The synthesis of complex N-glycans can be divided into three distinct stages. The first stage occurs primarily in the cytoplasm and rough endoplasmic reticulum, and involves the synthesis of Glc3Man9GlcNAc2-pyrophosphate-dolichol. The second stage begins with the transfer of GlcP3Man9GlcNAc2 from Glc3Man9GlcNAc2-pyrophosphate- dolichol to an Asn residue of the nascent glycoprotein followed by processing to Man5GlcNAc2-Asn-X. The third stage occurs primarily in the Golgi apparatus and starts with the action of GnT-I on Man5GlcNAc2-Asn-X followed by the removal of two mannose residues by mannosidase II to form the substrate for GnT-II (Fig. 1). GnT-II transfers GlcNAc from UDP-α-GlcNAc in the β1,2-linkage to the Manα1-6 arm of the N-glycan core, and is essential for normal complex N-glycan formation (Fig. 1).
Fig. 1

Reaction catalyzed by GnT-II (UDP-GlcNAc:Manα1-6R [GlcNAc to Manα1-6] β-1,2-N-acetylglucosaminyltransferase-II). R = 1-4GlcNAcβ1-4GlcNAc-Asn-X


Caenorhabditis Elegans Chinese Hamster Ovary Cell Autosomal Recessive Disease Bile Duct Carcinoma Mannose Residue 
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  1. Altmann F, Kornfeld G, Dalik T, Staudacher E, Glossl J (1993) Processing of asparagine-linked oligosaccharides in insect cells: N-acetylglucosaminyltransferase I and II activities in cultured lepidopteran cells. Glycobiology 3:619–625PubMedCrossRefGoogle Scholar
  2. Arata Y, Hirabayashi J, Kasai K (1997) Structure of the 32-kDa galectin gene of the nematode Caenorhabditis elegans. J Biol Chem 272:26669–26677PubMedCrossRefGoogle Scholar
  3. Bendiak B, Schachter H (1987a) Control of glycoprotein synthesis. XII. Purification of UDP-GlcNAc:α-D-mannoside β1-2-N-acetylglucosaminyltransferase II from rat liver. J Biol Chem 262:5775–5783Google Scholar
  4. Bendiak B, Schachter H (1987b) Control of glycoprotein synthesis. XIII. Kinetic mechanism, substrate specificity, and inhibition characteristics of UDP-G1CNAC:α-D-mannoside β1-2-N-acetylglucosarninyltransferase II from rat liver. J Biol Chem 262:5784–5790PubMedGoogle Scholar
  5. Buckhaults P, Chen L, Fregien N, Pierce M (1997) Transcriptional regulation of N-acetylglucosaminyltransferase V by the src oncogene. J Biol Chem 272:19575–19581PubMedCrossRefGoogle Scholar
  6. Campbell R, Tan J, Schachter H, Bendiak B, Marth J (1997) Targeted inactivation of the murine UDP-GlcNAc:alpha-6-D-mannoside betα-1,2-N-acetylglucosaminyltrans-ferase II gene. Glycobiology 7:1050Google Scholar
  7. Charuk JHM, Tan J, Bernardini M, Haddad S, Reithmeier RAF, Jaeken J, Schachter H (1995) Carbohydrate-deficient glycoprotein syndrome type II. An autosomal recessive N-acetylglucosaminyltransferase II deficiency different from typical hereditary erythroblastic multinuclearity, with a positive acidified-serum lysis test (HEMPAS). Eur J Biochem 230:797–805PubMedCrossRefGoogle Scholar
  8. Chen SH, Zhou SH, Tan J, Schachter H (1998) Transcriptional regulation of the human UDP-GlcNAc: alpha-6-D-mannoside beta-1-2-N-acetylglucosaminyltransferase II gene (MGAT2) which controls complex N-glycan synthesis. Glycoconjugate J 15:301–308CrossRefGoogle Scholar
  9. Chen SH, Zhou SH, Sarkar M, Spence AM, Schachter H (1999) Expression of three Caenorhabditis elegans N-acetylglucosaminyltransferase I genes during development. J Biol Chem 274:288–297PubMedCrossRefGoogle Scholar
  10. D’Agostaro GAF, Zingoni A, Moritz RL, Simpson RJ, Schachter H, Bendiak B (1995) Molecular cloning and expression of cDNA encoding the rat UDP-N-acetylglucosamine:alpha-6-D-mannoside betα-1,2-N-acetylglucosaminyltransferase II. J Biol Chem 270:15211–15221PubMedCrossRefGoogle Scholar
