Structure of a novel sialooligosaccharide from the urine of a patient with mucolipidosis.

Abstract
A mannose-containing sialooligosaccharide was isolated from the urine of a patient with a newly recognized mucolipidosis which showed a low liver .beta.-galactosidase activity and hyperglycopeptiduria. The chemical structure was determined by glycosidase digestion, Smith degradation and permethylation studies. The following structure was given for the oligosaccharide: NANA.alpha.2-6Gal.beta.1-4GlcNAc.beta.1-2Man.beta.1-3Man.beta.1-4GlcNAc. This compound appears to be derived from an incomplete catabolism of glycoproteins with aspartylglucosylamine type side chains.