Cystic fibrosis transmembrane conductance regulator is expressed in Mucin granules from calu-3 and primary human airway epithelial cells

Pierre Le Simple, Julie Goepp, Melissa L Palmer, Scott C. Fahrenkrug, Scott M O'Grady, Pasquale Ferraro, Renaud Robert, John W. Hanrahan

Research output: Contribution to journalArticle

11 Citations (Scopus)

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.

Original languageEnglish (US)
Pages (from-to)511-516
Number of pages6
JournalAmerican journal of respiratory cell and molecular biology
Volume49
Issue number4
DOIs
StatePublished - Oct 1 2013

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Cystic Fibrosis Transmembrane Conductance Regulator
Mucins
Mucus
Epithelial Cells
Cystic Fibrosis
Primary Cell Culture
Pathology
Airway Obstruction
Bicarbonates
Dehydration
Cell culture
Anions
Gels
Cell Line
Defects
Mutation

Keywords

  • CFTR
  • Cystic fibrosis
  • Mucus

Cite this

Cystic fibrosis transmembrane conductance regulator is expressed in Mucin granules from calu-3 and primary human airway epithelial cells. / Le Simple, Pierre; Goepp, Julie; Palmer, Melissa L; Fahrenkrug, Scott C.; O'Grady, Scott M; Ferraro, Pasquale; Robert, Renaud; Hanrahan, John W.

In: American journal of respiratory cell and molecular biology, Vol. 49, No. 4, 01.10.2013, p. 511-516.

Research output: Contribution to journalArticle

Le Simple, Pierre ; Goepp, Julie ; Palmer, Melissa L ; Fahrenkrug, Scott C. ; O'Grady, Scott M ; Ferraro, Pasquale ; Robert, Renaud ; Hanrahan, John W. / Cystic fibrosis transmembrane conductance regulator is expressed in Mucin granules from calu-3 and primary human airway epithelial cells. In: American journal of respiratory cell and molecular biology. 2013 ; Vol. 49, No. 4. pp. 511-516.
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