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Journal of Lipid Research, Vol. 6, 518-524, October 1965
Copyright © 1965 by Lipid Research, Inc.

Fatty acids in esters and cerebrosides of human brain in phenylketonuria

J. Lindsley Foote , Richard J. Allen , and Bernard W. Agranoff

Mental Health Research Institute, Department of Biological Chemistry, and Pediatric Neurological Unit, Department of Pediatrics, University of Michigan, Ann Arbor, Michigan

Fatty acids from cerebrosides and cerebroside sulfates and those bound by ester linkage from white and gray matter of frozen phenylketonuric brains were determined and compared with analyses of nonphenylketonuric brains. A decrease in the major monoenoic acid relative to the major saturated acid (18:1/18:0, or 24:1/24:0, or 24h:1/24h:0) of each fraction analyzed is observed in this disease. The amounts of cholesterol, cerebroside, and cerebroside sulfate in the brain are not altered in phenylketonuria.

The sum of the three polyunsaturated fatty acids 20:4, 22:5, and 22:6 constitute approximately 30% of the ester-bound fatty acids of human cortical gray matter.

Supplementary key words phenylketonuria • fatty acids • oleic acid • cerebrosides • human brain • white matter • gray matter • polyunsaturated fatty acids • hydroxy acids • cholesterol

Submitted on March 19, 1965
Accepted on May 20, 1965

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