L1077P CFTR pathogenic variant function rescue by Elexacaftor–Tezacaftor–Ivacaftor in cystic fibrosi...
L1077P CFTR pathogenic variant function rescue by Elexacaftor–Tezacaftor–Ivacaftor in cystic fibrosis patient-derived air–liquid interface (ALI) cultures and organoids: in vitro guided personalized therapy of non-F508del patients
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Author / Creator
Lo Cicero, Stefania , Castelli, Germana , Blaconà, Giovanna , Bruno, Sabina Maria , Sette, Giovanni , Pigliucci, Riccardo , Villella, Valeria Rachela , Esposito, Speranza , Zollo, Immacolata , Spadaro, Francesca , Maria, Ruggero De , Biffoni, Mauro , Cimino, Giuseppe , Amato, Felice , Lucarelli, Marco and Eramo, Adriana
Publisher
London: BioMed Central Ltd
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Language
English
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Publisher
London: BioMed Central Ltd
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Scope and Contents
Contents
Cystic fibrosis (CF) is caused by defects of the cystic fibrosis transmembrane conductance regulator (CFTR) gene. CFTR-modulating drugs may overcome specific defects, such as the case of Trikafta, which is a clinically approved triple combination of Elexacaftor, Tezacaftor and Ivacaftor (ETI) that exhibited a strong ability to rescue the function o...
Alternative Titles
Full title
L1077P CFTR pathogenic variant function rescue by Elexacaftor–Tezacaftor–Ivacaftor in cystic fibrosis patient-derived air–liquid interface (ALI) cultures and organoids: in vitro guided personalized therapy of non-F508del patients
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TN_cdi_doaj_primary_oai_doaj_org_article_b4d7cae7df80409786f2da0722aa6ceb
Permalink
https://devfeature-collection.sl.nsw.gov.au/record/TN_cdi_doaj_primary_oai_doaj_org_article_b4d7cae7df80409786f2da0722aa6ceb
Other Identifiers
ISSN
1465-993X,1465-9921
E-ISSN
1465-993X,1465-9921
DOI
10.1186/s12931-023-02516-0
