The Tohoku Journal of Experimental Medicine
Online ISSN : 1349-3329
Print ISSN : 0040-8727
ISSN-L : 0040-8727
Structure of a Novel Sialooligosaccharide from the Urine of a Patient with Mucolipidosis
MASAI KOSEKI, KOICHI TSURUMI
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Keywords: sialooligosaccharide, mucolipidosis, lysosomal storage disease, sialoglucide
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1978 Volume 124 Issue 4 Pages 361-366

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Abstract

A mannose-containing sialooligosaccharide has been isolated from the urine of a patient with a newly recognized mucolipidosis which showed a low liver β-galactosidase activity and hyperglycopeptiduria. The chemical structure has been determined by glycosidase digestion, Smith degradation and permethylation studies. The following structure has been given for the oligosaccharide: NANAα2-6Galβ1-4GlcNAcβ1-2Manβ1-3Manβ1-4GlcNAc. This compound appears to be derived from an incomplete catabolism of glycoproteins with aspartylglucosylamine type side chains.

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