Abstract
Two structurally related oligosaccharides have been isolated from the urine of a patient with GM 1-gangliosidosis type 2. The isolation procedure included ultrafiltration, gel chromatography on Sephadex G-25, and preparative paper chromatography. From structural studies including optical rotation, sugar analysis, methylation analysis, and chromium trioxide oxidation, the following structures are deduced: ▪ The octasaccharide has previously been reported to be present in both liver and urine of patients with GM 1-gangliosidosis type 1 and type 2. The pentasaccharide is a new compound and is an integral part of the octasaccharide. The yields of the octasaccharide and pentasaccharide were 17 and 8 mg/liter of urine, respectively. Both compounds are most probably degradation products derived from the core of glycoprotein carbohydrate chains.
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