Role of USP13 in physiology and diseases DOI Creative Commons
Qian Wang,

Zhenzhen Sun,

Weiwei Xia

et al.

Frontiers in Molecular Biosciences, Journal Year: 2022, Volume and Issue: 9

Published: Sept. 14, 2022

Ubiquitin specific protease (USP)-13 is a deubiquitinase that removes ubiquitin from substrates to prevent protein degradation by the proteasome. Currently, roles of USP13 in physiology and pathology have been reported. In physiology, highly associated with cell cycle regulation, DNA damage repair, myoblast differentiation, quality control endoplasmic reticulum, autophagy. pathology, it has reported important pathogenesis infection, inflammation, idiopathic pulmonary fibrosis (IPF), neurodegenerative diseases, cancers. This mini-review summarizes most recent advances studies involving its pathophysiological different conditions provides new insights into prevention treatment relevant as well further research on USP13.

Language: Английский

Single-Cell Profiling Reveals Immune Aberrations in Progressive Idiopathic Pulmonary Fibrosis DOI
Avraham Unterman, Amy Zhao, Nir Neumark

et al.

American Journal of Respiratory and Critical Care Medicine, Journal Year: 2024, Volume and Issue: 210(4), P. 484 - 496

Published: May 8, 2024

Changes in peripheral blood cell populations have been observed, but not detailed, at single-cell resolution idiopathic pulmonary fibrosis (IPF).

Language: Английский

Citations

21
The uPA/uPAR System Orchestrates the Inflammatory Response, Vascular Homeostasis, and Immune System in Fibrosis Progression DOI Open Access
Yosuke Kanno

International Journal of Molecular Sciences, Journal Year: 2023, Volume and Issue: 24(2), P. 1796 - 1796

Published: Jan. 16, 2023

Fibrotic diseases, such as systemic sclerosis (SSc), idiopathic pulmonary fibrosis, renal fibrosis and liver cirrhosis are characterized by tissue overgrowth due to excessive extracellular matrix (ECM) deposition. Fibrosis progression is caused ECM overproduction the inhibition of degradation several events, including inflammation, vascular endothelial dysfunction, immune abnormalities. Recently, it has been reported that urokinase plasminogen activator (uPA) its receptor (uPAR), known be fibrinolytic factors, orchestrate inflammatory response, homeostasis, homeostasis system. The uPA/uPAR system may show promise a potential therapeutic target for fibrotic diseases. This review considers role in

Language: Английский

Citations

31
Pulmonary fibrosis: Emerging diagnostic and therapeutic strategies DOI
Brintha Selvarajah, Manuela Platé, Rachel C. Chambers

et al.

Molecular Aspects of Medicine, Journal Year: 2023, Volume and Issue: 94, P. 101227 - 101227

Published: Nov. 24, 2023

Language: Английский

Citations

23
Immune cells crosstalk Pathways, and metabolic alterations in Idiopathic pulmonary fibrosis DOI

Purnima Tiwari,

Shobhit Verma,

Kaveri R. Washimkar

et al.

International Immunopharmacology, Journal Year: 2024, Volume and Issue: 135, P. 112269 - 112269

Published: May 22, 2024

Language: Английский

Citations

10
Comparison of nanoLC-MALDI-MS/MS with nanoLC-TIMS-MS/MS in the proteomic analysis of extracellular vesicles of bronchoalveolar lavage fluid DOI
Joanna Kasprzyk, Agnieszka Kamińska, Przemysław Mielczarek

et al.

Analytical Methods, Journal Year: 2025, Volume and Issue: unknown

Published: Jan. 1, 2025

This study compares nanoLC-MALDI-MS/MS and nanoLC-TIMS-MS/MS in analyzing EVs from BALF of asthma IPF patients, emphasizing EVs' roles inflammation, signaling pathways, their potential as diagnostic biomarkers therapeutic targets.

Language: Английский

Citations

1
Lung Organoids—The Ultimate Tool to Dissect Pulmonary Diseases? DOI Creative Commons
Veronika Bosáková, Marco De Zuani,

Lucie Sládková

et al.

