Increased alpha 3-fucosylation of alpha 1-acid glycoprotein in patients with congenital disorder of glycosylation type IA (CDG-Ia)

Increased fucosylation of the type (sialyI) Lewis(x) was detected on the ac ute-phase plasma protein at-acid glycoprotein (AGP) in patients with the co ngenital disorder of glycosylation type IA, This is remarkable, because in these patients the biosynthesis of guanosine 5'-diphosphate (GDP)-D-mannose is strongly decreased, and CDP-D-mannose is the direct precursor for GDP-L -fucose, the substrate for fucosyltransferases. The concomitantly occurring increased branching of the glycans of AGP and the increased fucosyltransfe rase activity in plasma suggest that a chronic hepatic inflammatory reactio n has induced the increase in fucosylation, (C) 2001 Federation of European Biochemical Societies. Published by Elsevier Science B.V. All rights reser ved.