J. Lipid Res.
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Journal of Lipid Research, Vol. 13, 128-136, January 1972
Copyright © 1972 by Lipid Research, Inc.

Generalized accumulation of neutral glycosphingolipids with GM2 ganglioside accumulation in the brain

Paul D. Snyder Jr. , William Krivit , and Charles C. Sweeley

Department of Biochemistry, Michigan State University, East Lansing, Michigan 48823, and Department of Pediatrics, University of Minnesota Medical School, Minneapolis, Minnesota 55455

Analyses have been made of glycosphingolipids from visceral organs and brain of a patient with an unusual lipid storage disorder diagnosed initially as classical Tay-Sachs disease. Levels of the lipids from fresh-frozen sections of gray and white matter, kidney, spleen, liver, and heart from this patient were compared with those of normal juvenile controls, and the fatty acid composition of accumulated glycosphingolipids was compared with reference compounds. This patient was found to have abnormally high concentrations of a globoside in liver, kidney, and spleen, asialo GM2 ganglioside in brain and liver, and GM2 ganglioside in the brain. On the basis of these findings along with the clinical manifestations of Tay-Sachs disease with visceral involvement (hepatosplenomegaly) and demonstration of total deficiency of both A and B components of ßbeta;-N-acetylhexosaminidase activity, this glycosphingolipidosis is the same as two previously reported cases of GM2 gangliosidosis with globoside accumulation and total ßbeta;-N-acetylhexosaminidase deficiency.

Supplementary key words globoside • Gm2 ganglioside • asialo Gm2 • glycosphingolipid storage disease • total hexosaminidase deficiency

Submitted on April 14, 1971
Accepted on September 1, 1971


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Y.-T. Li, K. Maskos, C.-W. Chou, R. B. Cole, and S.-C. Li
Presence of an Unusual GM2 Derivative, Taurine-conjugated GM2, in Tay-Sachs Brain
J. Biol. Chem., September 12, 2003; 278(37): 35286 - 35291.
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