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Glycobiology Advance Access published online on January 22, 2003
Glycobiology, doi:10.1093/glycob/cwg040
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Submitted on October 31, 2002
Revised on December 5, 2002
Accepted on December 5, 2002

© 2003 Oxford University Press

ORIGINAL ARTICLES

Increased fucosylation and reduced branching of serum glycoprotein N-glycans in all known subtypes of Congenital Disorder of Glycosylation I

Nico Callewaert 1, Els Schollen 2, Annelies Vanhecke 1, Jaak Jaeken 3, Gert Matthijs 2, Roland Contreras 1*

1 Department of Molecular Biomedical Research, Ghent University and Flanders Interuniversity Institute for Biotechnology, K.L.-Ledeganckstraat 35, B-9000 Ghent, Belgium
2 Center for Human Genetics, Campus Gasthuisberg, University of Leuven, Herestraat 49, B-3000 Leuven, Belgium
3 Center for Metabolic Disease, Campus Gasthuisberg, University of Leuven, Herestraat 49, B-3000 Leuven, Belgium

* To whom correspondence should be addressed. E-mail: roland.contreras{at}dmb.rug.ac.be.

Abstract

The N-glycans present on the total mixture of serum glycoproteins (serum N-glycome) were analysed for 24 subjects with Congenital Disorder of Glycosylation type I (CDG-I) and 7 healthy, age-matched individuals. No new N-glycan structures were observed in the sera of CDG-I patients as compared to normal sera. However, in all subtypes, we observed a significantly increased degree of core {alpha}-1,6-fucosylation of the biantennary glycans as compared to normal, as well as a significant decrease in the amount of triantennary glycans. These serum N-glycome changes appear to be a milder manifestation of some of the changes observed in adult liver cirrhosis patients (Callewaert et al., in preparation), which is compatible with the reported steatosis and fibrosis in CDG-I patients. In the CDG-Ia subgroup, the extent of the serum N-glycome changes correlates with the aberration of the serum transferrin iso-electric focusing pattern, which measures the severity of the lack of entire N-glycan chains (primary consequence of CDG-I) in the liver and is the standard diagnostic test for this category of inherited diseases.


Keywords: Congenital Disorders of Glycosylation, CDG, DNA-sequencer, glycopathology, N-glycan structure
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