Cloning of Genes for N-linked Oligosaccharide Synthesis in Endoplasmic Reticulum
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.
KeywordsEndoplasmic Reticulum Acid Phosphatase Schizosaccharomyces Pombe Human cDNA Library Glycosylation Defect
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- 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