Journal of Lipid Research, Vol. 8, 105-113, March 1967
Copyright © 1967 by Lipid Research, Inc.

Brain ceramide hexosides in Tay-Sachs disease and generalized gangliosidosis (G_m1-gangliosidosis)

Kunihiko Suzuki and Gloria C. Chen

The Saul R. Korey Department of Neurology, Albert Einstein College of Medicine, New York 10461

The carbohydrate composition was determined for ceramide hexosides isolated from brains of patients with Tay-Sachs disease and generalized gangliosidosis (hereby named G_m1-gangliosidosis).

Gray matter of patients with each disease showed a characteristic abnormal ceramide hexoside pattern. In Tay-Sachs gray matter, ceramide trihexoside is the major component, whereas ceramide tetrahexoside is barely detectable. In G_m1-gangliosidosis, ceramide tetrahexoside is the major ceramide hexoside, while ceramide trihexoside is present only in small amount. These two major components have been characterized as the asialo derivatives of, respectively, the "Tay-Sachs ganglioside" (G_m2-ganglioside) and the normal major monosialoganglioside (G_m1-ganglioside).

In both diseases, more than half the ceramide monohexoside of gray matter was glucocerebroside. Gray matter ceramide dihexoside, present in both diseases at higher than normal levels, was mostly ceramide lactoside, with possibly a small amount of ceramide digalactoside. Sulfatide contained only galactose.

The abnormal ceramide hexoside pattern is limited to gray matter: white matter showed normal ceramide hexosides, i.e. a preponderance of monohexosides and sulfatide, with no detectable glucocerebroside.

Supplementary key words Tay-Sachs disease (G_M2-gangliosidosis) • generalized gangliosidosis (G_M1-gangliosidosis) • systemic late infantile lipidosis • ceramide hexosides • glucocerebroside • ganglioside • gray matter • white matter • man

Submitted on September 15, 1966
Accepted on November 29, 1966

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