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Molecular Pathology of Idiopathic Pulmonary Fibrosis 2.0
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Molecular Pathology of Idiopathic Pulmonary Fibrosis 2.0

A special issue of International Journal of Molecular Sciences (ISSN 1422-0067). This special issue belongs to the section "Molecular Pathology, Diagnostics, and Therapeutics".

Deadline for manuscript submissions: closed (15 April 2023) | Viewed by 11506

Special Issue Editors

Prof. Dr. Elena Bargagli

E-Mail Website
Guest Editor
Respiratory Diseases and Lung Transplant Unit, Department of Medical and Surgical Sciences and Neurosciences, University of Siena, 53100 Siena, Italy
Interests: interstitial lung diseases; biomarkers; bronchoalveolar lavage; pathogenesis
Special Issues, Collections and Topics in MDPI journals
Dr. Paolo Cameli

E-Mail Website
Guest Editor
Respiratory Diseases and Lung Transplantation Unit, Department of Medicine, Surgery and Neurosciences, University Hospital of Siena, 53100 Siena, Italy
Interests: interstitial lung disease; KL-6; lung transplant; vasculitis; myositis
Special Issues, Collections and Topics in MDPI journals

Special Issue Information

Dear Colleagues,

This is a novel era in idiopathic pulmonary fibrosis research. In the last decade, we have experienced great advances of our knowledge and comprehension on the pathogenetic pathways involved in fibrotic lung diseases.

These novel findings have led to the approval of specific antifibrotic drugs that are currently the milestones of clinical management of these patients.

However, many questions remain unsolved: We still need to identify reliable biomarkers that could help in differential diagnosis, prognosis, and in patient stratification, as well as in the definition of the response to treatment. Other unmet needs include the definition of the etiology and risk factors for idiopathic pulmonary fibrosis development, as well as genetic definition of susceptibility. OMIC sciences are contributing greatly to this field, and this Special Issue aims to provide some answers to the unresolved questions on the Molecular Pathology of Idiopathic Pulmonary Fibrosis.

Prof. Dr. Elena Bargagli
Dr. Paolo Cameli
Guest Editors

Manuscript Submission Information

Manuscripts should be submitted online at www.mdpi.com by registering and logging in to this website. Once you are registered, click here to go to the submission form. Manuscripts can be submitted until the deadline. All submissions that pass pre-check are peer-reviewed. Accepted papers will be published continuously in the journal (as soon as accepted) and will be listed together on the special issue website. Research articles, review articles as well as short communications are invited. For planned papers, a title and short abstract (about 100 words) can be sent to the Editorial Office for announcement on this website.

Submitted manuscripts should not have been published previously, nor be under consideration for publication elsewhere (except conference proceedings papers). All manuscripts are thoroughly refereed through a single-blind peer-review process. A guide for authors and other relevant information for submission of manuscripts is available on the Instructions for Authors page. International Journal of Molecular Sciences is an international peer-reviewed open access semimonthly journal published by MDPI.

Please visit the Instructions for Authors page before submitting a manuscript. There is an Article Processing Charge (APC) for publication in this open access journal. For details about the APC please see here. Submitted papers should be well formatted and use good English. Authors may use MDPI's English editing service prior to publication or during author revisions.

Keywords

  • idiopathic pulmonary fibrosis
  • pathology
  • molecular biomarkers
  • pathogenesis
  • interstitial lung diseases
  • omics
  • prognosis
  • pathogenesis
  • proteomics
  • genetic

Published Papers (4 papers)