Frontiers in Cell and Developmental Biology, Journal Year: 2022, Volume and Issue: 10

Published: July 13, 2022

Organoids are complex multicellular three-dimensional (3D) in vitro models that designed to allow accurate studies of the molecular processes and pathologies human organs. can be derived from a variety cell types, such as primary progenitor cells, pluripotent stem or tumor-derived cells co-cultured with immune microbial further mimic tissue niche. Here, we focus on development 3D lung organoids their use disease drug screening tools. We introduce various experimental approaches used model diseases analyze advantages disadvantages. also discuss validation physiological relevance study diseases. Furthermore, summarize current host-pathogen interactions cystic fibrosis, chronic obstructive pulmonary disease, SARS-CoV-2 infection. Moreover, tumor cancer application personalized medicine research. Finally, outline future research field induced cell-derived organoids.

Language: Английский

Citations

30
Tissue fibroblasts are versatile immune regulators: An evaluation of their impact on the aging process DOI Creative Commons
Antero Salminen, Kai Kaarniranta, Anu Kauppinen

et al.

Ageing Research Reviews, Journal Year: 2024, Volume and Issue: 97, P. 102296 - 102296

Published: April 7, 2024

Fibroblasts are abundant stromal cells which not only control the integrity of extracellular matrix (ECM) but also act as immune regulators. It is known that structural within tissues can establish an organ-specific immunity expressing many immune-related genes and closely interact with cells. In fact, fibroblasts modify their properties to display both pro-inflammatory immunosuppressive activities in a context-dependent manner. After acute insults, promote tissue inflammation although they concurrently recruit enhance resolution inflammation. chronic pathological states, fibroblasts, especially senescent stimulate different return, cells, such M2 macrophages myeloid-derived suppressor (MDSC), evoke excessive conversion into myofibroblasts, thus aggravating severity fibrosis. Single-cell transcriptome studies on isolated from aged have confirmed express coding for cytokines, chemokines, complement factors, whereas lose some fibrogenic properties. The versatile close cooperation indicate crucial role aging process age-related diseases.

Language: Английский

Citations

8
Bufei huoxue capsule alleviates bleomycin-induced pulmonary fibrosis in mice via TGF-β1/Smad2/3 signaling DOI Creative Commons
Yuanyuan Li,

Wenguang Qin,

Qiuling Liang

et al.

Journal of Ethnopharmacology, Journal Year: 2023, Volume and Issue: 316, P. 116733 - 116733

Published: June 3, 2023

Bufei huoxue (BFHX) is a Traditional Chinese Medicine formulation that consists of Astragalus Exscapus L, Paeonia Lactiflora Pall, and Psoralea Aphylla L. It can ameliorate collagen deposition inhibit EMT. However, it remains unknown whether how BFHX alleviates IPF.Our work aimed to explore the therapeutic efficacy on IPF dissect mechanisms involved.A mouse model was induced by bleomycin. administered first day modeling maintained for 21 days. Pulmonary fibrosis inflammation were evaluated micro-CT, lung histopathology, pulmonary function assessment, cytokines in BALF. In addition, we examined signaling molecules involved EMT ECM immunofluorescence, western Blot, EdU, MMP (Δψm) assays.BFHX alleviated parenchyma as evidenced Hematoxylin-eosin (H&E), Masson's trichrome staining, improved function. treatment not only decreased levels interleukin (IL)-6 tumor necrosis factor-α (TNF-α), but also upregulated E-cadherin (E-Cad) downregulated α-smooth muscle actin (α-SMA), Ӏ (Col Ӏ), vimentin, fibronectin (FN). Mechanistically, repressed TGF-β1-driven Smad2/3 phosphorylation, which, turn, suppressed transition fibroblasts myofibroblasts vivo vitro.BFHX effectively reduces occurrence inhibits production inhibiting TGF-β1/Smad2/3 pathway, which provides potential novel strategy IPF.

