Specific cleavage of the glycosidic bond between the carbohydrate and ceramide portions in glycosphingolipids using trifluoroacetolysis.

A number of glycosphingolipids have been subjected to trifluoroacetolysis. Heating the glycosphingolipid at 100 degrees C for 48 h in mixtures of trifluoroacetic acid (TFA) and trifluoroacetic anhydride (TFAA) varying from 1:1 to 1:100 (v/v) resulted in complete cleavage of the glycosidic bond between the carbohydrate and the unsaturated base of the ceramide. The fatty acid(s) linked to the amino group of sphingosine were also released by transamidation.

Urinary abnormalities in fucosidosis. Characterization of a disaccharide and two glycoasparagines.

The urinary excretion of fucose-containing material was found to be highly increased in a patient with fucosidosis type 2. Three structurally related compounds, a disaccharide and two glycoasparagines, were isolated from the urine. The isolation procedure included ultrafiltration, gel chromatography on Sephadex G-25, preparative zone electrophoresis and paper chromatography.

Glucose-containing oligosaccharides in the urine of patients with glycogen storage disease type II and type III.

Patients with glycogen storage disease type II and type III were recently found to excrete increased amounts of a glucose-containing tetrasaccharide DGlcp(alpha1 leads to 6)DGlcp(alpha1 leads to 4)DGlcp(alpha1 leads to 4)DGlc [Lennartson, G., Lundblad, A., Sjöblad, S.