title: Characterization of one neutral and two acidic glycoasparagines isolated from the urine of patients with aspartylglycosylaminuria (AGU).
abstract: 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.
mesh_terms: 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
chemical_list: D006020:Glycopeptides; D001216:Asparagine; D009439:Neuraminidase