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 …

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 …

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 …

OBJECTIVE: To review and evaluate cystic fibrosis transmembrane conductance regulator
(CFTR) modulators for the treatment of cystic fibrosis (CF). DATA SOURCES: Literature was …

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 …

Cystic fibrosis (CF) is a genetic disorder that causes multiorgan morbidity and premature
death, most commonly from pulmonary dysfunction. Mutations in the CF transmembrane …

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 …

Background The next-generation cystic fibrosis transmembrane conductance regulator
(CFTR) corrector VX-659, in triple combination with tezacaftor and ivacaftor (VX-659 …

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 …

Cystic fibrosis (CF) is caused by genetic mutations that affect the cystic fibrosis
transmembrane conductance regulator (CFTR) protein. These mutations can impact the …