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Research

15 pages, 3432 KiB  
Article
Theophylline Attenuates BLM-Induced Pulmonary Fibrosis by Inhibiting Th17 Differentiation
by Soo-Jin Park, Hwa-Jeong Hahn, Sei-Ryang Oh and Hyun-Jun Lee
Int. J. Mol. Sci. 2023, 24(2), 1019; https://doi.org/10.3390/ijms24021019 - 5 Jan 2023
Cited by 9 | Viewed by 2141
Abstract
Idiopathic pulmonary fibrosis (IPF) is a chronic and refractory interstitial lung disease. Although there are two approved drugs for IPF, they were not able to completely cure the disease. Therefore, the development of new drugs is required for the effective treatment of IPF. [...] Read more.
Idiopathic pulmonary fibrosis (IPF) is a chronic and refractory interstitial lung disease. Although there are two approved drugs for IPF, they were not able to completely cure the disease. Therefore, the development of new drugs is required for the effective treatment of IPF. In this study, we investigated the effect of theophylline, which has long been used for the treatment of asthma, on pulmonary fibrosis. The administration of theophylline attenuated the fibrotic changes of lung tissues and improved mechanical pulmonary functions in bleomycin (BLM)-induced pulmonary fibrosis. Theophylline treatment suppressed IL-17 production through inhibiting cytokines controlling Th17 differentiation; TGF-β, IL-6, IL-1β, and IL-23. The inhibition of IL-6 and IL-1β by theophylline is mediated by suppressing BLM-induced ROS production and NF-κB activation in epithelial cells. We further demonstrated that theophylline inhibited TGF-β-induced epithelial-to-mesenchymal transition in epithelial cells through suppressing the phosphorylation of Smad2/3 and AKT. The inhibitory effects of theophylline on the phosphorylation of Smad2/3 and AKT were recapitulated in BLM-treated lung tissues. Taken together, these results demonstrated that theophylline prevents pulmonary fibrosis by inhibiting Th17 differentiation and TGF-β signaling. Full article
(This article belongs to the Special Issue Molecular Pathology of Idiopathic Pulmonary Fibrosis 2.0)
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13 pages, 2865 KiB  
Article
Loss of SP-A in the Lung Exacerbates Pulmonary Fibrosis
by Kyunghwa Kim, Dasom Shin, Gaheon Lee and Hyunsu Bae
Int. J. Mol. Sci. 2022, 23(10), 5292; https://doi.org/10.3390/ijms23105292 - 10 May 2022
Cited by 7 | Viewed by 2271
Abstract
Idiopathic pulmonary fibrosis (IPF) is a devastating and common chronic lung disease that is pathologically characterized by the destruction of lung architecture and the accumulation of extracellular matrix in the lung. Previous studies have shown an association between lung surfactant protein (SP) and [...] Read more.
Idiopathic pulmonary fibrosis (IPF) is a devastating and common chronic lung disease that is pathologically characterized by the destruction of lung architecture and the accumulation of extracellular matrix in the lung. Previous studies have shown an association between lung surfactant protein (SP) and the pathogenesis of IPF, as demonstrated by mutations and the altered expression of SP in patients with IPF. However, the role of SP in the development of lung fibrosis is poorly understood. In this study, the role of surfactant protein A (SP-A) was explored in experimental lung fibrosis induced with a low or high dose of bleomycin (BLM) and CRISPR/Cas9-mediated genetic deletion of SP-A. Our results showed that lung SP-A deficiency in mice promoted the development of fibrotic damage and exacerbated inflammatory responses to the BLM challenge. In vitro experiments with murine lung epithelial LA-4 cells demonstrated that in response to transforming growth factor-β1 (TGF-β1), LA-4 cells had a decreased protein expression of SP-A. Furthermore, exogenous SP administration to LA-4 cells inhibited the TGF-β1-induced upregulation of fibrotic markers. Overall, these findings suggest a novel antifibrotic mechanism of SP-A in the development of lung fibrosis, which indicates the therapeutic potential of the lung SP-A in preventing the development of IPF. Full article
(This article belongs to the Special Issue Molecular Pathology of Idiopathic Pulmonary Fibrosis 2.0)
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19 pages, 7286 KiB  
Article
RNA Sequencing of Epithelial Cell/Fibroblastic Foci Sandwich in Idiopathic Pulmonary Fibrosis: New Insights on the Signaling Pathway
