title: Increased alpha3-fucosylation of alpha(1)-acid glycoprotein in patients with congenital disorder of glycosylation type IA (CDG-Ia).
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.
authors: Glycoimmunology Group, Department of Molecular Cell Biology, Reaearch Institute Immunology and Inflammatory Disease, VU Medical Centre, Van der Boechorststraat 7, 1081 BT Amsterdam, The Netherlands. w.van_dijk.medchem@med.vu.nl
authors: W
authors: W
authors: Van Dijk
authors: C
authors: C
authors: Koeleman
authors: B
authors: B
authors: Van het Hof
authors: D
authors: D
authors: Poland
authors: C
authors: C
authors: Jakobs
authors: J
authors: J
authors: Jaeken
mesh_terms: D000581:Amidohydrolases / Q000378:metabolism; D002239:Carbohydrate Metabolism, Inborn Errors / Q000097:blood / Q000145:classification / Q000201:enzymology / Q000378:metabolism*; D005643:Fucose / Q000031:analogs & derivatives / Q000378:metabolism*; D005647:Fucosyltransferases / Q000097:blood / Q000378:metabolism; D006031:Glycosylation; D006801:Humans; D037102:Lectins / Q000378:metabolism; D008970:Molecular Weight; D009439:Neuraminidase / Q000378:metabolism; D009961:Orosomucoid / Q000737:chemistry / Q000378:metabolism*; D043524:Peptide-N4-(N-acetyl-beta-glucosaminyl) Asparagine Amidase; D011134:Polysaccharides / Q000737:chemistry / Q000378:metabolism
chemical_list: D037102:Lectins; D009961:Orosomucoid; D011134:Polysaccharides; D005643:Fucose; D005647:Fucosyltransferases; D009439:Neuraminidase; D000581:Amidohydrolases; D043524:Peptide-N4-(N-acetyl-beta-glucosaminyl) Asparagine Amidase