Brassart D, Baussant T, Wieruszeski J M, Strecker G, Montreuil J, Michalski J C
Unité Associée au CNRS no. 217, Université des Sciences et Techniques de Lille Flandre-Artois, Villeneuve d'Ascq, France.
Eur J Biochem. 1987 Nov 16;169(1):131-6. doi: 10.1111/j.1432-1033.1987.tb13589.x.
A mixture of sialylglycoasparagines and sialylglycopeptides was successively incubated with lysosomal extracts, at two pH values, first at pH 7.5 and then at pH 4. The 1H-NMR analysis of the sialyloligosaccharides released during the enzymatic digestion demonstrates the sequential action of aspartylglucosaminidase and an endo-N-acetyl-beta-D-glucosaminidase which release sialyloligosaccharides identical to the reference sugars isolated from the urine of patients suffering from sialidosis. This process represents a new catabolic pathway for N-glycosyl-proteins which may account for the appearance of the oligosaccharides stored in tissues and urine of patients suffering from lysosomal diseases.
将唾液酸糖天冬酰胺和唾液酸糖肽的混合物先后在两个pH值下与溶酶体提取物孵育，首先在pH 7.5，然后在pH 4。对酶促消化过程中释放的唾液酸寡糖进行的1H-NMR分析表明，天冬氨酰葡糖胺酶和一种内切N-乙酰-β-D-葡糖胺酶具有顺序作用，它们释放的唾液酸寡糖与从唾液酸沉积症患者尿液中分离出的参考糖相同。这一过程代表了N-糖基化蛋白的一种新的分解代谢途径，这可能解释了溶酶体疾病患者组织和尿液中储存的寡糖的出现。
