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Therapeutic Approaches for Patients with Cystic Fibrosis Not Eligible for Current CFTR Modulators
Review

CFTR Protein: Not Just a Chloride Channel?

1
Department of Pediatric Pulmonology and Cystic Fibrosis Clinic, Hôpital Universitaire des Enfants Reine Fabiola, Université Libre de Bruxelles (ULB), Avenue J.J. Crocq 15, 1020 Brussels, Belgium
2
Laboratoire Académique de Pédiatrie, Hôpital Universitaire des Enfants Reine Fabiola, Université Libre de Bruxelles (ULB), Avenue J.J. Crocq 15, 1020 Brussels, Belgium
*
Author to whom correspondence should be addressed.
Academic Editor: Gergely L. Lukacs
Cells 2021, 10(11), 2844; https://doi.org/10.3390/cells10112844
Received: 23 September 2021 / Revised: 15 October 2021 / Accepted: 19 October 2021 / Published: 22 October 2021
(This article belongs to the Special Issue Cystic Fibrosis: Cells, Physiopathology and Emerging Therapies)
Cystic fibrosis (CF) is a recessive genetic disease caused by mutations in a gene encoding a protein called Cystic Fibrosis Transmembrane Conductance Regulator (CFTR). The CFTR protein is known to acts as a chloride (Cl) channel expressed in the exocrine glands of several body systems where it also regulates other ion channels, including the epithelial sodium (Na+) channel (ENaC) that plays a key role in salt absorption. This function is crucial to the osmotic balance of the mucus and its viscosity. However, the pathophysiology of CF is more challenging than a mere dysregulation of epithelial ion transport, mainly resulting in impaired mucociliary clearance (MCC) with consecutive bronchiectasis and in exocrine pancreatic insufficiency. This review shows that the CFTR protein is not just a chloride channel. For a long time, research in CF has focused on abnormal Cl and Na+ transport. Yet, the CFTR protein also regulates numerous other pathways, such as the transport of HCO3, glutathione and thiocyanate, immune cells, and the metabolism of lipids. It influences the pH homeostasis of airway surface liquid and thus the MCC as well as innate immunity leading to chronic infection and inflammation, all of which are considered as key pathophysiological characteristics of CF. View Full-Text
Keywords: cystic fibrosis; CFTR protein; channel; chloride; bicarbonate; glutathione; thiocyanate; macrophages; neutrophils; lipids cystic fibrosis; CFTR protein; channel; chloride; bicarbonate; glutathione; thiocyanate; macrophages; neutrophils; lipids
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MDPI and ACS Style

Hanssens, L.S.; Duchateau, J.; Casimir, G.J. CFTR Protein: Not Just a Chloride Channel? Cells 2021, 10, 2844. https://doi.org/10.3390/cells10112844

AMA Style

Hanssens LS, Duchateau J, Casimir GJ. CFTR Protein: Not Just a Chloride Channel? Cells. 2021; 10(11):2844. https://doi.org/10.3390/cells10112844

Chicago/Turabian Style

Hanssens, Laurence S., Jean Duchateau, and Georges J. Casimir 2021. "CFTR Protein: Not Just a Chloride Channel?" Cells 10, no. 11: 2844. https://doi.org/10.3390/cells10112844

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