ArticleBiochemical and biophysical research communications2013
Ceramide mediates lung fibrosis in cystic fibrosis.
Article in Biochemical and biophysical research communications, 2013. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 25 papers.
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Who cites it
25 citing papers in PubMed, 44 citations in OpenAlex.
- Plasma Levels of the Bioactive Sphingolipid Metabolite S1P in Adult Cystic Fibrosis Patients: Potential Target for Immunonutrition?Nutrients · 2020Trial
- Sphingosine phosphate lyase insufficiency syndrome as a primary immunodeficiency state.Advances in biological regulation · 2024Review
- Integrated-omics analysis with explainable deep networks on pathobiology of infant bronchiolitis.NPJ systems biology and applications · 2024Article
- Functional roles of sphingolipids in immunity and their implication in disease.Experimental & molecular medicine · 2023Review
- Lipidomic alterations in human saliva from cystic fibrosis patients.Scientific reports · 2023Article
- Lipidomics Profiles and Lipid Metabolite Biomarkers in Serum of Coal Workers' Pneumoconiosis.Toxics · 2022Article
- Spns2 Transporter Contributes to the Accumulation of S1P in Cystic Fibrosis Human Bronchial Epithelial Cells.Biomedicines · 2021Article
- The development of HEC-866 and its analogues for the treatment of idiopathic pulmonary fibrosis.RSC medicinal chemistry · 2021Article
- Activation of Sphingomyelinase-Ceramide-Pathway in COVID-19 Purposes Its Inhibition for Therapeutic Strategies.Frontiers in immunology · 2021Article
- Plasma Lipid Profiling of Three Types of Drug-Induced Liver Injury in Japanese Patients: A Preliminary Study.Metabolites · 2020Article
- Cystic Fibrosis Defective Response to Infection Involves Autophagy and Lipid Metabolism.Cells · 2020Article
- An Innovative Lipidomic Workflow to Investigate the Lipid Profile in a Cystic Fibrosis Cell Line.Cells · 2020Article
- Article
- Crosstalk Between Acid Sphingomyelinase and Inflammasome Signaling and Their Emerging Roles in Tissue Injury and Fibrosis.Frontiers in cell and developmental biology · 2019Review
- Neutrophil elastase increases airway ceramide levels via upregulation of serine palmitoyltransferase.American journal of physiology. Lung cellular and molecular physiology · 2018Article
- Burn injury influences the T cell homeostasis in a butyrate-acid sphingomyelinase dependent manner.Cellular immunology · 2017Article
- Amitriptyline Usage Exacerbates the Immune Suppression Following Burn Injury.Shock (Augusta, Ga.) · 2016Article
- Microbial, host and xenobiotic diversity in the cystic fibrosis sputum metabolome.The ISME journal · 2016Article
- Metabolomics of pulmonary exacerbations reveals the personalized nature of cystic fibrosis disease.PeerJ · 2016Article
- Characterization of hepatic lipid profiles in a mouse model with nonalcoholic steatohepatitis and subsequent fibrosis.Scientific reports · 2015Article
Corrections and comments
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Authors and funding
5 authors at 1 institution in 2 countries.
Funding
Abstract
Fibrosis of the lung is one of the major clinical problems of cystic fibrosis and chronic obstructive pulmonary disease. However, the molecular mechanisms leading to pulmonary fibrosis are poorly characterized and require definition. Here, we demonstrate that chronic accumulation of ceramide in the lung contributes to the development of fibrosis in aged cystic fibrosis mice. Genetic or pharmacological normalization of ceramide in cystic fibrosis mice, which was achieved by heterozygosity of acid sphingomyelinase or chronic (6.5 month long) treatment of mice with pharmacological inhibitors of acid sphingomyelinase significantly decreased the development of lung fibrosis. Moreover, our studies demonstrate that long-term treatment of cystic fibrosis mice with pharmacological inhibitors of acid sphingomyelinase or genetic heterozygosity of the enzyme also minimizes pulmonary inflammatory cytokines in cystic fibrosis mice. This data identifies ceramide as a key molecule associated with pulmonary fibrosis in cystic fibrosis mice and demonstrate for the first time that prolonged inhibition of acid sphingomyelinase is able to attenuate fibrosis and inflammation in this animal model.
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Registered trials
Read under generation 80e0d062 · epoch 390. Bibliography from PubMed, PubMed Central and OpenAlex; grants from NIH RePORTER; trial links from ClinicalTrials.gov; estimates, votes and beliefs from the OpenQuestion graph.