International Journal of Molecular Sciences

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Special Issue Information

Dear Colleagues,

This Special Issue is a continuation of our previous successful Special Issue “Molecular and Cellular Mechanisms of Idiopathic Pulmonary Fibrosis and Interstitial Lung Diseases”.

Now, we are shedding light on the molecular and cellular mechanisms of lung fibrosis.

Lungs are exposed to many negative impacts with every breath we take, showing an extraordinary ability to repair damaged cells and tissues. A complex interplay between genetic background and the environment drives the response of lung tissues to acquired damage, and lung fibrosis is, in most cases, the final result of a maladaptive response to this complex interaction. Lung fibrosis causes a decline in pulmonary function, with patients suffering a worse quality of life and poor prognosis with early mortality.

Idiopathic pulmonary fibrosis (IPF) is a prototype of chronic, progressive interstitial lung disease (ILD), in which progressive lung scarring involves the lungs interstitium; although thought to be rare, its incidence has recently increased in the Western world and is similar to some types of cancer. At present, therapeutical options for treating IPF and other ILD are scarce and show little benefit.

This Special Issue is awaiting the submission of scientific research articles and reviews that describe the molecular and cellular mechanisms driving the progression of fibrosing lung diseases, with a particular focus on IPF, for a better understanding of their pathogenesis and the development of diagnostic markers, as well as the discovery of molecular targets suitable for tailored therapies.

Dr. Massimiliano Mancini
Guest Editor

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Related Special Issue

Published Papers (2 papers)

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Research

12 pages, 1487 KiB

Open AccessArticle

E-Selectin, ICAM-1, and ET-1 Biomarkers Address the Concern of the Challenging Diagnosis of Interstitial Lung Disease in Patients with Autoimmune Diseases

by Verónica Pulito-Cueto, Sara Remuzgo-Martínez, Fernanda Genre, Belén Atienza-Mateo, Víctor M. Mora-Cuesta, David Iturbe-Fernández, Leticia Lera-Gómez, María Sebastián Mora-Gil, Virginia Portilla, Alfonso Corrales, Ricardo Blanco, José M. Cifrián, Miguel A. González-Gay and Raquel López-Mejías

Int. J. Mol. Sci. 2023, 24(15), 12518; https://doi.org/10.3390/ijms241512518 - 07 Aug 2023

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Abstract

Interstitial lung disease (ILD) constitutes the most critical comorbidity in autoimmune diseases (ADs) and its early diagnosis remains a challenge for clinicians. Accordingly, we evaluated whether E-selectin, ICAM-1, and ET-1, key molecules in endothelial damage, could be useful biomarkers for the detection of AD-ILD⁺. We recruited patients with rheumatoid arthritis (RA)-ILD⁺ (n = 21) and systemic sclerosis (SSc)-ILD⁺ (n = 21). We included comparison groups of patients: RA-ILD⁻ (n = 25), SSc-ILD⁻ (n = 20), and idiopathic pulmonary fibrosis (IPF) (n = 21). Serum levels of these proteins were determined by ELISA. E-selectin, ICAM-1, and ET-1 serum levels were increased in RA-ILD⁺ and IPF patients in comparison to RA-ILD⁻ patients. Additionally, SSc-ILD⁺ and IPF patients exhibited higher ICAM-1 levels than those with SSc-ILD⁻. The ability of E-selectin, ICAM-1, and ET-1 to discriminate RA-ILD⁺ from RA-ILD⁻ patients, and ICAM-1 to distinguish SSc-ILD⁺ from SSc-ILD⁻ patients was confirmed using ROC curve analysis. Furthermore, elevated levels of ET-1 and E-selectin correlated with lung function decline in RA-ILD⁺ and SSc-ILD⁺ patients, respectively. In conclusion, our findings support the relevant role of E-selectin, ICAM-1, and ET-1 in RA-ILD⁺ patients as well as of ICAM-1 in SSc-ILD⁺ patients, constituting potential screening blood biomarkers of ILD in AD. Moreover, this study suggests ET-1 and E-selectin as possible indicators of worsening lung function in RA-ILD⁺ and SSc-ILD⁺ patients, respectively. Full article

(This article belongs to the Special Issue Molecular and Cellular Mechanisms of Idiopathic Pulmonary Fibrosis and Interstitial Lung Diseases 2.0)

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Graphical abstract

14 pages, 2700 KiB

Open AccessArticle

The Role of Erythrocyte Membrane Protein Band 4.1-like 3 in Idiopathic Pulmonary Fibrosis

by Min Kyung Kim, Jong-Uk Lee, Sun Ju Lee, Hun Soo Chang, Jong-Sook Park and Choon-Sik Park

Int. J. Mol. Sci. 2023, 24(12), 10182; https://doi.org/10.3390/ijms241210182 - 15 Jun 2023

Viewed by 1303

Abstract

Novel genetic and epigenetic factors involved in the development and prognosis of idiopathic pulmonary fibrosis (IPF) have been identified. We previously observed that erythrocyte membrane protein band 4.1-like 3 (EPB41L3) increased in the lung fibroblasts of IPF patients. Thus, we investigated the role of EPB41L3 in IPF by comparing the EPB41L3 mRNA and protein expression of lung fibroblast between patients with IPF and controls. We also investigated the regulation of epithelial–mesenchymal transition (EMT) in an epithelial cell line (A549) and fibroblast-to-myofibroblast transition (FMT) in a fibroblast cell line (MRC5) by overexpressing and silencing EPB41L3. EPB41L3 mRNA and protein levels, as measured using RT-PCR, real-time PCR, and Western blot, were significantly higher in fibroblasts derived from 14 IPF patients than in those from 10 controls. The mRNA and protein expression of EPB41L3 was upregulated during transforming growth factor-β-induced EMT and FMT. Overexpression of EPB41L3 in A549 cells using lenti-EPB41L3 transfection suppressed the mRNA and protein expression of N-cadherin and COL1A1. Treatment with EPB41L3 siRNA upregulated the mRNA and protein expression of N-cadherin. Overexpression of EPB41L3 in MRC5 cells using lenti-EPB41L3 transfection suppressed the mRNA and protein expression of fibronectin and α-SMA. Finally, treatment with EPB41L3 siRNA upregulated the mRNA and protein expression of FN1, COL1A1, and VIM. In conclusion, these data strongly support an inhibitory effect of EPB41L3 on the process of fibrosis and suggest the therapeutic potential of EPB41L3 as an anti-fibrotic mediator. Full article

(This article belongs to the Special Issue Molecular and Cellular Mechanisms of Idiopathic Pulmonary Fibrosis and Interstitial Lung Diseases 2.0)

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