Results 111 to 120 of about 12,467 (245)

Ivacaftor attenuates gentamicin-induced ototoxicity through the CFTR-Nrf2-HO1/NQO1 pathway

Redox Report
Objectives Gentamicin is one of the most common ototoxic drugs that can lower patients' quality of life. Oxidative stress is a key factors inducing sensory hair cell death during gentamicin administration.
Rui Hu, Fan Wu, Yi-Qing Zheng
doaj   +1 more source

Impact of the CFTR-potentiator ivacaftor on airway microbiota in cystic fibrosis patients carrying a G551D mutation.

PLoS ONE, 2015
BackgroundAirway microbiota composition has been clearly correlated with many pulmonary diseases, and notably with cystic fibrosis (CF), an autosomal genetic disorder caused by mutation in the CF transmembrane conductance regulator (CFTR).
Cédric Bernarde, Marlène Keravec, Jérôme Mounier, Stéphanie Gouriou, Gilles Rault, Claude Férec, Georges Barbier, Geneviève Héry-Arnaud   +7 more
doaj   +1 more source

The potentiator ivacaftor is essential for pharmacological restoration of F508del-CFTR function and mucociliary clearance in cystic fibrosis

JCI Insight
Pharmacological rescue of F508del-CFTR by the triple combination CFTR modulator therapy elexacaftor/tezacaftor/ivacaftor (ETI) leads to unprecedented clinical benefits in patients with cystic fibrosis (CF).
Anita Balázs, Tihomir Rubil, Christine K. Wong, Jasmin Berger, Marika Drescher, Kathrin Seidel, Mirjam Stahl, Simon Y. Graeber, Marcus A. Mall   +8 more
doaj   +1 more source

P080 Improved clinical outcomes following ivacaftor treatment in a cystic fibrosis patient homozygous for 3272–26A>G variant [PDF]

, 2023
Jasna Rodman Berlot, Malena Aldeco, Dušanka Lepej, M. Praprotnik, S. Šetina Šmid, Aleksandra Zver, Uroš Krivec   +6 more
openalex   +1 more source

Ivacaftor pharmacokinetics and lymphatic transport after enteral administration in rats

Frontiers in Pharmacology
Background: Ivacaftor is a modern drug used in the treatment of cystic fibrosis. It is highly lipophilic and exhibits a strong positive food effect.
Jiří Pozniak, Pavel Ryšánek, David Smrčka, Petr Kozlík, Tomáš Křížek, Jaroslava Šmardová, Anežka Nováková, Debanjan Das, Daniel Bobek, Mahak Arora, Jiří Hofmann, Tereza Doušová, Martin Šíma, Ondřej Slanař   +13 more
doaj   +1 more source

Cost-Effectiveness of Ivacaftor Therapy for Treatment of Cystic Fibrosis Patients With the G551D Gating Mutation [PDF]

, 2020
Kael Wherry, Ian O. Williamson, Richard H. Chapman, Karen M. Kuntz   +3 more
openalex   +1 more source

Molecular basis of cystic fibrosis and modern therapeutic approaches [PDF]

, 2019
Identifikacija molekularne povezanosti između genske nefunkcionalnosti CFTR gena te patofiziološke i kliničke pozadine cistične fibroze omogućila je razvoj suvremenih terapijskih modela koji su primarno utemeljeni na individualiziranom pristupu ...
Dragičević, Dorian
core   +2 more sources

Impact of Elexacaftor/Tezacaftor/Ivacaftor on Cystic Fibrosis Liver Tests in a Real-World Cohort with and Without Baseline CFLD [PDF]

, 2021
Megan E. Bowen, E. Sample, Ashley Collins, A. Singhal, Alison Brown   +4 more
openalex   +1 more source

Impact of lumacaftor/ivacaftor on the bacterial and fungal respiratory pathogens in cystic fibrosis: a prospective multicenter cohort study in Sweden

Therapeutic Advances in Respiratory Disease
Background: A significant decline in pulmonary exacerbation rates has been reported in CF patients homozygous for F508del treated with lumacaftor/ivacaftor. However, it is still unclear whether this reduction reflects a diminished microbiological burden.
Mahasin Al Shakirchi, Kimmo Sorjonen, Lena Hjelte, Lena Klingspor, Peter Bergman, Petrea Ericson, Marcus Svedberg, Ulrika Lindberg, Christine Hansen, Isabelle de Monestrol   +9 more
doaj   +1 more source

CFTR and ENaC functions in cystic fibrosis [PDF]

, 2014
La fibrosis quística se debe a la ausencia o defecto del canal transmembrana regulador de la fibrosis quística (CFTR), un canal de cloruro codificado en el gen cftr que juega un papel clave en la homeostasis del agua e iones.
Kotsias, Basilio Aristides, Marino, Gabriela Inés, Palma, Alejandra Graciela   +2 more
core