by Fiorella Calabrese, Francesca Lunardi, Veronica Tauro, Federica Pezzuto, Francesco Fortarezza, Luca Vedovelli, Eleonora Faccioli, Elisabetta Balestro, Marco Schiavon, Giovanni Esposito, Stefania Edith Vuljan, Chiara Giraudo, Dario Gregori, Federico Rea and Paolo Spagnolo
Int. J. Mol. Sci. 2022, 23(6), 3323; https://doi.org/10.3390/ijms23063323 - 19 Mar 2022
Cited by 11 | Viewed by 2761
Abstract
Idiopathic pulmonary fibrosis (IPF) is a progressive and fatal lung disease characterized by irreversible scarring of the distal lung. IPF is best described by its histopathological pattern of usual interstitial pneumonia (UIP), characterized by spatial heterogeneity with alternating interstitial fibrosis and areas of [...] Read more.
Idiopathic pulmonary fibrosis (IPF) is a progressive and fatal lung disease characterized by irreversible scarring of the distal lung. IPF is best described by its histopathological pattern of usual interstitial pneumonia (UIP), characterized by spatial heterogeneity with alternating interstitial fibrosis and areas of normal lung, and temporal heterogeneity of fibrosis characterized by scattered fibroblastic foci (FF), dense acellular collagen and honeycomb changes. FF, comprising aggregated fibroblasts/myofibroblasts surrounded by metaplastic epithelial cells (EC), are the cardinal pathological lesion and their presence strongly correlates with disease progression and mortality. We hypothesized that the EC/FF sandwich from patients with UIP/IPF has a distinct molecular signature which could offer new insights into the crosstalk of these two crucial actors in the disease. Laser capture microdissection with RNAseq was used to investigate the transcriptome of the EC/FF sandwich from IPF patients versus controls (primary spontaneous pneumothorax). Differentially expressed gene analysis identified 23 up-regulated genes mainly related to epithelial dysfunction. Gene ontology analysis highlighted the activation of different pathways, mainly related to EC, immune response and programmed cell death. This study provides novel insights into the IPF pathogenetic pathways and suggests that targeting some of these up-regulated pathways (particularly those related to secreto-protein/mucin dysfunction) may be beneficial in IPF. Further studies in a larger number of lung samples, ideally from patients with early and advanced disease, are needed to validate these findings. Full article
(This article belongs to the Special Issue Molecular Pathology of Idiopathic Pulmonary Fibrosis 2.0)
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17 pages, 5371 KiB  
Article
Transient Agarose Spot (TAS) Assay: A New Method to Investigate Cell Migration
by Apor Veres-Székely, Domonkos Pap, Beáta Szebeni, László Őrfi, Csenge Szász, Csenge Pajtók, Eszter Lévai, Attila J. Szabó and Ádám Vannay
Int. J. Mol. Sci. 2022, 23(4), 2119; https://doi.org/10.3390/ijms23042119 - 14 Feb 2022
Cited by 4 | Viewed by 3150
Abstract
Fibroblasts play a central role in diseases associated with excessive deposition of extracellular matrix (ECM), including idiopathic pulmonary fibrosis. Investigation of different properties of fibroblasts, such as migration, proliferation, and collagen-rich ECM production is unavoidable both in basic research and in the development [...] Read more.
Fibroblasts play a central role in diseases associated with excessive deposition of extracellular matrix (ECM), including idiopathic pulmonary fibrosis. Investigation of different properties of fibroblasts, such as migration, proliferation, and collagen-rich ECM production is unavoidable both in basic research and in the development of antifibrotic drugs. In the present study we developed a cost-effective, 96-well plate-based method to examine the migration of fibroblasts, as an alternative approach to the gold standard scratch assay, which has numerous limitations. This article presents a detailed description of our transient agarose spot (TAS) assay, with instructions for its routine application. Advantages of combined use of different functional assays for fibroblast activation in drug development are also discussed by examining the effect of nintedanib—an FDA approved drug against IPF—on lung fibroblasts. Full article
(This article belongs to the Special Issue Molecular Pathology of Idiopathic Pulmonary Fibrosis 2.0)
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