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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Format: | Article |
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MDPI AG
2022-09-01
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Series: | Journal of Personalized Medicine |
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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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institution | Directory Open Access Journal |
issn | 2075-4426 |
language | English |
last_indexed | 2024-03-09T19:58:38Z |
publishDate | 2022-09-01 |
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series | Journal of Personalized Medicine |
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 |
work_keys_str_mv | AT onofriolaselva elexacaftortezacaftorivacaftoraccelerateswoundrepairincysticfibrosisairwayepithelium AT massimoconese elexacaftortezacaftorivacaftoraccelerateswoundrepairincysticfibrosisairwayepithelium |