Cell culture models demonstrate that CFTR dysfunction leads to defective fatty acid composition and metabolism

Charlotte Andersson, M. Rabie Al-Turkmani, Juanito E. Savaille, Ragheed Alturkmani, Waddah Katrangi, Joanne E. Cluette-Brown, Munir M. Zaman, Michael Laposata, Steven D. Freedman

Research output: Contribution to journalArticlepeer-review

54 Scopus citations


Cystic fibrosis (CF) is associated with fatty acid alterations characterized by low linoleic and docosahexaenoic acid. It is not clear whether these fatty acid alterations are directly linked to cystic fibrosis transmembrane conductance regulator (CFTR) dysfunction or result from nutrient malabsorption. We hypothesized that if fatty acid alterations are a result of CFTR dysfunction, those alterations should be demonstrable in CF cell culture models. Two CF airway epithelial cell lines were used: 16HBE, sense and antisense CFTR cells, and C38/IB3-1 cells. Wild-type (WT) and CF cells were cultured in 10% fetal bovine serum (FBS) or 10% horse serum. Fatty acid levels were analyzed by GC-MS. Culture of both WT and CF cells in FBS resulted in very low linoleic acid levels. When cells were cultured in horse serum containing concentrations of linoleic acid matching those found in human plasma, physiological levels of linoleic acid were obtained and fatty acid alterations characteristic of CF tissues were then evident in CF compared with WT cells. Kinetic studies with radiolabeled linoleic acid demonstrated in CF cells increased conversion to longer and more-desaturated fatty acids such as arachidonic acid. In conclusion, these data demonstrate that CFTR dysfunction is associated with altered fatty acidmetabolism in cultured airway epithelial cells.

Original languageEnglish (US)
Pages (from-to)1692-1700
Number of pages9
JournalJournal of Lipid Research
Issue number8
StatePublished - Aug 2008
Externally publishedYes


  • Arachidonic acid
  • Confluence
  • Cystic fibrosis transmembrane conductance regulator
  • D6-desaturase
  • Docosahexaenoic acid
  • Essential fatty acid deficiency
  • Fetal bovine serum
  • Horse serum

ASJC Scopus subject areas

  • Biochemistry
  • Endocrinology
  • Cell Biology


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