Document Detail

Analysis of phospholipid molecular species in brains from patients with infantile and juvenile neuronal-ceroid lipofuscinosis using liquid chromatography-electrospray ionization mass spectrometry.
MedLine Citation:
PMID:  12603829     Owner:  NLM     Status:  MEDLINE    
Phospholipids (PL) in cerebral cortex from patients with infantile (INCL or CLN1) and juvenile (JNCL or CLN3) forms of neuronal ceroid-lipofuscinosis (NCL) and controls were analysed by normal phase HPLC and on-line electrospray ionization ion-trap mass spectrometric detection (LC-ESI-MS). The method provided quantitative data on numerous molecular species of different PL classes, which are not achieved by using the conventional chromatographic methods. Compared with the controls, the INCL brains contained proportionally more phosphatidylcholine (PC), and less phosphatidylethanolamine (PE) and phosphatidylserine (PS). Different molecular species of PC, PE, PS, phosphatidylinositol and sphingomyelin were quantified using multiple internal PL standards that differed in fatty acyl chain length and thus allowed correction for chain length dependency of instrument response. In INCL cortex, which had lost 65% of the normal PL content, the proportions of polyunsaturated molecular species, especially the PS and PE that contained docosahexaenoic acid (22:6n-3), were dramatically decreased. The membranes may have adapted to this alteration by increasing the proportions of PL molecules substituted with monounsaturated and short-chain fatty acids. Lysobisphosphatidic acid was highly elevated in the INCL brain and consisted mostly of polyunsaturated species. It is possible that changes in the composition of PL membranes accelerate progression of INCL by altering signalling and membrane trafficking in neurons.
Reijo Käkelä; Pentti Somerharju; Jaana Tyynelä
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Publication Detail:
Type:  Journal Article; Research Support, Non-U.S. Gov't    
Journal Detail:
Title:  Journal of neurochemistry     Volume:  84     ISSN:  0022-3042     ISO Abbreviation:  J. Neurochem.     Publication Date:  2003 Mar 
Date Detail:
Created Date:  2003-02-26     Completed Date:  2003-03-19     Revised Date:  2006-11-15    
Medline Journal Info:
Nlm Unique ID:  2985190R     Medline TA:  J Neurochem     Country:  England    
Other Details:
Languages:  eng     Pagination:  1051-65     Citation Subset:  IM    
Institute of Biomedicine/Biochemistry, Biomedicum Helsinki, University of Helsinki, Haartmaninkatu 8, 00014 Helsinki, Finland.
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MeSH Terms
Brain Chemistry
Cerebral Cortex / chemistry*,  metabolism*
Chromatography, High Pressure Liquid
Disease Progression
Lysophospholipids / analysis
Neuronal Ceroid-Lipofuscinoses / metabolism*
Phosphatidylcholines / analysis
Phosphatidylethanolamines / analysis
Phosphatidylglycerols / analysis
Phosphatidylinositols / analysis
Phosphatidylserines / analysis
Phospholipids / analysis*
Reference Values
Spectrometry, Mass, Electrospray Ionization
Sphingomyelins / analysis
Reg. No./Substance:
0/Lysophospholipids; 0/Monoglycerides; 0/Phosphatidylcholines; 0/Phosphatidylethanolamines; 0/Phosphatidylglycerols; 0/Phosphatidylinositols; 0/Phosphatidylserines; 0/Phospholipids; 0/Sphingomyelins; 0/bis(monoacylglyceryl)phosphate

From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine

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