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Fatty Acid Composition of Lipids in Cerebral Myelin and Synaptosomes in Phenylketonuria and Down Syndrome
Ronald C. Johnson, PhD;
Charles M. McKean, MD;
Shantilal N. Shah, PhD
Arch Neurol. 1977;34(5):288-294.
Abstract
• Lipid and fatty acid composition of myelin and synaptosomes isolated from brains of phenylketonuric (PKU) and Down syndrome (DS) patients and patients with neither of these disorders was determined. No differences were observed in the proportions of cholesterol to phospholipids in synaptosomes and cholesterol to galactolipids to phospholipids in myelin or in the fatty acid composition of phospholipids in synaptosomes. However, the myelin from PKU brains contained reduced proportions of unsaturated and long-chain fatty acids. We conclude that genetic PKU and its experimental model have in common a defect in cerebral fatty acid metabolism, and that cerebral lipid abnormalities in this condition, rather than being generalized, may be confined to the glial cell population that synthesizes myelin. We further conclude that DS is not associated with an abnormality of cerebral lipid metabolism.
Author Affiliations
From the Langley Porter Neuropsychiatric Institute, University of California-San Francisco, Brain-Behavior Research Center at Sonoma State Hospital, Eldridge.
Footnotes
Accepted for publication Jan 17, 1977.
Reprint requests to Brain-Behavior Research Center, Sonoma State Hospital, Eldridge, CA 95431 (Dr Shah).
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