Pharmacodynamics<unk>  is a "potentiator" of CFTR, meaning it increases the probability that the defective channel will be open and allow chloride ions pass through the channel pore. Pharmacokinetics Distribution<unk>  is approximately 99% bound to plasma proteins, primarily to alpha 1-acid glycoprotein and albumin.<unk>  does not bind to human red blood cells. Biotransformation<unk>  is extensively metabolised in humans. In vitro and in vivo data indicate that<unk>  is primarily metabolised by CYP3A. M1 and M6 are the two major metabolites of<unk>  in humans. M1 has approximately one-sixth the potency of<unk>  and is considered pharmacologically active. M6 has less than one-fiftieth the potency of<unk>  and is not considered pharmacologically active. Elimination Following oral administration, the majority of<unk>  (87.8%) is eliminated in the faeces after metabolic conversion. The major metabolites M1 and M6 accounted for approximately 65% of total dose eliminated with 22% as M1 and 43% as M6. There was negligible urinary excretion of<unk>  as unchanged parent. The apparent terminal half-life was approximately 12 hours following a single dose in the fed state. The apparent clearance (CL/F) of<unk>  was similar for healthy subjects and patients with CF. The mean (±SD) of CL/F for the 150 mg dose was 17.3 (8.4) L/h in healthy subjects at steady state.
Ivacaftor
Lifitegrast
Roxadustat
Arformoterol
Formoterol
Zafirlukast
Lumacaftor/ivacaftor
Fluticasone