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Molecular species compositions of lung and pancreas phospholipids in the cftr(tm1HGU/tm1HGU) cystic fibrosis mouse

Dombrowsky, Heike, Clark, Graeme T., Rau, Gunnar A., Bernhard, Wolfang and Postle, Anthony D. (2003) Molecular species compositions of lung and pancreas phospholipids in the cftr(tm1HGU/tm1HGU) cystic fibrosis mouse Pediatric Research, 53, (3), pp. 447-454. (doi:10.1203/01.PDR.0000049937.30305.8A).

Record type: Article


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 (22:6n-3) and increased arachidonate (20:4n-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 cftrtm1HGU/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 22:6n-3 in any phospholipid class from lung, pancreas, or liver from mice with the cftrtm1HGU/tm1HGU. Instead, the concentration of 20:4n-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 cftrtm1HGU/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 22:6n-3–containing oils to children with CF can be fully evaluated.

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Published date: 2003


Local EPrints ID: 27026
ISSN: 0031-3998
PURE UUID: b0bffef3-8fa3-4f23-9d01-bd0f3feb919b
ORCID for Anthony D. Postle: ORCID iD

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Date deposited: 26 Apr 2006
Last modified: 17 Jul 2017 16:05

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Author: Heike Dombrowsky
Author: Graeme T. Clark
Author: Gunnar A. Rau
Author: Wolfang Bernhard

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