ArticleJournal of cystic fibrosis : official journal of the European Cystic Fibrosis Society2019
Systemic levels of anti-PAD4 autoantibodies correlate with airway obstruction in cystic fibrosis.
Article in Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society, 2019. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 28 papers.
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Who cites it
28 citing papers in PubMed, 36 citations in OpenAlex.
- Myeloperoxidase and Other Markers of Neutrophil Activation Associate With Malaria and Malaria/HIV Coinfection in the Human Placenta.Frontiers in immunology · 2021Trial
- Novel Biomarker for Rheumatoid Arthritis: Serum Peptidylarginine Deiminase 4-Potentiating Activity in an Anti-Citrullinated Protein Antibody-Positive At-Risk Population.Scandinavian journal of immunology · 2026Article
- Inhibition of <italic>Pseudomonas aeruginosa</italic> Biofilm Formation by Peptidyl-Arginine Deiminases 2 and 4.Journal of innate immunity · 2026Article
- Article
- Exploring Neutrophil Heterogeneity and Plasticity in Health and Disease.Biomedicines · 2025Review
- Neutrophil extracellular traps and interleukin-1β in cystic fibrosis lung disease.Frontiers in immunology · 2025Review
- Alveolar epithelial cell dysfunction and epithelial-mesenchymal transition in pulmonary fibrosis pathogenesis.Frontiers in molecular biosciences · 2025Review
- Neutrophil-Derived Peptidyl Arginine Deiminase Activity Contributes to Pulmonary Emphysema by Enhancing Elastin Degradation.Journal of immunology (Baltimore, Md. : 1950) · 2024Article
- The Bifunctional Effects of Lactoferrin (LFcinB11) in Inhibiting Neural Cell Adhesive Molecule (NCAM) Polysialylation and the Release of Neutrophil Extracellular Traps (NETs).International journal of molecular sciences · 2024Article
- A crucial role of neutrophil extracellular traps in pulmonary infectious diseases.Chinese medical journal pulmonary and critical care medicine · 2024Review
- Review
- Cystic fibrosis autoantibody signatures associate withFrontiers in immunology · 2023Article
- Methods for the Assessment of NET Formation: From Neutrophil Biology to Translational Research.International journal of molecular sciences · 2022Review
- The S100A7 nuclear interactors in autoimmune diseases: a coevolutionary study in mammals.Immunogenetics · 2022Article
- Platelet-leukocyte crosstalk in COVID-19: How might the reciprocal links between thrombotic events and inflammatory state affect treatment strategies and disease prognosis?Thrombosis research · 2022Review
- Serum anti-PAD4 autoantibodies are present in cystic fibrosis children and increase with age and lung disease severity.Autoimmunity · 2022Article
- Outcome predictors in COVID-19: An analysis of emergent systemic inflammation indices in Mexican population.Frontiers in medicine · 2022Article
- An Immunoproteomic Survey of the Antibody Landscape: Insights and Opportunities Revealed by Serological Repertoire Profiling.Frontiers in immunology · 2022Review
- Neutrophil extracellular traps are present in the airways of ENaC-overexpressing mice with cystic fibrosis-like lung disease.BMC immunology · 2021Article
- Type-4 Phosphodiesterase (PDE4) Blockade Reduces NETosis in Cystic Fibrosis.Frontiers in pharmacology · 2021Article
Corrections and comments
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Authors and funding
8 authors at 4 institutions in 1 country.
Funding
Abstract
Cystic fibrosis (CF) airway disease is characterized by the long-term presence of neutrophil granulocytes. Formation of neutrophil extracellular traps (NETs) and/or autoantibodies directed against extracellular components of NETs are possible contributors to neutrophil-mediated lung damage in CF. The goal of this study was to measure their levels in CF adults compared to healthy controls and subjects with rheumatologic diseases known to develop NET-related autoantibodies and pathologies, rheumatoid arthritis (RA) and systemic lupus erythematosus (SLE). Sera were analyzed from the following number of subjects: 37 CF, 23 healthy controls (HC), 20 RA, and 21 SLE. CF had elevated serum myeloperoxidase (MPO) concentrations (347.5±56.1 ng/ml, mean+/-S.E.M., p = .0132) compared to HC (144.5±14.6 ng/ml) but not of neutrophil elastase (NE) complexed with alpha-1-antitrypsin, cell-free DNA or NE-DNA complexes. The peptidylarginine deiminase 4 (PAD4) enzyme is required for NET formation and associated DNA release in neutrophils. Serum levels of anti-PAD4 antibodies (Ab) were elevated in CF (p = .0147) compared to HC and showed an inverse correlation with a measure of lung function, FEV1% predicted (r = -0.5020, p = .015), as did MPO levels (r = -0.4801, p = .0026). Anti-PAD4 Ab levels in CF sera associated with lung infection by P. aeruginosa, but not that by S. aureus, age, sex, CF-related diabetes or the presence of musculoskeletal pain. Serum levels of anti-citrullinated protein Abs (ACPAs) and anti-nucleosome Abs were not elevated in CF compared to HC (p = .7498, p = .0678). In summary, adult CF subjects develop an autoimmune response against NET components that correlates with worsening 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.