Increased α3‐fucosylation of α1‐acid glycoprotein in patients with congenital disorder of glycosylation type IA (CDG‐Ia)
- 10 April 2001
- journal article
- Published by Wiley in FEBS Letters
- Vol. 494 (3) , 232-235
- https://doi.org/10.1016/s0014-5793(01)02349-3
Abstract
Increased fucosylation of the type (sialyl) Lewis(x) was detected on the acute-phase plasma protein alpha(1)-acid glycoprotein (AGP) in patients with the congenital 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 GDP-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 fucosyltransferase activity in plasma suggest that a chronic hepatic inflammatory reaction has induced the increase in fucosylation.Keywords
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