Phosphatidyl ethanolamine with increased polyunsaturated fatty acids in compensation for plasmalogen defect in the Zellweger syndrome brain

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• 作者：Makiko Saitoh ; Masayuki Itoh ; Sachio Takashima ; Masashi Mizuguchi ; Masao Iwamori
• 关键词：Peroxisome ; Zellweger syndrome ; Plasmalogen ; Phosphatidyl ethanolamine ; Polyunsaturated fatty acid
• 刊名：Neuroscience Letters
• 出版年：2009
• 出版时间：16 January 2009
• 年：2009
• 卷：449
• 期：3
• 页码：164-167
• 全文大小：350 K

文摘

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.