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Progress in Model Systems of Cystic Fibrosis Mucosal Inflammation to Understand Aberrant Neutrophil Activity
- Source :
- Frontiers in Immunology, Vol 11 (2020), Frontiers in Immunology
- Publication Year :
- 2019
-
Abstract
- In response to recurrent infection in cystic fibrosis (CF), powerful innate immune signals trigger polymorphonuclear neutrophil recruitment into the airway lumen. Exaggerated neutrophil proteolytic activity results in sustained inflammation and scarring of the airways. Consequently, neutrophils and their secretions are reliable clinical biomarkers of lung disease progression. As neutrophils are required to clear infection and yet a direct cause of airway damage, modulating adverse neutrophil activity while preserving their pathogen fighting function remains a key area of CF research. The factors that drive their pathological behavior are still under investigation, especially in early disease when aberrant neutrophil behavior first becomes evident. Here we examine the latest findings of neutrophils in pediatric CF lung disease and proposed mechanisms of their pathogenicity. Highlighted in this review are current and emerging experimental methods for assessing CF mucosal immunity and human neutrophil function in the laboratory.
- Subjects :
- 0301 basic medicine
lcsh:Immunologic diseases. Allergy
Human neutrophil
Cystic Fibrosis
Neutrophils
Mini Review
Immunology
Mucosal inflammation
Inflammation
Respiratory Mucosa
In Vitro Techniques
Cystic fibrosis
Models, Biological
model systems
03 medical and health sciences
0302 clinical medicine
medicine
Immunology and Allergy
Animals
Humans
Pathological
Pathogen
Respiratory Tract Infections
Innate immune system
business.industry
neutrophil
medicine.disease
infection
Disease Models, Animal
030104 developmental biology
medicine.symptom
Airway
business
lcsh:RC581-607
030215 immunology
Subjects
Details
- ISSN :
- 16643224
- Volume :
- 11
- Database :
- OpenAIRE
- Journal :
- Frontiers in immunology
- Accession number :
- edsair.doi.dedup.....98cd7ba647cbfa5e9871eea6fee44b25