来自一名天冬氨酰氨基葡糖苷尿症患者尿液中的四种单唾液酸和一种新型双唾液酸天冬酰胺 N-糖链的表征。

Characterization of four monosialo and a novel disialo Asn N-glycosides from the urine of a patient with aspartylglycosaminuria.

作者信息

Irie F, Murakoshi H, Suzuki T, Suzuki Y, Kon K, Ando S, Yoshida K, Hirabayashi Y

机构信息

Department of Biochemistry, University of Shizuoka, School of Pharmaceutical Science, Japan.

出版信息

Glycoconj J. 1995 Jun;12(3):290-7. doi: 10.1007/BF00731332.

DOI:10.1007/BF00731332

PMID:7496144

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 alpha 2-->8NeuAc alpha 2-->3Gal beta 1-->4GlcNAc beta 1-->Asn.

摘要

我们之前首次报道了两名患有天冬氨酰氨基葡糖苷酶缺乏症（AGU）的日本患者。在本研究中，通过凝胶过滤和阴离子交换色谱法，除了从一名患者的尿液中分离出四种已知的单唾液酸天冬氨酰N - 糖苷（AG - 1至AG - 4）外，还分离出了一种新型的二唾液酸天冬氨酰N - 糖苷（AG - 5）。AG - 5的最终纯化通过电化学色谱法、配备脉冲安培检测器的高效液相色谱法（HPLC - PAD）实现。AG - 5的产量约为每升尿液1毫克。AG - 1至AG - 5的化学结构通过气液色谱法、全甲基化研究、快原子轰击质谱法（FAB - MS）和核磁共振（NMR）进行了表征。基于结构分析，AG - 5具有以下新型结构：NeuAcα2→8NeuAcα2→3Galβ1→4GlcNAcβ1→Asn。