Characterization of a Blood Group B Glycolipid, Accumulating in the Pancreas of a Patient with Fabry's Disease

Abstract

Abstract An accumulation of a ceramide hexasaccharide was found in the pancreas of a patient with Fabry's disease, in addition to the accumulation of ceramide trihexoside and ceramide digalactoside. The ceramide hexasaccharide was isolated and identified as a blood group B-active glycolipid with the following structure: [see PDF for equation] The proportion of two structures, one containing Gall → 3GlcNAc and one containing Gall → 4GlcNAc, is 4:1.