Journal of Lipid Research, Vol. 9, 129-136, January 1968
Copyright © 1968 by Lipid Research, Inc.

Gangliosides in subacute sclerosing leukoencephalitis: isolation and fatty acid composition of nine fractions

Robert Ledeen , Kenneth Salsman , and Maria Cabrera

The Saul R. Korey Department of Neurology and the Department of Biochemistry, Albert Einstein College of Medicine of Yeshiva University, Bronx, New York 10461

Gangliosides from brain of an 8 yr old boy with subacute sclerosing leukoencephalitis have been studied in terms of pattern and structure. Thin-layer chromatography showed that both gray and white matter have a highly abnormal pattern, with elevation of the relative proportion of four gangliosides corresponding to minor species in normal brain. The total level of lipid-bound sialic acid, however, was not increased, which indicated a compensating loss of other gangliosides. Two of the proliferating species were monosialogangliosides (G₅ and G₆) (Korey nomenclature), and two were disialo types (G_2A and G_3A). Studies of their carbohydrate structures are described.

Nine ganglioside fractions were isolated by preparative TLC in combination with column chromatography, and the fatty acid compositions were determined. Seven contained stearate as the major component, while two (G₃a and G₆) had relatively large proportions of oleate and palmitate. Five of the fractions contained two fatty acids of long chain-length and unknown structure.

Supplementary key words gangliosides • abnormal pattern • subacute sclerosing leukoencephalitis • ganglioside fractions • separation • fatty acid composition • carbohydrate structure • brain • disease • glycolipids • demyelination

Submitted on June 19, 1967
Accepted on September 18, 1967

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