A comparative study of sialyloligosaccharides isolated from sialidosis and galactosialidosis urine

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Abstract
Summary: Sialic acid‐containing carbohydrates were isolated from sialidosis urine by a combination of gel‐filtration on Bio‐Gel P‐6 and medium‐pressure anion‐exchange chromatography on Mono Q. The Mono Q fractions were subjected to 500‐MHz1H‐NMR spectroscopy, sugar analysis and analytical HPLC on Lichrosorb‐NH2. These methods indicated the presence of variousN‐acetyllactosamine type sialyloligosaccharides differing from each other in branching pattern and sialic acid linkage types. Among the structures were fully and partially sialylated mono‐, di‐, tri‐ and tetra‐antennary compounds. A comparison with the results from galactosialidosis urine indicated that essentially the same carbohydrates were present in both urines, but that the relative amounts of the various sialyloligosaccharides differ to some extent. Sialidosis urinary oligosaccharides contained relatively more α2–6 linked sialic acid than oligosaccharides from galactosialidosis urine. It could be concluded that the additionalβ‐galactosidase deficiency in galactosialidosis did not influence the nature of the excreted material and that the sialidase deficiency determined completely the defective catabolism of glycoproteins in both sialidosis and galactosialidosis.

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