Congenital disorders of glycosylation type I leads to altered processing of N-linked glycans, as well as underglycosylation
- 15 October 2001
- journal article
- Published by Portland Press Ltd. in Biochemical Journal
- Vol. 359 (2) , 249-54
- https://doi.org/10.1042/0264-6021:3590249
Abstract
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.Keywords
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