Miniatura

Fitxers

cells-12-02084-v2.pdf (3.8 MB)

Tipus de document

Article

Versió

Versió publicada

Data de publicació

2023-08-17

Llicència de publicació

cc by (c) Mercader Barceló, Josep et al., 2023
Si us plau utilitzeu sempre aquest identificador per citar o enllaçar aquest document: https://hdl.handle.net/2445/202706

Mitochondrial Dysfunction in Lung Resident Mesenchymal Stem Cells from Idiopathic Pulmonary Fibrosis Patients

Títol de la revista

Autors

Director/Tutor

ISSN de la revista

Títol del volum

Recurs relacionat

https://doi.org/10.3390/cells12162084

Resum

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.

Matèries

Fibrosi pulmonar, Mitocondris

Matèries (anglès)

Pulmonary fibrosis, Mitochondria

Citació

Col·leccions

Articles publicats en revistes (Institut d'lnvestigació Biomèdica de Bellvitge (IDIBELL))

Citació

MERCADER BARCELÓ, Josep, MARTÍN MEDINA, Aina, TRUYOLS VIVES, Joan, ESCARRER GARAU, Gabriel, ELOWSSON RENDIN, Linda, MONTES WORBOYS, Ana, RÍO BOCOS, Carlos, MUNCUNILL FARRENY, Josep, VELASCO ROCA, Julio, CEDERBERG, Anna, KADEFORS, Måns, MOLINA MOLINA, María, WESTERGREN-THORSSON, Gunilla, SALA LLINÀS, Ernest. Mitochondrial Dysfunction in Lung Resident Mesenchymal Stem Cells from Idiopathic Pulmonary Fibrosis Patients. _Cells_. 2023. Vol. 12, núm. 16. [consulta: 20 de gener de 2026]. ISSN: 2073-4409. [Disponible a: https://hdl.handle.net/2445/202706]

Exportar metadades

JSON - METS

Compartir registre