Potentiator ivacaftor abrogates pharmacological correction of ΔF508 CFTR in cystic fibrosis
DM Cholon, NL Quinney, ML Fulcher… - Science translational …, 2014 - science.org
Cystic fibrosis (CF) is caused by mutations in the CF transmembrane conductance regulator
(CFTR). Newly developed “correctors” such as lumacaftor (VX-809) that improve CFTR …
(CFTR). Newly developed “correctors” such as lumacaftor (VX-809) that improve CFTR …
[HTML][HTML] Clinical development of triple-combination CFTR modulators for cystic fibrosis patients with one or two F508del alleles
JL Taylor-Cousar, MA Mall, BW Ramsey… - ERJ open …, 2019 - Eur Respiratory Soc
Cystic fibrosis (CF) is caused by mutations in the CF transmembrane conductance regulator
gene (CFTR) that result in diminished quantity and/or function of the CFTR anion channel …
gene (CFTR) that result in diminished quantity and/or function of the CFTR anion channel …
Some gating potentiators, including VX-770, diminish ΔF508-CFTR functional expression
G Veit, RG Avramescu, D Perdomo… - Science translational …, 2014 - science.org
Cystic fibrosis (CF) is caused by mutations in the CF transmembrane regulator (CFTR) that
result in reduced anion conductance at the apical membrane of secretory epithelia …
result in reduced anion conductance at the apical membrane of secretory epithelia …
Cystic fibrosis transmembrane conductance regulator–modifying medications: the future of cystic fibrosis treatment
RS Pettit - Annals of Pharmacotherapy, 2012 - journals.sagepub.com
OBJECTIVE: To review and evaluate cystic fibrosis transmembrane conductance regulator
(CFTR) modulators for the treatment of cystic fibrosis (CF). DATA SOURCES: Literature was …
(CFTR) modulators for the treatment of cystic fibrosis (CF). DATA SOURCES: Literature was …
Cystic fibrosis transmembrane conductance regulator modulators: precision medicine in cystic fibrosis
EB Burgener, RB Moss - Current opinion in pediatrics, 2018 - journals.lww.com
CFTR modulator therapy targets the protein defective in CF and boosts its function, but the
drug must match mutation pathobiology. Ivacaftor, a CFTR potentiator, was the first …
drug must match mutation pathobiology. Ivacaftor, a CFTR potentiator, was the first …
Lumacaftor and ivacaftor in the management of patients with cystic fibrosis: current evidence and future prospects
K Kuk, JL Taylor-Cousar - Therapeutic advances in …, 2015 - journals.sagepub.com
Cystic fibrosis (CF) is a genetic disorder that causes multiorgan morbidity and premature
death, most commonly from pulmonary dysfunction. Mutations in the CF transmembrane …
death, most commonly from pulmonary dysfunction. Mutations in the CF transmembrane …
Ivacaftor in subjects with cystic fibrosis who are homozygous for the F508del-CFTR mutation
Background Ivacaftor (VX-770) is a cystic fibrosis transmembrane conductance regulator
(CFTR) potentiator that was approved in the United States for the treatment of cystic fibrosis …
(CFTR) potentiator that was approved in the United States for the treatment of cystic fibrosis …
[HTML][HTML] VX-659–tezacaftor–ivacaftor in patients with cystic fibrosis and one or two Phe508del alleles
JC Davies, SM Moskowitz, C Brown… - New england journal …, 2018 - Mass Medical Soc
Background The next-generation cystic fibrosis transmembrane conductance regulator
(CFTR) corrector VX-659, in triple combination with tezacaftor and ivacaftor (VX-659 …
(CFTR) corrector VX-659, in triple combination with tezacaftor and ivacaftor (VX-659 …
[HTML][HTML] Elexacaftor co-potentiates the activity of F508del and gating mutants of CFTR
Trikafta, the combination of elexacaftor (VX-445), tezacaftor (VX-661) and ivacaftor (VX-770),
was approved for therapy of cystic fibrosis (CF) patients with at least one allele of the CFTR …
was approved for therapy of cystic fibrosis (CF) patients with at least one allele of the CFTR …
Targeting a genetic defect: cystic fibrosis transmembrane conductance regulator modulators in cystic fibrosis
N Derichs - European Respiratory Review, 2013 - Eur Respiratory Soc
Cystic fibrosis (CF) is caused by genetic mutations that affect the cystic fibrosis
transmembrane conductance regulator (CFTR) protein. These mutations can impact the …
transmembrane conductance regulator (CFTR) protein. These mutations can impact the …
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