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Journal of Lipid Research, Vol. 6, 211-219, April 1965
Copyright © 1965 by Lipid Research, Inc.

Lipids of the spleen in Gaucher's disease

William D. Suomi and Bernard W. Agranoff

Department of Biological Chemistry and Mental Health Research Institute, The University of Michigan, Ann Arbor, Michigan

Thin-layer chromatography (TLC) was used to analyze lipids of eight spleens of patients with Gaucher's disease. Four non-Gaucher spleens were also analyzed. Phospholipid concentrations are not increased in Gaucher spleens, while several classes of neutral lipids are moderately increased.

Acid hydrolysis followed by hexose determination was applied to total lipids and to separated fractions. Specific oxidase reactions demonstrated that glucose present is primarily in ceramide glucoside and ceramide lactoside, and galactose is primarily in ceramide lactoside. No ceramide galactoside was detected. The two hexoses found account for the anthrone-positive material in the lipid extract. Two TLC spots seen for Gaucher ceramide glucoside differ only with respect to fatty acid chain length. Long-chain base is all C₁₈-sphingosine. Small amounts of hydroxy acids are present in ceramide lactoside, but not in ceramide glucoside. Ceramide lactoside is present in similar amounts in Gaucher and non-Gaucher spleens.

Supplementary key words spleen • lipids • cerebrosides • fatty acids • man • Gaucher's disease • galactose oxidase • thin-layer chromatography • glycosphingolipids

Submitted on June 16, 1964
Accepted on November 5, 1964

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