Document Detail

Cell culture models demonstrate that CFTR dysfunction leads to defective fatty acid composition and metabolism.
MedLine Citation:
PMID:  18441018     Owner:  NLM     Status:  MEDLINE    
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 acid metabolism in cultured airway epithelial cells.
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
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Publication Detail:
Type:  Journal Article; Research Support, N.I.H., Extramural; Research Support, Non-U.S. Gov't     Date:  2008-04-25
Journal Detail:
Title:  Journal of lipid research     Volume:  49     ISSN:  0022-2275     ISO Abbreviation:  J. Lipid Res.     Publication Date:  2008 Aug 
Date Detail:
Created Date:  2008-07-14     Completed Date:  2008-09-11     Revised Date:  2009-11-18    
Medline Journal Info:
Nlm Unique ID:  0376606     Medline TA:  J Lipid Res     Country:  United States    
Other Details:
Languages:  eng     Pagination:  1692-700     Citation Subset:  IM    
Division of Gastroenterology, Beth Israel Deaconess Medical Center, Boston, MA 02215, USA.
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MeSH Terms
Antisense Elements (Genetics)
Bronchi / cytology
Cell Count
Cells, Cultured
Culture Media
Cystic Fibrosis / physiopathology
Cystic Fibrosis Transmembrane Conductance Regulator / genetics,  physiology*
Epithelial Cells / metabolism
Fatty Acids / metabolism*
Linoleic Acid / metabolism,  pharmacology
Grant Support
Reg. No./Substance:
0/Antisense Elements (Genetics); 0/CFTR protein, human; 0/Culture Media; 0/Fatty Acids; 126880-72-6/Cystic Fibrosis Transmembrane Conductance Regulator; 2197-37-7/Linoleic Acid

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