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依列卡福妥/替扎卡福妥/依伐卡托可加速囊性纤维化气道上皮的伤口修复。

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

作者信息

Laselva Onofrio, Conese Massimo

机构信息

Department of Clinical and Experimental Medicine, University of Foggia, 71122 Foggia, Italy.

出版信息

J Pers Med. 2022 Sep 25;12(10):1577. doi: 10.3390/jpm12101577.

DOI:10.3390/jpm12101577
PMID:36294716
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC9605106/
Abstract

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 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 patients.

摘要

背景

囊性纤维化（CF）气道上皮在损伤后的修复过程中表现出改变。体外研究表明，鲁马卡托/依伐卡托（奥瑞希纳）可能有助于CF患者气道上皮的完整性。我们的目的是评估新型三联组合药物依列卡托/替扎卡托/依伐卡托（ETI）对CF气道上皮细胞伤口修复的影响。

方法

采用基于尖端的划痕试验研究过表达该突变的CFBE14o-细胞单层的伤口修复情况。在伤口修复过程中加入ETI。

结果

ETI有效挽救了CFTR F508del的成熟和活性，加速了伤口闭合，并提高了受损CF细胞单层的伤口愈合率。

结论

三联校正剂/增效剂组合ETI在改善CF患者气道上皮伤口愈合方面显示出前景。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/f559/9605106/6374680af249/jpm-12-01577-g003.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/f559/9605106/eae7e1bd1bbb/jpm-12-01577-g001.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/f559/9605106/4538f25bc8d5/jpm-12-01577-g002.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/f559/9605106/6374680af249/jpm-12-01577-g003.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/f559/9605106/eae7e1bd1bbb/jpm-12-01577-g001.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/f559/9605106/4538f25bc8d5/jpm-12-01577-g002.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/f559/9605106/6374680af249/jpm-12-01577-g003.jpg

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