ArticleThe open respiratory medicine journal2010
The role of sphingolipids and ceramide in pulmonary inflammation in cystic fibrosis.
Article in The open respiratory medicine journal, 2010. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 16 papers.
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Trials whose registry record cites this paper, or whose number appears in the abstract. A trial that started after this paper was published is citing it as background, not reporting it.
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Who cites it
16 citing papers in PubMed, 13 citations in OpenAlex.
- Effects of GM1 ganglioside and its derivatives on ETI-rescued F508del-CFTR maturation and host-pathogen interactions in cystic fibrosis bronchial cells.Glycoconjugate journal · 2025Article
- Genomic regions associated with bovine respiratory disease in pacific northwest Holstein cattle.Frontiers in veterinary science · 2025Article
- Mapping the oxidative landscape in cystic fibrosis: methodological frontiers and application.Frontiers in pharmacology · 2025Review
- Review
- Phospholipid analysis of two influenza A virus-infected cell lines differing in their viral replication kinetics.Archives of virology · 2023Article
- Metabonomic analysis of plasma biochemical changes in pyrexia rats after treatment with Gegenqinlian decoction, aspirin and itraconazole by UHPLC-FT-ICR-MS.Journal of pharmaceutical analysis · 2020Article
- Cystic Fibrosis: Fighting Together Against Coronavirus Infection.Frontiers in medicine · 2020Article
- Ceramide Suppresses Influenza A Virus ReplicationJournal of virology · 2019Article
- 16S rRNA gene sequencing reveals site-specific signatures of the upper and lower airways of cystic fibrosis patients.Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society · 2018Article
- Ceramide synthase 2 deficiency aggravates AOM-DSS-induced colitis in mice: role of colon barrier integrity.Cellular and molecular life sciences : CMLS · 2017Article
- Ceramide synthesis regulates T cell activity and GVHD development.JCI insight · 2017Article
- Sphingolipids in neutrophil function and inflammatory responses: Mechanisms and implications for intestinal immunity and inflammation in ulcerative colitis.Advances in biological regulation · 2017Review
- Reduced GM1 ganglioside in CFTR-deficient human airway cells results in decreased β1-integrin signaling and delayed wound repair.American journal of physiology. Cell physiology · 2014Article
- Sphingolipids and brain resident macrophages in neuroinflammation: an emerging aspect of nervous system pathology.Clinical & developmental immunology · 2013Review
- Protein histidine [de]phosphorylation in insulin secretion: abnormalities in models of impaired insulin secretion.Naunyn-Schmiedeberg's archives of pharmacology · 2011Review
- Beyond the cherry-red spot: Ocular manifestations of sphingolipid-mediated neurodegenerative and inflammatory disorders.Survey of ophthalmologyReview
Corrections and comments
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Authors and funding
4 authors at 2 institutions in 1 country.
Funding
No grant is acknowledged in the PubMed record.
Abstract
Sphingolipids and in particular ceramide have been shown to be critically involved in the response to many receptor-mediated, but also receptor-independent, mainly stress stimuli. Recent studies demonstrate that ceramide plays an important role in the pathogenesis of cystic fibrosis, a hereditary metabolic disorder caused by mutations of the Cystic Fibrosis Transmembrane Conductance Regulator. Patients with cystic fibrosis suffer from chronic pulmonary inflammation and microbial lung infections, in particular with Pseudomonas aeruginosa. Chronic pulmonary inflammation in these patients seems to be the initial pathophysiological event. Inflammation may finally result in the high infection susceptibility of these patients, fibrosis and loss of lung function. Recent studies demonstrated that ceramide accumulates in lungs of cystic fibrosis mice and causes age-dependent pulmonary inflammation as indicated by accumulation of neutrophils and macrophages in the lung and increased pulmonary concentrations of Interleukins 1 and 8, death of bronchial epithelial cells, deposition of DNA in bronchi and high susceptibility to Pseudomonas aeruginosa infections. Genetic or pharmacological inhibition of the acid sphingomyelinase blocks excessive ceramide production in lungs of cystic fibrosis mice and corrects pathological lung findings. First clinical studies confirm that inhibition of the acid sphingomyelinase with small molecules might be a novel strategy to treat patients with cystic fibrosis.
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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.