Temporal regulation of CFTR expression during ovine lung development: Implications for CF gene therapy

Fiona C. Broackes-Carter, Nathalie Mouchel, Deborah Gill, Stephen Hyde, John Bassett, Ann Harris*

*Corresponding author for this work

Research output: Contribution to journalArticlepeer-review

69 Scopus citations


The cystic fibrosis transmembrane conductance regulator (CFTR) protein is a small conductance chloride ion channel that may interact directly with other channels including the epithelial sodium channel (ENaC). CFTR is known to be more abundant in the airway epithelium during the second trimester of human development than after birth. This could be a consequence of the change in function of the respiratory epithelium from chloride secretion to sodium absorption near term. Alternatively it might reflect an additional role for CFTR in the developing airway epithelium. Though the lung epithelia of CF fetuses and infants rarely show gross histological abnormalities, there is often evidence of inflammation. Our aim was to establish whether CFTR expression levels correlated with specific developmental stages or differentiated functions in the ovine fetal lung. We evaluated CFTR expression using a quantitative assay of mRNA at 14 time points through gestation and showed highest levels at the start of the second trimester followed by a gradual decline through to term. In contrast, ENaC expression increased from the start of the third trimester. These results support a role for CFTR in differentiation of the respiratory epithelium and suggest that its expression levels are not merely reflecting major changes in the sodium/chloride bulk flow close to term. These observations may have significant implications for the likely success of CF gene therapy in the postnatal lung.

Original languageEnglish (US)
Pages (from-to)125-131
Number of pages7
JournalHuman molecular genetics
Issue number2
StatePublished - Jan 15 2002

ASJC Scopus subject areas

  • Genetics(clinical)
  • Genetics
  • Molecular Biology


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