title: CFTR pharmacology.
abstract: CFTR protein is an ion channel regulated by cAMP-dependent phosphorylation and expressed in many types of epithelial cells. CFTR-mediated chloride and bicarbonate secretion play an important role in the respiratory and gastrointestinal systems. Pharmacological modulators of CFTR represent promising drugs for a variety of diseases. In particular, correctors and potentiators may restore the activity of CFTR in cystic fibrosis patients. Potentiators are also potentially useful to improve mucociliary clearance in patients with chronic obstructive pulmonary disease. On the other hand, CFTR inhibitors may be useful to block fluid and electrolyte loss in secretory diarrhea and slow down the progression of polycystic kidney disease.
authors.affiliation: U.O.C. Genetica Medica, Istituto Giannina Gaslini, Via Gerolamo Gaslini 5, 16147, Genoa, Italy.
authors.forename: Olga
authors.initials: O
authors.lastname: Zegarra-Moran
authors.affiliation: U.O.C. Genetica Medica, Istituto Giannina Gaslini, Via Gerolamo Gaslini 5, 16147, Genoa, Italy. galietta@unige.it.
authors.forename: Luis J V
authors.initials: LJ
authors.lastname: Galietta
mesh_terms: D000818:Animals; D001639:Bicarbonates / Q000378:metabolism; D002712:Chlorides / Q000378:metabolism; D003550:Cystic Fibrosis / Q000188:drug therapy* / Q000235:genetics / Q000378:metabolism; D019005:Cystic Fibrosis Transmembrane Conductance Regulator / Q000819:agonists* / Q000037:antagonists & inhibitors* / Q000235:genetics / Q000378:metabolism; D055808:Drug Discovery / Q000379:methods*; D004847:Epithelial Cells / Q000378:metabolism; D006801:Humans; D009079:Mucociliary Clearance / Q000187:drug effects; D009154:Mutation
chemical_list: D001639:Bicarbonates; D002712:Chlorides; D019005:Cystic Fibrosis Transmembrane Conductance Regulator
keywords: CFTR; Channel blocker; Chloride channel; Cystic fibrosis