[{"from": "human", "value": "Please give me some details about this molecule. [C][C][Branch1][C][C][Branch1][C][C][C][=C][C][=Branch2][Ring1][=C][=C][Branch2][Ring1][=Branch2][C][=C][Ring1][=Branch1][N][C][=Branch1][C][=O][C][=C][N][C][=C][C][=C][C][=C][Ring1][=Branch1][C][Ring1][#Branch2][=O][O][C][Branch1][C][C][Branch1][C][C][C]"}, {"from": "gpt", "value": "The molecule is a drug used for the management of Cystic Fibrosis (CF). It is manufactured and distributed by Vertex Pharmaceuticals. It was approved by the Food and Drug Administration on January 31, 2012, and by Health Canada in late 2012.  Ivacaftor is administered as a monotherapy and also administered in combination with other drugs for the management of CF.  Cystic Fibrosis is an autosomal recessive disorder caused by one of several different mutations in the gene for the Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) protein, an ion channel involved in the transport of chloride and sodium ions across cell membranes. CFTR is active in epithelial cells of organs such as of the lungs, pancreas, liver, digestive system, and reproductive tract. Alterations in the CFTR gene result in altered production, misfolding, or function of the protein and consequently abnormal fluid and ion transport across cell membranes. As a result, CF patients produce thick, sticky mucus that clogs the ducts of organs where it is produced making patients more susceptible to complications such as infections, lung damage, pancreatic insufficiency, and malnutrition.  Prior to the development of ivacaftor, management of CF primarily involved therapies for the control of infections, nutritional support, clearance of mucus, and management of symptoms rather than improvements in the underlying disease process or lung function (FEV1). Notably, ivacaftor was the first medication approved for the management of the underlying causes of CF (abnormalities in CFTR protein function) rather than control of symptoms."}]