Cystic fibrosis and CFTR Cystic fibrosis is an autosomal recessive genetic disorder of the CFTR protein which reduces chloride and sodium ion transport through the cell membrane, causing thicker than normal mucus secretions. The CFTR protein is found in epithelial cells of the lung, liver, pancreas, digestive tract, and reproductive tracts. CFTR has a role in the production of mucus, sweat, and digestive fluids. The thickened mucus can lead to inflammation, respiratory infections, and clogged ducts. Mechanism of action<unk>  is a tridrug treatment in which the medications work together to increase the transport of chloride and sodium ions, and reducing thick mucus production. 440x440px Complex of ivacaftor with CFTR Ivacaftor CFTR channel potentiator Ivacaftor is a selective small-molecule potentiator of the CFTR protein that increases the protein's ability to open chloride channels. Its effectiveness is highly dependent on the amount of CFTR protein at the cell surface and the responsiveness of the mutant CFTR protein. Ivacaftor's primary target is to treat class III CFTR gating mutations like G551D as well as other less common mutations. In the crystalline figure, you can see ivacaftor, shown as a gray ball and stick model on the bottom-right, bound to CFTR docked in a cleft formed by transmembrane helices at the protein-lipid interface. CFTR correctors<unk><unk> <unk>  and<unk>  act as CFTR correctors to repair F508del processing by binding to the CFTR protein to increase the availability of CFTR protein on the cell surface. They work by modulating the position of the CFTR protein into the right position on the cell surface. The combination of increased CFTR protein in the correct position on the cell surface with ivacaftor's potentiation of chloride channel opening results in increased transport of chloride and thinned mucus secretions. Daily recommended dosage of Trikafta. Two combination<unk> ,<unk> , Ivacaftor tablets and one ivacaftor tablet Pharmacokinetics<unk>  is primarily metabolized by CYP3A4/5. This medication should be taken with a high fat meal to improve absorption through the gut. It is excreted as metabolites or unchanged mainly through feces and to a smaller extent urine. The mean effective half-life of<unk> ,<unk> , and ivacaftor is 27.4 hours, 25.1 hours, and 15 hours, respectively.
Elexacaftor/tezacaftor/ivacaftor
Lumacaftor/ivacaftor
Beclometasone/formoterol/glycopyrronium bromide
Lifitegrast
Mometasone/formoterol
Beclometasone/formoterol
Budesonide/formoterol
Aclidinium bromide/formoterol