Characterization of one neutral and two acidic glycoasparagines isolated from the urine of patients with aspartylglycosylaminuria (AGU).
One neutral and two acidic glycoasparagines were isolated from the urine of patients with aspartylglycosylaminuria (AGU). The neutral one was identified as beta-Gal-(1 leads to 4)-beta-GlcNAc-Asn. The acidic ones were composed of 1 mole of sialic acid and 2 moles each of galactose and N-acetylglucosamine, attached to asparagine, and were isomeric with respect to the position of sialic acid attachment since they produced the same glycoasparagine on incubation with the neuraminidase [EC 3.2.1.18] from Clostridium perfringens. The structure of the resulting sialic acid-free glycoasparagine was determined to be beta-Gal-beta-GlcNAc-beta-Gal-(1 leads to 4)-beta-GlcNAc-Asn based mainly on the results of sequential enzymatic degradations.
D001216:Asparagine / Q000031:analogs & derivatives* / Q000652:urine; D003016:Clostridium perfringens / Q000201:enzymology; D006020:Glycopeptides / Q000652:urine*; D006801:Humans; D008661:Metabolism, Inborn Errors / Q000652:urine*; D008968:Molecular Conformation; D009439:Neuraminidase
D006020:Glycopeptides; D001216:Asparagine; D009439:Neuraminidase