Urinary sialyloligosaccharides in adult type sialidosis: occurrence of two positional isomers.

Abstract

Two positional isomers of sialyloligosaccharides were isolated from the urine of a patient with adult type sialidosis with partial deficiency of beta-galactosidase. From structural studies including sugar analysis, enzyme degradation and methylation analysis, the following structures are deduced; 1. alpha-AcNeu-(2 leads to 3)-beta-Gal-(1 leads to 4)-beta-GlcNAc-(1 leads to 2)-alpha-Man-(1 leads to 3)-beta-Man-(1 leads to 4)-GlcNAc 2. alpha-AcNeu-(2 leads to 3)-beta-Gal-(1 leads to 4)-beta-GlcNAc-(1 leads to 2)-alpha-Man-(1 leads to 6)-beta-Man-(1 leads to 4)-GlcNAc The sialyloligosaccharide 1 has previously been reported to be present in liver and urine of patients with mucolipidosis I, II and variant forms. The sialyloligosaccharide 2 was first demonstrated in human urine.

• Authors: M Kuriyama, T Ariga, S Ando, M Suzuki, T Yamada, T Miyatake
• Journal: The Japanese journal of experimental medicine (Jpn J Exp Med) Vol. 51 Issue 2 Pg. 129-32 (Apr 1981) ISSN: 0021-5031 [Print] Japan
• PMID: 6792402 (Publication Type: Journal Article, Research Support, Non-U.S. Gov't)

Chemical References

• Oligosaccharides
• Sialic Acids
• sialooligosaccharides
• beta-Galactosidase

Topics

• Chemical Phenomena
• Chemistry
• Chromatography, Gas
• Humans
• Isomerism
• Lactose Intolerance (urine)
• Oligosaccharides (urine)
• Sialic Acids (urine)
• beta-Galactosidase