ArticleJournal of cystic fibrosis : official journal of the European Cystic Fibrosis Society2017
Monocyte derived macrophages from CF pigs exhibit increased inflammatory responses at birth.
Article in Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society, 2017. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 26 papers.
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Who cites it
26 citing papers in PubMed, 38 citations in OpenAlex.
- Antiviral defenses are diminished at birth in cystic fibrosis pig airways.Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society · 2026Article
- Considerations for early life genetic therapies in cystic fibrosis.American journal of physiology. Lung cellular and molecular physiology · 2026Review
- Analysis of CFTR mRNA and Protein in Peripheral Blood Mononuclear Cells via Quantitative Real-Time PCR and Western Blot.International journal of molecular sciences · 2024Article
- Effects of Akt Activator SC79 on Human M0 Macrophage Phagocytosis and Cytokine Production.Cells · 2024Article
- Animals in Respiratory Research.International journal of molecular sciences · 2024Review
- Human CFTR deficient iPSC-macrophages reveal impaired functional and transcriptomic response uponFrontiers in immunology · 2024Article
- CAGE sequencing reveals CFTR-dependent dysregulation of type I IFN signaling in activated cystic fibrosis macrophages.Science advances · 2023Article
- Single-Cell RNA Sequencing Reveals New Basic and Translational Insights in the Cystic Fibrosis Lung.American journal of respiratory cell and molecular biology · 2023Review
- ScRNA-seq expression ofLife science alliance · 2022Article
- Macrophages from gut-corrected CF mice express human CFTR and lack a pro-inflammatory phenotype.Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society · 2022Article
- Mucosal Immunity in Cystic Fibrosis.Journal of immunology (Baltimore, Md. : 1950) · 2021Review
- Review
- Review
- Rescue from Pseudomonas aeruginosa Airway Infection via Stem Cell Transplantation.Molecular therapy : the journal of the American Society of Gene Therapy · 2021Article
- LasR-deficient Pseudomonas aeruginosa variants increase airway epithelial mICAM-1 expression and enhance neutrophilic lung inflammation.PLoS pathogens · 2021Article
- Editorial: Special Issue on "Therapeutic Approaches for Cystic Fibrosis".International journal of molecular sciences · 2020Article
- Strong toll-like receptor responses in cystic fibrosis patients are associated with higher lung function.Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society · 2020Article
- Consequences of CRISPR-Cas9-Mediated CFTR Knockout in Human Macrophages.Frontiers in immunology · 2020Article
- Cystic Fibrosis Plasma Blunts the Immune Response to Bacterial Infection.American journal of respiratory cell and molecular biology · 2019Article
- Elastase Exocytosis by Airway Neutrophils Is Associated with Early Lung Damage in Children with Cystic Fibrosis.American journal of respiratory and critical care medicine · 2019Article
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Authors and funding
8 authors at 1 institution in 2 countries.
Funding
Abstract
backgroundWe sought to address whether CF macrophages have a primary functional defect as a consequence of CFTR loss and thus contribute to the onset of infection and inflammation observed in CF lung disease.
methodsMonocyte derived macrophages (MDMs) were prepared from newborn CF and non-CF pigs. CFTR mRNA expression was quantified by rtPCR and anion channel function was determined using whole cell patch clamp analysis. IL8 and TNFα release from MDMs in response to lipopolysaccharide stimulation was measured by ELISA.
resultsCFTR was expressed in MDMs by Q-rtPCR at a lower level than in epithelial cells. MDMs exhibited functional CFTR current at the cell membrane and this current was absent in CF MDMs. CF MDMs demonstrated an exaggerated response to lipopolysaccharide stimulation.
conclusionsIn the absence of CFTR function, macrophages from newborn CF pigs exhibit an increased inflammatory response to a lipopolysaccharide challenge. This may contribute to the onset and progression of CF lung disease.
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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.