Abstract
Sialyl-glycopeptides containing an O-glycosidically linked tetrasaccharide chain were obtained from the urine of a patient suffering from mucolipidosis I. Isolation of these compounds was achieved by gel filtration, ion-exchange chromatography and preparative paper chromatography. Their structures were determined by a combination of carbohydrate and amino acid analysis, dansylation, periodate oxidation, methylation studies, enzymatic hydrolysis and 1H-NMR spectroscopy, to be as follows: (formula; see text) wherein R = peptide linked through -Thr-, -Ser-Thr- or -Thr-Ser-. The finding of these glycopeptides in urine shows that mucolipidosis I is characterized by a general "glycoprotein-specific" sialidase deficiency. The possibility of the existence of a human endo-alpha-N-acetylgalactosaminidase is discussed.
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