Advances in Pulmonary Fibrosis

A special issue of Cells (ISSN 2073-4409). This special issue belongs to the section "Cellular Pathology".

Deadline for manuscript submissions: 1 December 2025 | Viewed by 1087

Special Issue Editor


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Guest Editor
Ramón y Cajal Research Fellow, Head of the Telomeres and Tumor Microenvironment Unit, Fundación Rioja Salud, Piqueras 98, 26006, Logroño, Spain
Interests: lung

Special Issue Information

Dear Colleagues, 

Idiopathic pulmonary fibrosis (IPF) is a fibrosing interstitial lung disease characterized by a progressive decline in exercise capacity, difficulty breathing, recurrent infections, and a severe impairment in lung function, which makes the patients dependent on long-term oxygen treatment. Due to the lack of effective treatments, the median survival time of patients from diagnosis is 2–4 years. Currently, lung transplantation is the only therapeutic option and is carried out in fewer than 5% of IPF patients with a very severe stage of disease. Thus, there is an urgent need to identify novel mechanisms implicated in the onset and development of this disease, as well as potential therapies. 

Potential topics for inclusion in this Special Issue include the following:

  • Novel biomarkers;
  • Experimental therapeutics;
  • Animal models (cellular and molecular pathophysiology and experimental therapies);
  • The identification of novel mechanisms in IPF.

Dr. Sergio Piñeiro Hermida
Guest Editor

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Keywords

  • pulmonary fibrosis
  • animal models
  • biomarkers
  • experimental therapeutics

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Published Papers (1 paper)

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Research

18 pages, 3381 KB  
Article
EPDR1 Links Fibroblast Dysfunction to Disease Severity in Idiopathic Pulmonary Fibrosis
by Jong-Uk Lee, Seung-Lee Park, Min Kyung Kim, Eunjeong Seo, Hun-Gyu Hwang, Jung Hyun Kim, Hun Soo Chang and Choon-Sik Park
Cells 2025, 14(19), 1515; https://doi.org/10.3390/cells14191515 - 28 Sep 2025
Viewed by 784
Abstract
Idiopathic pulmonary fibrosis (IPF) is a progressive lung disease characterized by aberrant fibroblast activation, lysosomal dysfunction, and cellular senescence. Transcriptomic analyses have identified ependymin-related 1 (EPDR1) as a fibroblast-enriched gene in IPF, but its biological function remains unclear. EPDR1 expression was assessed in [...] Read more.
Idiopathic pulmonary fibrosis (IPF) is a progressive lung disease characterized by aberrant fibroblast activation, lysosomal dysfunction, and cellular senescence. Transcriptomic analyses have identified ependymin-related 1 (EPDR1) as a fibroblast-enriched gene in IPF, but its biological function remains unclear. EPDR1 expression was assessed in lung fibroblasts, lung tissues, bronchoalveolar lavage fluid (BALF), and serum from IPF patients and controls using qPCR, Western blotting, ELISA, and immunohistochemistry. Lysosomal function, autophagic flux, and senescence markers were analyzed in primary fibroblasts following siRNA-mediated EPDR1 knockdown. EPDR1 was significantly upregulated in IPF-derived fibroblasts and localized to fibrotic regions enriched with α-SMA+, COL1A1+, and FN1+ myofibroblasts of IPF-derived lung tissues. EPDR1 levels were markedly elevated in the BALF and serum of IPF patients and correlated with increased mortality. IPF fibroblasts exhibited reduced lysosomal acidification and impaired autophagic flux, indicated by p62 and LC3B accumulation. EPDR1 knockdown restored lysosomal function; enhanced autophagic degradation; and reduced senescence markers, including p21, p16, and SA-β-gal activity. EPDR1 drives lysosomal dysfunction and fibroblast senescence in IPF. Its elevated expression in lung tissue and biological fluids, together with its association with prognosis, highlights EPDR1 as a potential biomarker and therapeutic target in IPF. Full article
(This article belongs to the Special Issue Advances in Pulmonary Fibrosis)
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