - Anaphase bridges

KW - Dicentric chromosome

KW - Idiopathic pulmonary fibrosis

KW - Micronuclei

KW - RTEL1

KW - Telomere dysfunction

KW - TERT

UR - http://www.scopus.com/inward/record.url?scp=85124103543&partnerID=8YFLogxK

U2 - 10.3390/biomedicines10020310

DO - 10.3390/biomedicines10020310

M3 - Journal article

C2 - 35203522

AN - SCOPUS:85124103543

VL - 10

JO - Biomedicines

JF - Biomedicines

SN - 2227-9059

IS - 2

M1 - 310

ER -