Very long chain fatty acids in X-linked adrenoleukodystrophy brain after treatment with Lorenzo's oil.
Ann Neurol
; 36(5): 741-6, 1994 Nov.
Article
em En
| MEDLINE
| ID: mdl-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 (240 and 260) and increased plasma and liver erucic (221) and nervonic (241) acids. The levels of plasma linoleic (182 n-6), eicosopentaenoic (205 n-3), and docosahexaenoic (226 n-3) acids were also reduced, while the biochemical marker for essential fatty acid deficiency (203 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.
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Base de dados:
MEDLINE
Assunto principal:
Cromossomo X
/
Química Encefálica
/
Trioleína
/
Ácidos Erúcicos
/
Adrenoleucodistrofia
/
Ácidos Graxos
/
Ligação Genética
Idioma:
En
Revista:
Ann Neurol
Ano de publicação:
1994
Tipo de documento:
Article
País de afiliação:
Austrália