CEREBRAL AND VISCERAL GLYCOLIPIDS IN A CASE OF TAY-SACHS DISEASE

Cerebral and visceral glycolipids of a patient with the Tay-Sachs disease were investigated in the present study. The disease was found to be characterized by excessive accumulation of Tay-Sachs ganglioside (Go) in the brain, but any storage of ganglioside was not detected in other visceral organs such as the spleen, kidney or liver. Tay-Sachs globoside was also present only in the Tay-Sachs brain. It was found that only a trace amount of glucocerebroside and a small amount of ceramide lactoside were present in the brain with the Tay-Sachs disease. The fatty acid composition of ceramide lactoside, globoside or ganglioside (Go) in the Tay-Sachs brain was different from that of cerebroside and sulfatide, being composed mostly of stearic acid. Tay-Sachs ganglioside (Go) contained mostly C14-sphingosine, besides small amounts of C18-dihydrosphingosine and C20-sphingosine. Moreover, it was noted that ceramide dihexoside showing two bands on thin layer chromatography was the major glycolipids in the Tay-Sachs spleen glycolipids and that the Tay-Sachs liver contained mostly ceramide trihexoside and hematoside. A normal neonatal human brain was used for comparison. Contrary to the Tay-Sachs ganglioside, the amounts of major gangliosides in the normal neonatal human brain were roughly in the order of G11>G1>G111. © 1969 BY THE JOURNAL OF BIOCHEMISTRY.