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Departments of Pediatrics and Human Genetics (Drs. Rizzo, Phillips, and Cranin), Medical College of Virginia, Richmond, VA; and the Laboratory of Cellular Metabolism (Drs. Watkins, Campbell, and Avigan), National Heart, Lung, and Blood Institute, National Institutes of Health, Bethesda, MD.
Monounsaturated fatty acids, especially oleic acid (C18:1), decreased the content of saturated very-long-chain fatty acids (VLFA) in cultured skin fibroblasts from patients with adrenoleukodystrophy (ALD) and controls. When confluent ALD fibroblasts were incubated with oleic acid for 5 days in lipid-free medium, which eliminates uptake of exogenous VLFA, the mean cell content of C26:0 was decreased by 33.7 ± 10.1%. Oleic acid inhibited C26:0 synthesis in ALD fibroblasts by 58% and total fatty acid synthesis by 68 to 78%. Therefore, the elevated C26:0 levels in ALD cells may be lowered by inhibiting fatty acid biosynthesis, and inhibition of saturated VLFA synthesis by oleate may be useful in treating ALD.
Address correspondence and reprint requests to Dr. Rizzo, Department of Pediatrics, Medical College of Virginia, PO Box 259, MCV Station, Richmond, VA 23298.
Supported in part by a grant from A.D. Williams Foundation of the Medical College of Virginia, NIH grant AM 33914–01, and by Basil O'Connor Starter Research Grant No. 5–476 from the March of Dimes Birth Defects Foundation (W.B.R.).
Accepted for publication July 26, 1985.
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