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Tezacaftor is a direct inhibitor of sphingolipid delta-4 desaturase enzyme (DEGS).
Ciobanu DZ, Liessi N, Tomati V, Capurro V, Bertozzi SM, Summa M, Bertorelli R, Loberto N, Dobi D, Aureli M, Nobbio L, Bandiera T, Pedemonte N, Bassi R, Armirotti A. Ciobanu DZ, et al. Among authors: capurro v. J Cyst Fibros. 2024 Nov;23(6):1167-1172. doi: 10.1016/j.jcf.2024.05.004. Epub 2024 May 24. J Cyst Fibros. 2024. PMID: 38789319 Free article.
Rescue by elexacaftor-tezacaftor-ivacaftor of the G1244E cystic fibrosis mutation's stability and gating defects are dependent on cell background.
Tomati V, Costa S, Capurro V, Pesce E, Pastorino C, Lena M, Sondo E, Di Duca M, Cresta F, Cristadoro S, Zara F, Galietta LJV, Bocciardi R, Castellani C, Lucanto MC, Pedemonte N. Tomati V, et al. Among authors: capurro v. J Cyst Fibros. 2023 May;22(3):525-537. doi: 10.1016/j.jcf.2022.12.005. Epub 2022 Dec 19. J Cyst Fibros. 2023. PMID: 36543707
The L467F-F508del Complex Allele Hampers Pharmacological Rescue of Mutant CFTR by Elexacaftor/Tezacaftor/Ivacaftor in Cystic Fibrosis Patients: The Value of the Ex Vivo Nasal Epithelial Model to Address Non-Responders to CFTR-Modulating Drugs.
Sondo E, Cresta F, Pastorino C, Tomati V, Capurro V, Pesce E, Lena M, Iacomino M, Baffico AM, Coviello D, Bandiera T, Zara F, Galietta LJV, Bocciardi R, Castellani C, Pedemonte N. Sondo E, et al. Among authors: capurro v. Int J Mol Sci. 2022 Mar 15;23(6):3175. doi: 10.3390/ijms23063175. Int J Mol Sci. 2022. PMID: 35328596 Free PMC article.
45 results