Strecker G, Montreuil J
C R Acad Hebd Seances Acad Sci D. 1978 Oct 16;287(9):887-90.
By adding the sequence beta-(1 leads to 4)-GlcNAc-beta-(1 leads to)-Asn or alpha Fuc-(1 leads to 6 or 3)-beta-(1 leads to 4)-GlcNAc-beta-(1 leads to)-Asn to the oligosaccharides isolated from human urines of mannosidosis, fucosidosis and sialidosis, we are able to reconstitute numerous structures of asparaginyl-glycans. We postulate i) that these structures which have not yet been characterized pre-exist in glycans of human glycoproteins, probably in the cytoplasm or/and the cell membrane; ii) that they are products of the action of endo-beta-N-acetyl-glucosaminidases which are protected because of the lack of exoglycosidases and accumulate in the cells, and then in the urine.
通过将序列β-(1→4)-GlcNAc-β-(1→)-Asn或αFuc-(1→6或3)-β-(1→4)-GlcNAc-β-(1→)-Asn添加到从患有甘露糖苷贮积症、岩藻糖苷贮积症和唾液酸贮积症的人类尿液中分离出的寡糖上，我们能够重构许多天冬酰胺基聚糖的结构。我们推测：i) 这些尚未被表征的结构预先存在于人类糖蛋白的聚糖中，可能存在于细胞质或/和细胞膜中；ii) 它们是内切β-N-乙酰氨基葡萄糖苷酶作用的产物，由于缺乏外切糖苷酶而受到保护，并在细胞中积累，然后在尿液中积累。
