Document Detail

Molecular species compositions of lung and pancreas phospholipids in the cftr(tm1HGU/tm1HGU) cystic fibrosis mouse.
MedLine Citation:
PMID:  12595593     Owner:  NLM     Status:  MEDLINE    
Fatty acid analysis of phospholipid compositions of lung and pancreas cells from a cystic fibrosis transmembrane regulator (CFTR) negative mouse (cftr(-/-))suggested that a decreased concentration of docosahexaenoate (22:6(n-3)) and increased arachidonate (20:4(n-6)) may be related to the disease process in cystic fibrosis (CF). Consequently, we have determined compositions of the major phospholipids of lung, pancreas, liver, and plasma from a different mouse model of CF, the cftr(tm1HGU/tm1HGU) mouse, compared with ZTM:MF-1 control mice. Electrospray ionization mass spectrometry permitted the quantification of all of the individual molecular species of phosphatidylcholine (PtdCho), phosphatidylethanolamine (PtdEtn), phosphatidylglycerol (PtdGly), phosphatidylserine (PtdSer), and phosphatidylinositol (PtdIns). There was no deficiency of 22:6(n-3) in any phospholipid class from lung, pancreas, or liver from mice with the cftr(tm1HGU/tm1HGU). Instead, the concentration of 20:4(n-6) was significantly decreased in plasma PtdCho species and in pancreas and lung species of PtdEtn, PtdSer, and PtdIns. These results demonstrate the variability of membrane phospholipid compositions in different mouse models of CF and suggest that in cftr(tm1HGU/tm1HGU) mice, the apparent deficiency was of 20:4n-6- rather than of 22:6n-3-containing phospholipid species. They highlight a need for detailed phospholipid molecular species analysis of cells expressing mutant CFTR from children with CF before the therapeutic effects of administering high doses of 22:6(n-3)-containing oils to children with CF can be fully evaluated.
Heike Dombrowsky; Graeme T Clark; Gunnar A Rau; Wolfgang Bernhard; Anthony D Postle
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Publication Detail:
Type:  Journal Article; Research Support, Non-U.S. Gov't    
Journal Detail:
Title:  Pediatric research     Volume:  53     ISSN:  0031-3998     ISO Abbreviation:  Pediatr. Res.     Publication Date:  2003 Mar 
Date Detail:
Created Date:  2003-02-21     Completed Date:  2003-08-27     Revised Date:  2006-11-15    
Medline Journal Info:
Nlm Unique ID:  0100714     Medline TA:  Pediatr Res     Country:  United States    
Other Details:
Languages:  eng     Pagination:  447-54     Citation Subset:  IM    
Department of Child Health, Division of Infection, Inflammation and Repair, School of Medicine, University of Southampton, Southampton, SO16 6YD, United Kingdom.
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MeSH Terms
Cell Membrane / chemistry
Cystic Fibrosis / metabolism*
Cystic Fibrosis Transmembrane Conductance Regulator / genetics*
Lung / chemistry*
Mass Spectrometry
Mice, Inbred CFTR
Pancreas / chemistry*
Phospholipids / analysis*
Specific Pathogen-Free Organisms
Reg. No./Substance:
0/Phospholipids; 126880-72-6/Cystic Fibrosis Transmembrane Conductance Regulator

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