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 
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© 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

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