Elexacaftor/Tezacaftor/Ivacaftor Accelerates Wound Repair in Cystic Fibrosis Airway Epithelium

Background: Cystic fibrosis (CF) airway epithelium shows alterations in repair following damage. In vitro studies showed that lumacaftor/ivacaftor (Orkambi) may favor airway epithelial integrity in CF patients. Our aim was to evaluate the effect of the novel triple combination elexacaftor/tezacaftor...

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Main Authors: Onofrio Laselva, Massimo Conese
Format: Article
Language:English
Published: MDPI AG 2022-09-01
Series:Journal of Personalized Medicine
Subjects:
Online Access:https://www.mdpi.com/2075-4426/12/10/1577
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author Onofrio Laselva
Massimo Conese
author_facet Onofrio Laselva
Massimo Conese
author_sort Onofrio Laselva
collection DOAJ
description Background: Cystic fibrosis (CF) airway epithelium shows alterations in repair following damage. In vitro studies showed that lumacaftor/ivacaftor (Orkambi) may favor airway epithelial integrity in CF patients. Our aim was to evaluate the effect of the novel triple combination elexacaftor/tezacaftor/ivacaftor (ETI) on wound repair in CF airway epithelial cells. Methods: A tip-based scratch assay was employed to study wound repair in monolayers of CFBE14o- cells overexpressing the <i>F508del</i> mutation. ETI was added during wound repair. Results: ETI efficiently rescued CFTR F508del maturation and activity, accelerated wound closure and increased wound healing rates of the injured CF cell monolayers. Conclusions: The triple corrector/potentiator combination ETI shows promise in ameliorating wound healing of the airway epithelium in <i>F508del</i> patients.
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spelling doaj.art-076e50170dd740fb9b5ae5fd74ae4ff62023-11-24T00:48:43ZengMDPI AGJournal of Personalized Medicine2075-44262022-09-011210157710.3390/jpm12101577Elexacaftor/Tezacaftor/Ivacaftor Accelerates Wound Repair in Cystic Fibrosis Airway EpitheliumOnofrio Laselva0Massimo Conese1Department of Clinical and Experimental Medicine, University of Foggia, 71122 Foggia, ItalyDepartment of Clinical and Experimental Medicine, University of Foggia, 71122 Foggia, ItalyBackground: Cystic fibrosis (CF) airway epithelium shows alterations in repair following damage. In vitro studies showed that lumacaftor/ivacaftor (Orkambi) may favor airway epithelial integrity in CF patients. Our aim was to evaluate the effect of the novel triple combination elexacaftor/tezacaftor/ivacaftor (ETI) on wound repair in CF airway epithelial cells. Methods: A tip-based scratch assay was employed to study wound repair in monolayers of CFBE14o- cells overexpressing the <i>F508del</i> mutation. ETI was added during wound repair. Results: ETI efficiently rescued CFTR F508del maturation and activity, accelerated wound closure and increased wound healing rates of the injured CF cell monolayers. Conclusions: The triple corrector/potentiator combination ETI shows promise in ameliorating wound healing of the airway epithelium in <i>F508del</i> patients.https://www.mdpi.com/2075-4426/12/10/1577wound repairairway epithelial cellsCFTRcystic fibrosisCFTR modulators
spellingShingle Onofrio Laselva
Massimo Conese
Elexacaftor/Tezacaftor/Ivacaftor Accelerates Wound Repair in Cystic Fibrosis Airway Epithelium
Journal of Personalized Medicine
wound repair
airway epithelial cells
CFTR
cystic fibrosis
CFTR modulators
title Elexacaftor/Tezacaftor/Ivacaftor Accelerates Wound Repair in Cystic Fibrosis Airway Epithelium
title_full Elexacaftor/Tezacaftor/Ivacaftor Accelerates Wound Repair in Cystic Fibrosis Airway Epithelium
title_fullStr Elexacaftor/Tezacaftor/Ivacaftor Accelerates Wound Repair in Cystic Fibrosis Airway Epithelium
title_full_unstemmed Elexacaftor/Tezacaftor/Ivacaftor Accelerates Wound Repair in Cystic Fibrosis Airway Epithelium
title_short Elexacaftor/Tezacaftor/Ivacaftor Accelerates Wound Repair in Cystic Fibrosis Airway Epithelium
title_sort elexacaftor tezacaftor ivacaftor accelerates wound repair in cystic fibrosis airway epithelium
topic wound repair
airway epithelial cells
CFTR
cystic fibrosis
CFTR modulators
url https://www.mdpi.com/2075-4426/12/10/1577
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