Characterization of Two Glycoasparagines Isolated from the Urine of Patients with Aspartylglycosylaminuria (AGU)1

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Abstract
Two major glycoasparagines (2-acetamido-N-(4'-L-aspartyl)-2-deoxy-β-D-glycosylamines) were isolated from the urine of patients with aspartylglycosylaminuria (AGU). They were composed of equimolar amounts of sialic acid, galactose, glucosamine, and aspartic acid. They were isomeric with respect to the position of sialic acid attachment, since they produced the same glycoasparagine on incubation with the neuraminidase from Clostridium perfringens. The structure of the resulting sialic acid-free glycoasparagine was determined as β-Gal-(1→4)-β-GlcNAc-Asn based on the following findings. It produced galactose on incubation with β-galactosidase, and N-acetyllactosamine and aspartic acid on incubation with 4-L-aspartylglycosylamine amido hydrolase.

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