ArticleThe Journal of biological chemistry2010
Metabolomic profiling reveals biochemical pathways and biomarkers associated with pathogenesis in cystic fibrosis cells.
Article in The Journal of biological chemistry, 2010. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 37 papers.
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Who cites it
37 citing papers in PubMed, 98 citations in OpenAlex.
- Multi-modal tissue-aware graph neural network forbioRxiv : the preprint server for biology · 2026Article
- Photoaffinity Ligand of Cystic Fibrosis Corrector VX-445 Identifies SCCPDH as an Off-Target.ACS chemical biology · 2025Article
- CF airway epithelia display exaggerated host defense responses and prolonged cilia loss during RSV infection.Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society · 2025Article
- Ventromedial hypothalamic nucleus subset stimulates tissue thermogenesis via preoptic area outputs.Molecular metabolism · 2024Article
- Proteomics and metabolomics profiling reveal panels of circulating diagnostic biomarkers and molecular subtypes in stable COPD.Respiratory research · 2023Article
- Identification of Exhaled Metabolites in Children with Cystic Fibrosis.Metabolites · 2022Article
- Sweat metabolomics before and after intravenous antibiotics for pulmonary exacerbation in people with cystic fibrosis.Respiratory medicine · 2022Article
- The cystic fibrosis lung microenvironment alters antibiotic activity: causes and effects.European respiratory review : an official journal of the European Respiratory Society · 2021Review
- Mechanistic Investigation of Xuebijing for Treatment of Paraquat-Induced Pulmonary Fibrosis by Metabolomics and Network Pharmacology.ACS omega · 2021Article
- Distinctive metabolic profiles between Cystic Fibrosis mutational subclasses and lung function.Metabolomics : Official journal of the Metabolomic Society · 2021Article
- Metabolomics profiling of tobacco exposure in children with cystic fibrosis.Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society · 2020Article
- Proteomics and Metabolomics for Cystic Fibrosis Research.International journal of molecular sciences · 2020Review
- Rhinovirus Infection Drives Complex Host Airway Molecular Responses in Children With Cystic Fibrosis.Frontiers in immunology · 2020Article
- An Intriguing Involvement of Mitochondria in Cystic Fibrosis.Journal of clinical medicine · 2019Review
- Metabonomics reveals altered metabolites related to inflammation and energy utilization at recovery of cystic fibrosis lung exacerbation.Metabolism open · 2019Article
- Urinary metabolomics reveals unique metabolic signatures in infants with cystic fibrosis.Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society · 2019Article
- Modulation of glucose-related metabolic pathways controls glucose level in airway surface liquid and fight oxidative stress in cystic fibrosis cells.Journal of bioenergetics and biomembranes · 2019Article
- Host metabolites stimulate the bacterial proton motive force to enhance the activity of aminoglycoside antibiotics.PLoS pathogens · 2019Article
- Impairment of CFTR activity in cultured epithelial cells upregulates the expression and activity of LDH resulting in lactic acid hypersecretion.Cellular and molecular life sciences : CMLS · 2019Article
- Aberrant GSH reductase and NOX activities concur with defective CFTR to pro-oxidative imbalance in cystic fibrosis airways.Journal of bioenergetics and biomembranes · 2018Article
Corrections and comments
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Authors and funding
9 authors at 4 institutions in 2 countries.
Funding
No grant is acknowledged in the PubMed record.
Abstract
Cystic fibrosis (CF) is a life-shortening disease caused by a mutation in the cystic fibrosis transmembrane conductance regulator (CFTR) gene. To gain an understanding of the epithelial dysfunction associated with CF mutations and discover biomarkers for therapeutics development, untargeted metabolomic analysis was performed on primary human airway epithelial cell cultures from three separate cohorts of CF patients and non-CF subjects. Statistical analysis revealed a set of reproducible and significant metabolic differences between the CF and non-CF cells. Aside from changes that were consistent with known CF effects, such as diminished cellular regulation against oxidative stress and osmotic stress, new observations on the cellular metabolism in the disease were generated. In the CF cells, the levels of various purine nucleotides, which may function to regulate cellular responses via purinergic signaling, were significantly decreased. Furthermore, CF cells exhibited reduced glucose metabolism in glycolysis, pentose phosphate pathway, and sorbitol pathway, which may further exacerbate oxidative stress and limit the epithelial cell response to environmental pressure. Taken together, these findings reveal novel metabolic abnormalities associated with the CF pathological process and identify a panel of potential biomarkers for therapeutic development using this model system.
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