Cloning of Genes for N-linked Oligosaccharide Synthesis in Endoplasmic Reticulum

  • Ken-ichi Nakayama
  • Yoshifumi Jigami


A congenital disorder of glycosylation syndrome (CDGS) is an autosomal recessive disorder on protein N-glycosylation. The mutated genes causing CDGS are found in N-linked oligosaccharide synthesis gene in the endoplasmic reticulum (ER) (Chantret et al. 2003; Frank et al. 2004). The N-glycosylation pathway in the ER is almost the same in any eukaryotic cells from yeast to human. Since most of the N-glycosylation genes and corresponding mutant strains have been isolated from yeast, we cloned and characterized human N-glycosylation genes in the ER by the phenotypic complementation of yeast N-glycosylation mutant cells.


Endoplasmic Reticulum Acid Phosphatase Schizosaccharomyces Pombe Human cDNA Library Glycosylation Defect 


Unable to display preview. Download preview PDF.

Unable to display preview. Download preview PDF.


  1. Chantret I, Dancourt J, Dupre T, Delenda C, Bucher S, Vuillaumier-Barrot S, Ogier de Baulny H, Peletan C, Danos O, Seta N, Durand G, Oriol R, Codogno P, Moore SE (2003) A deficiency in dolichyl-P-glucose:Glc1Man9GlcNAc2-PP-dolichyl alpha3-glucosyltransferase defines a new subtype of congenital disorders of glycosylation. J Biol Chem 278:9962–9971PubMedCrossRefGoogle Scholar
  2. Frank CG, Grubenmann CE, Eyaid W, Berger EG, Aebi M, Hennet T (2004) Identification and functional analysis of a defect in the human ALG9 gene: definition of congenital disorder of glycosylation type IL. Am J Hum Genet 75:146–150PubMedCrossRefGoogle Scholar
  3. Umeda K, Yoko-o T, Nakayama K, Suzuki T, Jigami Y (2000) Schizosaccharomyces pombe gmd3(+)/ alg11(+) is a functional homologue of Saccharomyces cerevisiae ALG11 which is involved in Nlinked oligosaccharide synthesis. Yeast 16:1261–1271PubMedCrossRefGoogle Scholar

Copyright information

© Springer 2008

Authors and Affiliations

  • Ken-ichi Nakayama
    • 1
  • Yoshifumi Jigami
    • 2
  1. 1.Health Technology Research CenterNational Institute of Advanced Industrial Science and Technology (AIST)Takamatsu, KagawaJapan
  2. 2.Research Institute for Cell EngineeringNational Institute of Advanced Industrial Science and Technology (AIST)Tsukuba, IbarakiJapan

Personalised recommendations