[甘露糖苷贮积症患者尿中排泄的甘露糖苷的化学性质]

[Chemistry of urinary mannosides excreted in mannosidosis].

作者信息

Strecker G, Fournet B, Bouquelet S, Montreuil J, Dhondt J L, Farriaux J P

出版信息

Biochimie. 1976;58(5):579-86. doi: 10.1016/s0300-9084(76)80227-1.

Abstract

Mannose-rich oligosaccharides have been isolated from urines of 5 patients with mannosidosis. Their compositon and structure were determined. Three of them have been previously described by Norden et al: alpha p-Manp-(1 leads to 3) beta-d-Manp-(1 leads to 4) d-GlcNAcp; alpha-p-Manp-(1 leads to 2), alpha-d-Manp-(1 leads to 3) beta-d-Manp-(1 leads to 4) d-GlcNAc and alpha-d-Manp-(1 leads to 2) alpha-d-Manp-(1 leads to 2) alpha-d-Manp-(1 leads to 3) beta-d-Manp-(1 leads to 4) d-GlcNAcp, but the four others are new entities: alpha-d-Manp-(1 leads to 3) (alpha-d-Manp-(1 leads to 2) alpha-d-Manp-(1 leads to 2) alpha-d-Manp-(1 leads to 6) beta-d-Manp-(1 leads to 4) GlcNAcp; alpha-d-Manp-(1 leads to 2) alpha-d-Manp-(1 leads to 3) (alpha-d-Manp-(1 leads to 2) alpha-d-Manp-(1 leads to 6) beta-d-Manp-(1 leads to 4) GlcNAcp; alpha-d-Manp-(1 leads to 2), alpha-d-Man-(1 leads to 3) (alpha-d-Manp-(1 leads to 6) beta-d-Manp-(1 leads to 4) GlcNAp and alpha-d-Manp-(1 leads to 2) alpha-d-Manp-(1 leads to 3) (alpha-d-Manp-(1 leads to 6) beta-d-Manp-(1 leads to 4) GlcNAcp. These structures are related to the glycans of "oligomannosidic type" present in numerous glycoproteins. All possess a N-acetylglucosamine residue in terminal reducing position and reinforce the hypothesis of Kobata et al. and Montreuil et al. that catabolism of glycans N-glucosidically linked to the protein moiety begins by the aciton of a beta-endo-N-acetylglucosaminidase.

摘要

已从5名甘露糖苷贮积症患者的尿液中分离出富含甘露糖的寡糖。确定了它们的组成和结构。其中三种先前已由诺登等人描述过：α-p-甘露糖基-(1→3)-β-d-甘露糖基-(1→4)-d-葡萄糖胺基；α-p-甘露糖基-(1→2)，α-d-甘露糖基-(1→3)-β-d-甘露糖基-(1→4)-d-葡萄糖胺和α-d-甘露糖基-(1→2)-α-d-甘露糖基-(1→2)-α-d-甘露糖基-(1→3)-β-d-甘露糖基-(1→4)-d-葡萄糖胺基，但另外四种是新的化合物：α-d-甘露糖基-(1→3)-(α-d-甘露糖基-(1→2)-α-d-甘露糖基-(1→2)-α-d-甘露糖基-(1→6)-β-d-甘露糖基-(1→4)-葡萄糖胺基；α-d-甘露糖基-(1→2)-α-d-甘露糖基-(1→3)-(α-d-甘露糖基-(1→2)-α-d-甘露糖基-(1→6)-β-d-甘露糖基-(1→4)-葡萄糖胺基；α-d-甘露糖基-(1→2)，α-d-甘露糖基-(1→3)-(α-d-甘露糖基-(1→6)-β-d-甘露糖基-(1→4)-葡萄糖胺基和α-d-甘露糖基-(1→2)-α-d-甘露糖基-(1→3)-(α-d-甘露糖基-(1→6)-β-d-甘露糖基-(1→4)-葡萄糖胺基。这些结构与许多糖蛋白中存在的“寡甘露糖型”聚糖相关。所有这些都在末端还原位置具有一个N-乙酰葡萄糖胺残基，并支持了小幡等人以及蒙特勒伊等人的假说，即与蛋白质部分以N-糖苷键连接的聚糖的分解代谢始于β-内-N-乙酰葡萄糖胺酶的作用。