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NEUROLOGY 1985;35:401
© 1985 American Academy of Neurology

Sjögren-Larsson syndrome

{Delta}5- and {Delta}6-fatty acid desaturases in skin fibroblasts

Joel Avigan, Brian D. Campbell, David A. Yost, Olle Hernell, Gösta Holmgren and Sten F. Jagell

Laboratory of Cellular Metabolism, National Heart, Lung, and Blood Institute, National Institutes of Health, Bethesda, MD (Drs. Avigan and Yost and Mr. Campbell); the Department of Pediatrics (Drs. Hernell and Holmgren) and the Department of Clinical Genetics (Drs. Holmgren and Jagell), University Hospital of Umeå, Umeå, Sweden.

Cultured skin fibroblasts from two patients with Sjögren-Larsson syndrome (SLS) and from a normal control were analyzed for trienoic and tetraenoic fatty acids. In addition, we assayed desaturation of [ l - 14C]linoleic acid in cells from four patients and four controls. There was no significant effect of the disease on the composition of polyunsaturated fatty acids or on the rate of linoleic acid desaturation in fibroblasts. The results indicate the presence of {Delta}5- and {Delta}6-fatty acid desaturases in cells from SLS patients.

Address correspondence and reprint requests to Dr. Avigan, Laboratory of Cellular Metabolism, National Heart, Lung, and Blood Institute, National Institutes of Health, Bldg. 10 Room 5N307, Bethesda, MD 20205.

Accepted for publication July 9, 1984




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