Altered phospholipid molecular species and glycolipid composition in brain, liver and fibroblasts of Zellweger syndrome

Celine Miyazaki, Makiko Saitoh, Masayuki Itoh, Sumimasa Yamashita, Makoto Miyagishi, Sachio Takashima, Ann B. Moser, Masao Iwamori, Masashi Mizuguchi

Research output: Contribution to journalArticle

Abstract

We studied the altered molecular species of lipids in brain and liver tissues, and fibroblasts from patients with Zellweger syndrome (ZS). ZS cerebellum samples contained a higher amount of sphingomyelin with shorter chain fatty acids compared to that in normal controls. The amount of phosphatidylethanolamine (PE) was less than half of that in controls, with the absence of the PE-type of plasmalogen. Gangliosides were accumulated in the brains and fibroblasts of ZS patients. To investigate whether or not impaired beta-oxidation of very long chain fatty acids and/or plasmalogen synthesis affects glycolipids metabolism, RNAi of peroxisomal acylCo-A oxidase (ACOX1) and glyceronephosphate O-acyltransferase (GNPAT) was performed using cultured neural cells. In neuronal F3-Ngn1 cells, ACOX1 and GNPAT silencing up-regulated ceramide galactosyltransferase (UGT8) mRNA expression, and down-regulated UDP-glucose ceramide glucosyltransferase (UGCG). These results suggest that both impaired beta-oxidation of very long chain fatty acids and plasmalogen synthesis affect glycolipid metabolism in neuronal cells.

Original languageEnglish (US)
Pages (from-to)71-75
Number of pages5
JournalNeuroscience Letters
Volume552
DOIs
StatePublished - Sep 27 2013

Keywords

  • Ganglioside
  • Peroxisome
  • Plasmalogen
  • RNAi
  • Sphingomyelin
  • Zellweger syndrome

ASJC Scopus subject areas

  • Neuroscience(all)

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  • Cite this

    Miyazaki, C., Saitoh, M., Itoh, M., Yamashita, S., Miyagishi, M., Takashima, S., Moser, A. B., Iwamori, M., & Mizuguchi, M. (2013). Altered phospholipid molecular species and glycolipid composition in brain, liver and fibroblasts of Zellweger syndrome. Neuroscience Letters, 552, 71-75. https://doi.org/10.1016/j.neulet.2013.07.045