title: Phosphatidyl ethanolamine with increased polyunsaturated fatty acids in compensation for plasmalogen defect in the Zellweger syndrome brain.
abstract: To elucidate the neuropathological mechanism of Zellweger syndrome (ZS), we studied changes in the molecular species of glycerophospholipids in the cerebral tissue by thin-layer chromatography (TLC) and fast atom bombardment mass spectrometry (FABMS). First, we estimated the amount of plasmalogens by TLC. Plasmalogen-type phosphatidyl ethanolamine (PE) accounted for 30% of the total PE in the control brain, but was absent in the ZS brain. Plasmalogen-type phosphatidyl choline (PC) was undetectable in both control and ZS brains. Next, we analyzed plasmalogen-type PE by FABMS. Oleic (18:1), arachidonic (20:4) and docosapentanoic (22:5) acids were present in the control gray matter, but not in the ZS gray matter. In compensation for the defect of plasmalogen, the level of diacyl PE with polyunsaturated fatty acids, 20:4, 22:4, 22:5 and 22:6, was higher in the ZS brain than that in the control brain. These results indicate an alteration in the molecular species of PE, which may cause abnormal neural membrane fluidity and excessive vulnerability to oxygen stress.
authors.affiliation: Department of Developmental Medical Sciences, Graduate School of Medicine, The University of Tokyo, Hongo 7-3-1, Bunkyo-ku, Tokyo 113-8655, Japan. makisaito-tky@umin.ac.jp
authors.forename: Makiko
authors.initials: M
authors.lastname: Saitoh
authors.forename: Masayuki
authors.initials: M
authors.lastname: Itoh
authors.forename: Sachio
authors.initials: S
authors.lastname: Takashima
authors.forename: Masashi
authors.initials: M
authors.lastname: Mizuguchi
authors.forename: Masao
authors.initials: M
authors.lastname: Iwamori
mesh_terms: D001921:Brain / Q000378:metabolism*; D002855:Chromatography, Thin Layer / Q000379:methods; D005231:Fatty Acids, Unsaturated / Q000378:metabolism*; D005260:Female; D006801:Humans; D007223:Infant; D008297:Male; D010714:Phosphatidylethanolamines / Q000032:analysis*; D010955:Plasmalogens / Q000032:analysis*; D016339:Spectrometry, Mass, Fast Atom Bombardment / Q000379:methods; D015211:Zellweger Syndrome / Q000473:pathology*
chemical_list: D005231:Fatty Acids, Unsaturated; D010714:Phosphatidylethanolamines; D010955:Plasmalogens