Urinary Abnormalities in Fucosidosis. Characterization of a Disaccharide and Two Glycoasparagines

Abstract

The urinary excretion of fucose-containing material was highly increased in a patient with fucosidosis type 2. Three structurally related compounds, a disaccharide and 2 glycoasparagines, were isolated from the urine. The isolation procedure included ultrafiltration, gel chromatography on Sephadex G-25, preparative zone electrophoresis and paper chromatography. From structural studies including optical rotation, sugar analysis, methylation analysis, ninhydrin degradation, reduction with lithium aluminium hydride and partial hydrolysis, the structures were deduced. The yields of these compounds were 1.7, 40 and 6 mg/l, respectively. The origin of the disaccharide and the 2 glycoasparagines is probably the core region of glycoprotein carbohydrate chains.