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Article

The combination elexacaftor/tezacaftor/ivacaftor (ETI) modulates the de novo synthethic pathway of ceramides in a genotype-independent manner.

Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society - 1 Jul 2023

Liessi Nara, Tomati Valeria, Capurro Valeria, Loberto Nicoletta, Garcia-Aloy Mar, Franceschi Pietro, Aureli Massimo, Pedemonte Nicoletta, Armirotti Andrea

Abstract excerpt

We report here how the triple combination of drugs elexacaftor/tezacaftor/ivacaftor (ETI) alters the balance of the de-novo synthethic pathway of sphingolipids in primary cells of human bronchial epithelium. The treatment with ETI roughly doubles the levels of dihydrosphingolipids, possibly by modulating the delta(4)-desaturase enzymes that convert dihydroceramides into ceramides. This appears to be an off-target...

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Identifiers

PMID: 37088636
DOI: 10.1016/j.jcf.2023.04.012

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PubMed metadata and Literature Corpus relationships. Corpus snapshot updated 7 Sept 2026. Manifest publication-graph-cohort-2026-09-07.1.

Source receipt: pubmed, retrieved 7 Sept 2026.

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