从天冬氨酰葡糖胺尿症（AGU）患者尿液中分离出的一种中性和两种酸性糖天冬酰胺的表征。

Characterization of one neutral and two acidic glycoasparagines isolated from the urine of patients with aspartylglycosylaminuria (AGU).

作者信息

Sugahara K, Funakoshi S, Funakoshi I, Alla P, Yamashina I

出版信息

J Biochem. 1976 Aug;80(2):195-201. doi: 10.1093/oxfordjournals.jbchem.a131264.

DOI:10.1093/oxfordjournals.jbchem.a131264

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.

摘要

从天冬氨酰葡糖胺尿症（AGU）患者的尿液中分离出一种中性和两种酸性糖天冬酰胺。中性糖天冬酰胺被鉴定为β - 半乳糖 -（1→4）-β - N - 乙酰葡糖胺 - 天冬酰胺。酸性糖天冬酰胺由1摩尔唾液酸、各2摩尔半乳糖和N - 乙酰葡糖胺连接到天冬酰胺组成，并且由于它们与产气荚膜梭菌的神经氨酸酶[EC 3.2.1.18]孵育时产生相同的糖天冬酰胺，所以在唾液酸连接位置上是异构体。主要基于顺序酶促降解的结果，确定所得无唾液酸糖天冬酰胺的结构为β - 半乳糖 - β - N - 乙酰葡糖胺 - β - 半乳糖 -（1→4）-β - N - 乙酰葡糖胺 - 天冬酰胺。