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. 2001 Oct 15;359(Pt 2):249-54.
doi: 10.1042/0264-6021:3590249.

Congenital disorders of glycosylation type I leads to altered processing of N-linked glycans, as well as underglycosylation

P Mills  1 , K Mills, P Clayton, A Johnson, D Whitehouse, B Winchester
Affiliations

Congenital disorders of glycosylation type I leads to altered processing of N-linked glycans, as well as underglycosylation

P Mills et al. Biochem J. .

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.

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