Characterization of four monosialo and a novel disialo Asn N-glycosides from the urine of a patient with aspartylglycosaminuria |
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| Authors: | Fumitoshi Irie Hitomi Murakoshi Takashi Suzuki Yasuo Suzuki Kazuo Kon Susumu Ando Kunihiro Yoshida Yoshio Hirabayashi |
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| Affiliation: | (1) Department of Biochemistry, University of Shizuoka, School of Pharmaceutical Science, Shizuoka-shi, 422 Shizuoka, Japan;(2) Department of Membrane Biochemistry, Tokyo Metropolitan Institute of Gerontology, Itabashi-ku, 173 Tokyo, Japan;(3) Department of Medicine (Neurology), Shinshu University School of Medicine, Asahi, Matsumoto-shi, 390 Nagano, Japan;(4) Laboratory for Glyco Cell Biology, Frontier Research Program, The Institute of Physical and Chemical Research (RIKEN), Hirosawa, Wako-shi, 351-01 Saitama, Japan |
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| Abstract: | 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 2  8NeuAc2 3Gal 1 4GlCNAc1 Asn. |
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| Keywords: | glycoprotein aspartylglycosaminuria pulsed amperometric detector |
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