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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)
Authors:Magnhild Rasmussen  Ann B Moser  Janet Borel  Surinder Khangoora  Hugo W Moser
Institution:(1) Department of Neurogenetics, The Kennedy Krieger Institute, 707 North Broadway, 21205 Baltimore, MD
Abstract: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 olis (ldquoLorenzo's Oilrdquo) 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.Special issue dedicated to Dr. Marjorie B. Lees.
Keywords:Adrenoleukodystrphy  erucic acid  very long chain fatty acids  dietary therapy
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