Mills P, Mills K, Clayton P, Johnson A, Whitehouse D, Winchester B
Biochemistry, Endocrinology and Metabolism Unit, Institute of Child Health at Great Ormond Street Hospital, University College London, 30 Guilford Street, London, WC1N 1EH, UK.
Biochem J. 2001 Oct 15;359(Pt 2):249-54. doi: 10.1042/0264-6021:3590249.
The N-linked glycans on transferrin and alpha(1)-antitrypsin from patients with congenital disorders of glycosylation type I have increased fucosylation and branching relative to normal controls. The elevated levels of monofucosylated biantennary glycans are probably due to increased alpha-(1-->6) fucosylation. The presence of bi- and trifucosylated triantennary and tetra-antennary glycans indicated that peripheral alpha-(1-->3), as well as core alpha-(1-->6), fucosylation is increased. Altered processing was observed on both the fully and underglycosylated glycoforms.
与正常对照相比,I型糖基化先天性疾病患者的转铁蛋白和α1抗胰蛋白酶上的N-连接聚糖具有增加的岩藻糖基化和分支。单岩藻糖基化双天线聚糖水平升高可能是由于α-(1→6)岩藻糖基化增加所致。双岩藻糖基化和三岩藻糖基化的三天线和四天线聚糖的存在表明外周α-(1→3)以及核心α-(1→6)岩藻糖基化增加。在完全糖基化和糖基化不足的糖型上均观察到加工改变。