Brain, liver, and adipose tissue erucic and very long chain fatty acid levels in adrenoleukodystrophy patients treated with glyceryl trierucate and trioleate oils (Lorenzo's oil).
Brain, liver, and adipose lipids were studied in the postmortem tissues of four adrenoleukodystrophy patients who had been treated with a mixture of glyceryl trioleate and trierucate oils ("Lorenzo's Oil") and compared to 7 untreated ALD patients and 3 controls. The dietary therapy appeared to reduce the levels of saturated very long chain fatty acids in the plasma, adipose tissue and liver; in the brain they were reduced in only one of the four patients. While substantial amounts of erucic acid were present in some of the tissues even 12 months after therapy had been discontinued, the levels in brain did not exceed those in controls at any time. The failure of erucic acid to enter the brain in significant quantity may be a factor in the disappointing results of dietary therapy for adrenoleukodystrophy.
Rasmussen
M
M
Kennedy Krieger Institute, Department of Neurogenetics, Baltimore, MD 21205.
Moser
A B
AB
Borel
J
J
Khangoora
S
S
Moser
H W
HW
D000273:Adipose Tissue / Q000187:drug effects* / Q000378:metabolism; D000293:Adolescent; D000326:Adrenoleukodystrophy / Q000178:diet therapy* / Q000378:metabolism; D000328:Adult; D001921:Brain / Q000187:drug effects* / Q000378:metabolism; D002648:Child; D002675:Child, Preschool; D004042:Dietary Fats, Unsaturated / Q000494:pharmacology*; D004338:Drug Combinations; D004883:Erucic Acids / Q000378:metabolism / Q000494:pharmacology*; D005227:Fatty Acids / Q000737:chemistry / Q000378:metabolism; D006801:Humans; D008099:Liver / Q000187:drug effects* / Q000378:metabolism; D014304:Triolein / Q000494:pharmacology*
D004042:Dietary Fats, Unsaturated; D004338:Drug Combinations; D004883:Erucic Acids; D005227:Fatty Acids; C079420:Lorenzo's oil; C049811:erucic acid; D014304:Triolein