成人型唾液酸沉积症中的尿唾液酸寡糖：两种位置异构体的出现

Urinary sialyloligosaccharides in adult type sialidosis: occurrence of two positional isomers.

作者信息

Kuriyama M, Ariga T, Ando S, Suzuki M, Yamada T, Miyatake T

出版信息

Jpn J Exp Med. 1981 Apr;51(2):129-32.

Abstract

Two positional isomers of sialyloligosaccharides were isolated from the urine of a patient with adult type sialidosis with partial deficiency of beta-galactosidase. From structural studies including sugar analysis, enzyme degradation and methylation analysis, the following structures are deduced; 1. alpha-AcNeu-(2 leads to 3)-beta-Gal-(1 leads to 4)-beta-GlcNAc-(1 leads to 2)-alpha-Man-(1 leads to 3)-beta-Man-(1 leads to 4)-GlcNAc 2. alpha-AcNeu-(2 leads to 3)-beta-Gal-(1 leads to 4)-beta-GlcNAc-(1 leads to 2)-alpha-Man-(1 leads to 6)-beta-Man-(1 leads to 4)-GlcNAc The sialyloligosaccharide 1 has previously been reported to be present in liver and urine of patients with mucolipidosis I, II and variant forms. The sialyloligosaccharide 2 was first demonstrated in human urine.

摘要

从一名患有成人型唾液酸沉积症且β-半乳糖苷酶部分缺乏的患者尿液中分离出两种唾液酸寡糖的位置异构体。通过包括糖分析、酶降解和甲基化分析在内的结构研究，推断出以下结构：1. α-乙酰神经氨酸-(2→3)-β-半乳糖-(1→4)-β-氨基葡萄糖-(1→2)-α-甘露糖-(1→3)-β-甘露糖-(1→4)-氨基葡萄糖 2. α-乙酰神经氨酸-(2→3)-β-半乳糖-(1→4)-β-氨基葡萄糖-(1→2)-α-甘露糖-(1→6)-β-甘露糖-(1→4)-氨基葡萄糖唾液酸寡糖1先前已报道存在于黏脂贮积症I、II及变异型患者的肝脏和尿液中。唾液酸寡糖2首次在人尿中被证实。