Expression of the cystic fibrosis gene in human development

Ann Harris*, Gillian Chalkley, Shelley Goodman, Lindsay Coleman

*Corresponding author for this work

Research output: Contribution to journalArticlepeer-review

80 Scopus citations

Abstract

The specialised epithelia lining the respiratory tract, pancreatic ducts, male genital ducts and sweat gland ducts are defective in the severe inherited disease, cystic fibrosis (CF). We have looked at the expression of the CF gene in human fetal tissues to throw light on the development of function in specialised ductal epithelia and to determine the age of onset of the CF disease process. The CF gene is already seen to be transcribed in mid-trimester fetal lung, pancreas and male genital ducts. Hence, by this developmental stage, and before they are fully differentiated, these epithelia have the capability to perform important transport functions. Epithelial cell cultures derived from fetal pancreas and male genital ducts maintain expression of the CF gene in vitro and so form good models for analysing CF gene function and differentiation of these specialised epithelia.

Original languageEnglish (US)
Pages (from-to)305-310
Number of pages6
JournalDevelopment
Volume113
Issue number1
StatePublished - Sep 1991

Keywords

  • Cystic fibrosis
  • Development
  • Ductal epithelium

ASJC Scopus subject areas

  • Molecular Biology
  • Developmental Biology

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