title: Cystic fibrosis transmembrane conductance regulator is expressed in mucin granules from Calu-3 and primary human airway epithelial cells.
abstract: Cystic fibrosis (CF) is caused by mutations in the tightly regulated anion channel cystic fibrosis transmembrane conductance regulator (CFTR), yet much of the pathology in this disease results from mucus obstruction of the small airways and other organs. Mucus stasis has been attributed to the abnormal luminal environment of CF airways, which results from dehydration of the mucus gel or low bicarbonate concentration. We show here that CFTR and MUC5AC are present in single mucin-containing granules isolated from a human airway epithelial cell line and from highly differentiated airway primary cell cultures. CFTR was not detected in MUC5AC granules from CFTR knockdown cells or CF primary cells. The results suggest a direct link between CFTR and the mucus defect.
authors.affiliation: 1 Department of Physiology, McGill University, Montreal, Quebec, Canada.
authors.forename: Pierre
authors.initials: P
authors.lastname: LeSimple
authors.forename: Julie
authors.initials: J
authors.lastname: Goepp
authors.forename: Melissa L
authors.initials: ML
authors.lastname: Palmer
authors.forename: Scott C
authors.initials: SC
authors.lastname: Fahrenkrug
authors.forename: Scott M
authors.initials: SM
authors.lastname: O'Grady
authors.forename: Pasquale
authors.initials: P
authors.lastname: Ferraro
authors.forename: Renaud
authors.initials: R
authors.lastname: Robert
authors.forename: John W
authors.initials: JW
authors.lastname: Hanrahan
mesh_terms: D002460:Cell Line; D003550:Cystic Fibrosis / Q000235:genetics / Q000378:metabolism*; D019005:Cystic Fibrosis Transmembrane Conductance Regulator / Q000096:biosynthesis* / Q000235:genetics / Q000378:metabolism; D004847:Epithelial Cells / Q000378:metabolism*; D006801:Humans; D055271:Mucin 5AC / Q000235:genetics / Q000378:metabolism; D009077:Mucins / Q000235:genetics / Q000378:metabolism*; D020545:Respiratory Mucosa / Q000166:cytology / Q000378:metabolism*
chemical_list: C505032:CFTR protein, human; C524678:MUC5AC protein, human; D055271:Mucin 5AC; D009077:Mucins; D019005:Cystic Fibrosis Transmembrane Conductance Regulator