Language: Английский

Citations

14
Stem cell-based therapy for fibrotic diseases: mechanisms and pathways DOI Creative Commons

Marjan Taherian,

Paria Bayati, Nazanin Mojtabavi

et al.

Stem Cell Research & Therapy, Journal Year: 2024, Volume and Issue: 15(1)

Published: June 18, 2024

Abstract Fibrosis is a pathological process, that could result in permanent scarring and impairment of the physiological function affected organ; this condition which categorized under term organ failure affect various organs different situations. The involvement major organs, such as lungs, liver, kidney, heart, skin, associated with high rate morbidity mortality across world. Fibrotic disorders encompass broad range complications be traced to illnesses impairments; these from simple skin scars beauty issues severe rheumatologic or inflammatory systemic sclerosis well idiopathic pulmonary fibrosis. Besides, overactivation immune responses during any causing tissue damage contribute pathogenic fibrotic events accompanying healing response; for instance, inflammation resulting engraftment cause formation grafted tissue, even cases where system deals hard clear infections, follow adverse effects. A good example complication post-Covid19 lung fibrosis impair life individuals extensive involvement. However, effective therapies halt slow down progression are missing current clinical settings. Considering immunomodulatory regenerative potential distinct stem cell types, their application an anti-fibrotic agent, capable attenuating has been investigated by many researchers. Although majority studies addressing effects cells indicated potent capabilities, underlying mechanisms, pathways impact processes remain poorly understood. Here, we first, review properties types utilized so far treatments discuss challenges limitations applications settings; then, will summarize general organ-specific mechanisms contributing fibrosis; finally, describe considered employed exerting events.

Language: Английский

Citations

6
Modelling bronchial epithelial-fibroblast cross-talk in idiopathic pulmonary fibrosis (IPF) using a human-derived in vitro air liquid interface (ALI) culture DOI Creative Commons
Sarah L. Barron,

Owen Wyatt,

Andy J. O'Connor

et al.

Scientific Reports, Journal Year: 2024, Volume and Issue: 14(1)

Published: Jan. 2, 2024

Abstract Idiopathic Pulmonary Fibrosis (IPF) is a devastating form of respiratory disease with life expectancy 3–4 years. Inflammation, epithelial injury and myofibroblast proliferation have been implicated in initiation and, recently, epithelial-fibroblastic crosstalk has identified as central driver. However, the ability to interrogate this limited due absence vitro models that mimic physiological conditions. To investigate IPF dysregulated cross-talk, primary normal human bronchial (NHBE) cells lung fibroblasts (NHLF) or diseased (DHLF) from patients, were co-cultured direct contact at air–liquid interface (ALI). Intercellular was assessed by comparing cellular phenotypes co-cultures respective monocultures, through optical, biomolecular electrical methods. A co-culture-dependent decrease epithelium thickness, basal cell mRNA (P63, KRT5) an increase transepithelial resistance (TEER) observed. This effect significantly enhanced DHLF lead induction mesenchymal transition (EMT) increased expression TGFβ-2, ZO-1 DN12. When stimulated exogenous TGFβ, NHBE NHLF monocultures showed significant upregulation EMT (COL1A1, FN1, VIM, ASMA) senescence (P21) markers, respectively. In contrast, NHLF/NHBE co-culture indicated protective role cross-talk against TGFβ-induced EMT, fibroblast-to-myofibroblast (FMT) inflammatory cytokine release (IL-6, IL-8, IL-13, IL-1β, TNF-α). no phenotypic upon stimulation, likely constitutively high TGFβ isoforms prior any stimulation. The model developed provides alternative method generate IPF-related vitro, NHBEs. These findings highlight importance fibroblast signaling but give rise differential responses compared co-cultures, when exposed pro-inflammatory stimulus. holds implications for translation conclusions drawn monoculture studies important step development more biomimetic IPF. summary, we believe system study fibroblast-epithelial crosstalk, within context IPF, platform which will aid identification validation novel targets.

Language: Английский

Citations

5