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Volume 22
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10.3390/ijms22147606
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Review

Anti-Inflammatory Influences of Cystic Fibrosis Transmembrane Conductance Regulator Drugs on Lung Inflammation in Cystic Fibrosis

by
Kiera H. Harwood
1,
Rachel M. McQuade
2,3,
Andrew Jarnicki
4,* and
Elena K. Schneider-Futschik
1,*
1
Department of Biochemistry & Pharmacology, Faculty of Medicine, Dentistry and Health Sciences, School of Biomedical Sciences, The University of Melbourne, Parkville, VIC 3010, Australia
2
Gut-Axis Injury and Repair Laboratory, Department of Medicine Western Health, Melbourne University, Melbourne, VIC 3021, Australia
3
The Florey Institute of Neuroscience and Mental Health, Parkville, VIC 3010, Australia
4
Lung Disease Research Laboratory, Department of Biochemistry & Pharmacology, Melbourne University, Melbourne, VIC 3021, Australia
*
Authors to whom correspondence should be addressed.
Int. J. Mol. Sci. 2021, 22(14), 7606; https://doi.org/10.3390/ijms22147606
Submission received: 15 June 2021 / Revised: 4 July 2021 / Accepted: 13 July 2021 / Published: 16 July 2021
(This article belongs to the Special Issue Target Dysfunctional Inflammation in Cystic Fibrosis Airway)
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Abstract

Cystic fibrosis (CF) is caused by a defect in the cystic fibrosis transmembrane conductance regulator protein (CFTR) which instigates a myriad of respiratory complications including increased vulnerability to lung infections and lung inflammation. The extensive influx of pro-inflammatory cells and production of mediators into the CF lung leading to lung tissue damage and increased susceptibility to microbial infections, creates a highly inflammatory environment. The CF inflammation is particularly driven by neutrophil infiltration, through the IL-23/17 pathway, and function, through NE, NETosis, and NLRP3-inflammasome formation. Better understanding of these pathways may uncover untapped therapeutic targets, potentially reducing disease burden experienced by CF patients. This review outlines the dysregulated lung inflammatory response in CF, explores the current understanding of CFTR modulators on lung inflammation, and provides context for their potential use as therapeutics for CF. Finally, we discuss the determinants that need to be taken into consideration to understand the exaggerated inflammatory response in the CF lung.
Keywords: cystic fibrosis; ivacaftor; lumacaftor; CFTR modulator; inflammation; lung inflammation; anti-inflammatory treatment cystic fibrosis; ivacaftor; lumacaftor; CFTR modulator; inflammation; lung inflammation; anti-inflammatory treatment

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MDPI and ACS Style

Harwood, K.H.; McQuade, R.M.; Jarnicki, A.; Schneider-Futschik, E.K. Anti-Inflammatory Influences of Cystic Fibrosis Transmembrane Conductance Regulator Drugs on Lung Inflammation in Cystic Fibrosis. Int. J. Mol. Sci. 2021, 22, 7606. https://doi.org/10.3390/ijms22147606

AMA Style

Harwood KH, McQuade RM, Jarnicki A, Schneider-Futschik EK. Anti-Inflammatory Influences of Cystic Fibrosis Transmembrane Conductance Regulator Drugs on Lung Inflammation in Cystic Fibrosis. International Journal of Molecular Sciences. 2021; 22(14):7606. https://doi.org/10.3390/ijms22147606

Chicago/Turabian Style

Harwood, Kiera H., Rachel M. McQuade, Andrew Jarnicki, and Elena K. Schneider-Futschik. 2021. "Anti-Inflammatory Influences of Cystic Fibrosis Transmembrane Conductance Regulator Drugs on Lung Inflammation in Cystic Fibrosis" International Journal of Molecular Sciences 22, no. 14: 7606. https://doi.org/10.3390/ijms22147606

APA Style

Harwood, K. H., McQuade, R. M., Jarnicki, A., & Schneider-Futschik, E. K. (2021). Anti-Inflammatory Influences of Cystic Fibrosis Transmembrane Conductance Regulator Drugs on Lung Inflammation in Cystic Fibrosis. International Journal of Molecular Sciences, 22(14), 7606. https://doi.org/10.3390/ijms22147606

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