• Medientyp: E-Artikel
  • Titel: Increased α3‐fucosylation of α1‐acid glycoprotein in patients with congenital disorder of glycosylation type IA (CDG‐Ia)
  • Beteiligte: Van Dijk, Willem; Koeleman, Carolien; Van het Hof, Bert; Poland, Dennis; Jakobs, Cornelis; Jaeken, Jaak
  • Erschienen: Wiley, 2001
  • Erschienen in: FEBS Letters
  • Sprache: Englisch
  • DOI: 10.1016/s0014-5793(01)02349-3
  • ISSN: 0014-5793; 1873-3468
  • Schlagwörter: Cell Biology ; Genetics ; Molecular Biology ; Biochemistry ; Structural Biology ; Biophysics
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  • Beschreibung: <jats:p>Increased fucosylation of the type (sialyl) Lewis<jats:sup>x</jats:sup> was detected on the acute‐phase plasma protein α<jats:sub>1</jats:sub>‐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)‐<jats:sc>D</jats:sc>‐mannose is strongly decreased, and GDP‐<jats:sc>D</jats:sc>‐mannose is the direct precursor for GDP‐<jats:sc>L</jats:sc>‐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.</jats:p>
  • Zugangsstatus: Freier Zugang