Structure of a novel sialooligosaccharide from the urine of a patient with mucolipidosis.
A mannose-containing sialooligosaccharide has been 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 has been determined by glycosidase digestion, Smith degradation and permethylation studies. The following structure has been given for the oligosaccharide: NANAalpha2-6Galbeta1-4GlcNAcbeta1-2Manbeta1-3Manbeta1-4GlcNAc. This compound appears to be derived from an incomplete catabolism of glycoproteins with aspartylglucosylamine type side chains.
D055598:Chemical Phenomena; D002621:Chemistry; D006801:Humans; D009081:Mucolipidoses / Q000378:metabolism / Q000652:urine*; D009844:Oligosaccharides / Q000652:urine*; D012794:Sialic Acids / Q000652:urine*
D009844:Oligosaccharides; D012794:Sialic Acids