Fatty acids in esters and cerebrosides of human brain in phenylketonuria

Abstract

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.

Keywords

• phenylketonuria
• fatty acids
• oleic acid
• cerebrosides
• human brain
• white matter