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Congenital disorders of glycosylation: genetic model systems lead the way.
Journal article

Congenital disorders of glycosylation: genetic model systems lead the way.

  • Aebi M Institut für Mikrobiologie, ETH Zürich, Switzerland. aebi@micro.biol.ethz.ch
  • Hennet T
  • 2001-04-18
Published in:
  • Trends in cell biology. - 2001
Animals
Carbohydrates
Endoplasmic Reticulum
Glycosylation
Humans
Lipopolysaccharides
Metabolism, Inborn Errors
Models, Genetic
Saccharomyces cerevisiae
English N-linked glycosylation is the most frequent modification of secretory proteins in eukaryotic cells. The highly conserved glycosylation process is initiated in the endoplasmic reticulum (ER), where the Glc(3)Man(9)GlcNAc(2) oligosaccharide is assembled on the lipid carrier dolichylpyrophosphate and then transferred to selected asparagine residues of polypeptide chains. In recent years, several inherited human diseases, congenital disorders of glycosylation (CDG), have been associated with deficiencies in this pathway. The ER-associated glycosylation pathway has been studied in the budding yeast Saccharomyces cerevisiae, and this model system has been invaluable in elucidating the molecular basis of novel types of CDG.
Language
  • English
Open access status
closed
Identifiers
Persistent URL
https://fredi.hepvs.ch/global/documents/222658
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