Lipids of the Spleen in Gaucher's Disease.

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 ?espect to fatty acid chain length. Long-chain base is all Cl8-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.