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Journal of Lipid Research, Vol. 7, 337-340, May 1966
Copyright © 1966 by Lipid Research, Inc.

Abnormal gangliosides in Tay-Sachs disease, Niemann-Pick's disease, and gargoylism

D. A. Booth , H. Goodwin , and J. N. Cumings

Institute of Neurology, The National Hospital, Queen Square, London, England

The molar ratios of N-acetyl neuraminic acid, hexose, hexosamine, and sphingosine have been determined for the abnormal ganglioside in Tay-Sachs disease that was previously detected as a fast-moving band in thin-layer chromatography, and in two abnormal fast-moving bands of gangliosides from the cortex and white matter of the brain in cases of gargoylism and Niemann-Pick's disease. The fastest-moving ganglioside band in these two conditions contains neither hexosamine nor glucose.

Supplementary key words thin-layer chromatography • fast-moving band • gangliosides • white matter • cortex • Niemann-Pick • gargoylism • Tay-Sachs • molar ratios • N-acetyl neuraminic acid • hexosamine • hexose • sphingosine

Submitted on August 16, 1965
Accepted on December 17, 1965

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