Please use this identifier to cite or link to this item:
Title: Anti-fibrotic Effects Of Pirfenidone And Rapamycin In Primary Ipf Fibroblasts And Human Alveolar Epithelial Cells
Author: Molina Molina, María
Machahua, Carlos
Vicens Zygmunt, Vanesa
Llatjós, Roger
Escobar, I.
Sala Llinas, Ernest
Luburich Hernaiz, Patricio
Dorca i Sargatal, Jordi
Montes Worboys, Ana
Keywords: Fibrosi pulmonar
Matriu extracel·lular
Pulmonary fibrosis
Extracellular matrix
Issue Date: 27-Apr-2018
Publisher: Biomed Central Ltd
Abstract: Background: Pirfenidone, a pleiotropic anti-fibrotic treatment, has been shown to slow down disease progression of idiopathic pulmonary fibrosis (IPF), a fatal and devastating lung disease. Rapamycin, an inhibitor of fibroblast proliferation could be a potential anti-fibrotic drug to improve the effects of pirfenidone. Methods: Primary lung fibroblasts from IPF patients and human alveolar epithelial cells (A549) were treated in vitro with pirfenidone and rapamycin in the presence or absence of transforming growth factor beta 1 (TGF-beta). Extracellular matrix protein and gene expression of markers involved in lung fibrosis (tenascin-c, fibronectin, collagen I (COM Al], collagen III [COL3A1] and alpha-smooth muscle actin [alpha-SMA]) were analyzed. A cell migration assay in pirfenidone, rapamycin and TGF-beta-containing media was performed. Results: Gene and protein expression of tenascin-c and fibronectin of fibrotic fibroblasts were reduced by pirfenidone or rapamycin treatment Pirfenidone-rapamycin treatment did not revert the epithelial to mesenchymal transition pathway activated by TGF-beta. However, the drug combination significantly abrogated fibroblast to myofibroblast transition. The inhibitory effect of pirfenidone on fibroblast migration in the scratch-wound assay was potentiated by rapamycin combination. Conclusions: These findings indicate that the combination of pirfenidone and rapamycin widen the inhibition range of fibrogenic markers and prevents fibroblast migration. These results would open a new line of research for an anti-fibrotic combination therapeutic approach.
Note: Reproducció del document publicat a:
It is part of: Bmc Pulmonary Medicine, 2018, Vol. 18:63
Related resource:
Appears in Collections:Articles publicats en revistes (Institut d'lnvestigació Biomèdica de Bellvitge (IDIBELL))

Files in This Item:
File Description SizeFormat 
Molina-MolinaM.pdf1.86 MBAdobe PDFView/Open

This item is licensed under a Creative Commons License Creative Commons