Characterization of four monosialo and a novel disialo Asn N-glycosides from the urine of a patient with aspartylglycosaminuria.
We previously reported for the first time two Japanese patients with aspartylglycosaminuria (AGU). A novel disialo Asn N-glycoside (AG-5) has been isolated from the urine of one of the patients in addition to four known monosialo Asn N-glycosides (AG-1 to AG-4) by gel filtration and anion exchange chromatography in this study. Final purification of AG-5 was achieved by an electrochemical chromatographic method, high performance liquid chromatography with pulsed amperometric detector (HPLC-PAD). The yield of AG-5 was approximately 1 mg l-1 urine. The chemical structures of AG-1 to AG-5 were characterized by gas-liquid chromatography, a permethylation study, fast atom bombardment-mass spectrometry (FAB-MS), and nuclear magnetic resonance (NMR). Based on the structural analysis, AG-5 had the following novel structure: NeuAc alpha 2-->8NeuAc alpha 2-->3Gal beta 1-->4GlcNAc beta 1-->Asn.
D000117:Acetylglucosamine / Q000031:analogs & derivatives* / Q000652:urine; D000328:Adult; D002240:Carbohydrate Sequence; D002851:Chromatography, High Pressure Liquid; D005260:Female; D006027:Glycosides / Q000652:urine*; D006801:Humans; D008969:Molecular Sequence Data
D006027:Glycosides; C000009:N-acetylglucosaminylasparagine; D000117:Acetylglucosamine