2026-05-22T08:52:43Zhttps://repisalud.isciii.es/rest/oai/request

oai:repisalud.isciii.es:20.500.12105/237172024-11-28T18:33:37Zcom_20.500.12105_15322com_20.500.12105_2051col_20.500.12105_16962col_20.500.12105_16967

00925njm 22002777a 4500 dc Mercader-Barceló, Josep author Martín-Medina, Aina author Truyols-Vives, Joan author Escarrer-Garau, Gabriel author Elowsson, Linda author Montes-Worboys, Ana author Rio-Bocos, Carlos author Muncunill Farreny, Josep author Velasco, Julio author Cederberg, Anna author Kadefors, Måns author Molina-Molina, Maria author Westergren-Thorsson, Gunilla author Sala Llinàs, Ernest author 2023-08-17 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 TGFβ-exposed LRMSC presented different expression profiles of mitochondrial-related genes compared to CON TGFβ-treated cells, suggesting that TGFβ 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. Mercader-Barceló J, Martín-Medina A, Truyols-Vives J, Escarrer-Garau G, Elowsson L, Montes-Worboys A, et al. Mitochondrial Dysfunction in Lung Resident Mesenchymal Stem Cells from Idiopathic Pulmonary Fibrosis Patients. Cells. 2023 Aug 17;12(16):2084. 10.3390/cells12162084 2073-4409 Cells https://hdl.handle.net/20.500.13003/19946 37626894 L2025065790 2-s2.0-85169152321 https://hdl.handle.net/20.500.12105/23717 1056771600001 Mitochondrial Dysfunction in Lung Resident Mesenchymal Stem Cells from Idiopathic Pulmonary Fibrosis Patients