European journal of clinical investigation
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Eur. J. Clin. Invest. · Nov 2000
Phospholipid composition in late infantile neuronal ceroid lipofuscinosis.
Neuronal ceroid lipofuscinosis (NCL) is a relatively common group of inherited neurodegenerative disorders characterised by the accumulation of autofluorescent lipopigments (ceroid) similar to lipofuscin. Because of this property, studies have concentrated on fatty acid metabolism and lipid peroxidation. ⋯ The molecular composition of PC and PE subclasses in skin fibroblasts of healthy subjects and patients suffering from LINCL is here described for the first time. While few differences are noticeable in the fatty acid composition of PC and PE and the molecular species distribution of diacylGPC and diacylGPE, the alkenylacyl GPE (or ethanolamine plasmalogens) were found to differ significantly between patients and healthy controls.