Please use this identifier to cite or link to this item:
http://hdl.handle.net/2445/202706
Title: | Mitochondrial Dysfunction in Lung Resident Mesenchymal Stem Cells from Idiopathic Pulmonary Fibrosis Patients |
Author: | Mercader Barceló, Josep Martín Medina, Aina Truyols Vives, Joan Escarrer Garau, Gabriel Elowsson, Linda Montes Worboys, Ana Río Bocos, Carlos Muncunill Farreny, Josep Velasco Roca, Julio Cederberg, Anna Kadefors, Måns Molina Molina, Maria Westergren-Thorsson, Gunilla Sala Llinàs, Ernest |
Keywords: | Fibrosi pulmonar Mitocondris Pulmonary fibrosis Mitochondria |
Issue Date: | 17-Aug-2023 |
Publisher: | MDPI AG |
Abstract: | Idiopathic pulmonary fibrosis (IPF) is characterized by an aberrant repair response with uncontrolled turnover of extracellular matrix involving mesenchymal cell phenotypes, where lung resident mesenchymal stem cells (LRMSC) have been supposed to have an important role. However, the contribution of LRMSC in lung fibrosis is not fully understood, and the role of LRMSC in IPF remains to be elucidated. Here, we performed transcriptomic and functional analyses on LRMSC isolated from IPF and control patients (CON). Both over-representation and gene set enrichment analyses indicated that oxidative phosphorylation is the major dysregulated pathway in IPF LRMSC. The most relevant differences in biological processes included complement activation, mesenchyme development, and aerobic electron transport chain. Compared to CON LRMSC, IPF cells displayed impaired mitochondrial respiration, lower expression of genes involved in mitochondrial dynamics, and dysmorphic mitochondria. These changes were linked to an impaired autophagic response and a lower mRNA expression of pro-apoptotic genes. In addition, IPF TGF beta-exposed LRMSC presented different expression profiles of mitochondrial-related genes compared to CON TGF beta-treated cells, suggesting that TGF beta reinforces mitochondrial dysfunction. In conclusion, these results suggest that mitochondrial dysfunction is a major event in LRMSC and that their occurrence might limit LRMSC function, thereby contributing to IPF development. |
Note: | Reproducció del document publicat a: https://doi.org/10.3390/cells12162084 |
It is part of: | Cells, 2023, vol. 12, num. 16 |
URI: | http://hdl.handle.net/2445/202706 |
Related resource: | https://doi.org/10.3390/cells12162084 |
ISSN: | 2073-4409 |
Appears in Collections: | Articles publicats en revistes (Institut d'lnvestigació Biomèdica de Bellvitge (IDIBELL)) |
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cells-12-02084-v2.pdf | 3.89 MB | Adobe PDF | View/Open |
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