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使用洛伦佐油治疗后X连锁肾上腺脑白质营养不良症患者大脑中的极长链脂肪酸

Very long chain fatty acids in X-linked adrenoleukodystrophy brain after treatment with Lorenzo's oil.

作者信息

Poulos A, Gibson R, Sharp P, Beckman K, Grattan-Smith P

机构信息

Department of Chemical Pathology, Women's and Children's Hospital, North Adelaide, Australia.

出版信息

Ann Neurol. 1994 Nov;36(5):741-6. doi: 10.1002/ana.410360509.

DOI:10.1002/ana.410360509
PMID:7979219
Abstract

The fatty acid composition of postmortem brain and liver from an adrenoleukodystrophy patient whose diet was supplemented with Lorenzo's oil (glycerol trioleate and glycerol trierucate) for 9 months was determined. The diet depressed plasma and liver saturated very long chain fatty acids (24:0 and 26:0) and increased plasma and liver erucic (22:1) and nervonic (24:1) acids. The levels of plasma linoleic (18:2 n-6), eicosopentaenoic (20:5 n-3), and docosahexaenoic (22:6 n-3) acids were also reduced, while the biochemical marker for essential fatty acid deficiency (20:3 n-9) was markedly increased in liver. However, we were unable to detect any corresponding changes in brain indicating that little erucic acid crossed the blood-brain barrier. Our findings suggest that dietary supplementation with Lorenzo's oil is of limited value in correcting the accumulation of saturated very long chain fatty acids in the brain of patients with adrenoleukodystrophy.

摘要

对一名肾上腺脑白质营养不良患者进行了研究,该患者在9个月的时间里饮食中添加了洛伦佐油(甘油三油酸酯和甘油三芥酸酯),之后测定了其死后大脑和肝脏的脂肪酸组成。这种饮食降低了血浆和肝脏中饱和极长链脂肪酸(24:0和26:0)的水平,并增加了血浆和肝脏中芥酸(22:1)和神经酸(24:1)的含量。血浆中亚油酸(18:2 n-6)、二十碳五烯酸(20:5 n-3)和二十二碳六烯酸(22:6 n-3)的水平也有所降低,而肝脏中必需脂肪酸缺乏的生化标志物(20:3 n-9)显著增加。然而,我们未能在大脑中检测到任何相应变化,这表明很少有芥酸穿过血脑屏障。我们的研究结果表明,在纠正肾上腺脑白质营养不良患者大脑中饱和极长链脂肪酸的积累方面,饮食中添加洛伦佐油的价值有限。

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