Altered phospholipid molecular species and glycolipid composition in brain, liver and fibroblasts of Zellweger syndrome.
Neuroscience Letters(2013)
摘要
•We analyzed the lipid molecular species of five patients’ tissues with Zellweger syndrome.•The relative amount of sphingomyelin with shorter chain fatty acid was slightly increased in patients’ cerebellum.•Increase of glycolipids was confirmed in Zellweger syndrome.•ACOX1 and GNPAT RNAi study suggested that both of them could modulate glycolipid metabolism in neuronal cells.
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关键词
ZS,DBP,PE,VLCFA,PC,ACOX1,GNPAT,CNS,CMH,CDH,CTH,UGCG,UGT8,G6PDH,PBS
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