Very long chain fatty acids in X-linked adrenoleukodystrophy brain after treatment with Lorenzo's oil.
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.
Poulos
A
A
Department of Chemical Pathology, Women's and Children's Hospital, North Adelaide, Australia.
Gibson
R
R
Sharp
P
P
Beckman
K
K
Grattan-Smith
P
P
D000293:Adolescent; D000326:Adrenoleukodystrophy / Q000188:drug therapy* / Q000235:genetics / Q000378:metabolism*; D001923:Brain Chemistry / Q000187:drug effects*; D002648:Child; D004338:Drug Combinations; D004883:Erucic Acids / Q000627:therapeutic use*; D005227:Fatty Acids / Q000378:metabolism*; D005260:Female; D008040:Genetic Linkage; D006801:Humans; D008297:Male; D008875:Middle Aged; D014304:Triolein / Q000627:therapeutic use*; D014960:X Chromosome
D004338:Drug Combinations; D004883:Erucic Acids; D005227:Fatty Acids; C079420:Lorenzo's oil; D014304:Triolein