VERY LONG-CHAIN FATTY-ACIDS IN X-LINKED ADRENOLEUKODYSTROPHY BRAIN AFTER TREATMENT WITH LORENZOS OIL

被引：59

作者：

POULOS, A

GIBSON, R

SHARP, P

BECKMAN, K

GRATTANSMITH, P

机构：

[1] FLINDERS MED CTR,DEPT PEDIAT,ADELAIDE,SA,AUSTRALIA

[2] WESTMEAD HOSP,DEPT PAEDIAT,WESTMEAD,NSW 2145,AUSTRALIA

来源：

ANNALS OF NEUROLOGY | 1994年 / 36卷 / 05期

关键词：

D O I：

10.1002/ana.410360509

中图分类号：

R74 [神经病学与精神病学];

学科分类号：

摘要：

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-3) 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.

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页码：741 / 746

页数：6

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