  11. DeBose-Boyd RA, Nyame AK, Cummings RD (1998) Molecular cloning and characterization of an alphα-1,3-fucosyltransferase, CEFT-1, from Caenorhabditis elegans. Glycobiology 8:905–PubMedCrossRefGoogle Scholar
  12. Dennis JW, Granovsky M, Warren CE (1999) Protein glycosylation in development and disease. Bioessays 21:412–421PubMedCrossRefGoogle Scholar
  13. Drickamer K, Dodd RB (1999) C-type lectin-like domains in Caenorhabditis elegans: predictions from the complete genome sequence. Glycobiology 9:1357–1369PubMedCrossRefGoogle Scholar
  14. Foster JM, Yudkin B, Lockyer AE, Roberts DB (1995) Cloning and sequence analysis of Gmll, a Drosophila melanogaster homologue of the cDNA encoding murine Golgi alpha-mannosidase II. Gene 154:183–186PubMedCrossRefGoogle Scholar
  15. Gottlieb C, Baenziger J, Kornfeld S (1975) Deficient uridine diphosphate-N-acetylglucosamine:glycoprotein N-acetylglucosaminyltransferase activity in a clone of Chinese hamster ovary cells with altered surface glycoproteins. J Biol Chem 250:3303–3309PubMedGoogle Scholar
  16. Hagen FK, Nehrke K (1998) cDNA cloning and expression of a family of UDP-N-acetyl-D-galactosamine:polypeptide N-acetylgalactosaminyltransferase sequence homologs from Caenorhabditis elegans. J Biol Chem 273:8268–8PubMedCrossRefGoogle Scholar
  17. Harpaz N, Schachter H (1980) Control of glycoprotein synthesis. IV. Bovine colostrum UDP-N-acetylglucosamine: α-D-mannoside β-2-N-acetylglucosaminyltransferase I. Separation from UDP-N-acetylglucosamine: α-D-mannoside β-2-N-acetylglucosaminyltransferase II, partial purification and substrate specificity. J Biol Chem 255:4885–4893PubMedGoogle Scholar
  18. Herman T, Horvitz HR (1999) Three proteins involved in Caenorhabditis elegans vulval invagination are similar to components of a glycosylation pathway. Proc Natl Acad Sci USA 96:974–979PubMedCrossRefGoogle Scholar
  19. Hirabayashi J, Ubukata T, Kasai K (1996) Purification and molecular characterization of a novel 16-kDa galectin from the nematode Caenorhabditis elegans. J Biol Chem 271:2497–2505PubMedCrossRefGoogle Scholar
  20. Ioffe E, Stanley P (1994) Mice lacking N-acetylglucosaminyltransferase I activity die at midgestation, revealing an essential role for complex or hybrid N-linked carbohydrates. Proc Natl Acad Sci USA 91:728–732PubMedCrossRefGoogle Scholar
  21. Jaeken J, Carchon H, Stibler H (1993) The carbohydrate-deficient glycoprotein syndromes— pre-Golgi and Golgi disorders? Glycobiology 3:423–428PubMedCrossRefGoogle Scholar
  22. Jaeken J, Schachter H, Carchon H, Decock P, Coddeville B, Spik G (1994) Carbohydrate deficient glycoprotein syndrome type II: a deficiency in Golgi localised N-acetylglucosaminyltransferase II. Arch Dis Child 71:123–127PubMedCrossRefGoogle Scholar
  23. Jaeken J, Spik G, Schachter H (1996) Carbohydrate-deficient glycoprotein syndrome Type II: an autosomal recessive disease due to mutations in the N-acetylglucosaminyl transferase II gene. In: Montreuil J, Vliegenthart JFG, Schachter H (eds) Glycoproteins and disease. Elsevier, Amsterdam, pp 457–467CrossRefGoogle Scholar
  24. Johnston IR, McGuire EJ, Jourdian GW, Roseman S (1966) Incorporation of N-acetyl-D-glucosamine into glycoproteins. J Biol Chem 241:5735–5737PubMedGoogle Scholar
  25. Jurado LA, Coloma A, Cruces J (1999) Identification of a human homolog of the Drosophila rotated abdomen gene (POMT1) encoding a putative protein O-mannosyl-transferase, and assignment to human chromosome 9q34.1. Genomics 58:171–180PubMedCrossRefGoogle Scholar
  26. Kang R, Saito H, Ihara Y, Miyoshi E, Koyama N, Sheng Y, Taniguchi N (1996) Transcriptional regulation of the N-acetylglucosaminyltransferase V gene in human bile duct carcinoma cells (HuCC-T1) is mediated by Ets-1. J Biol Chem 271:26706–26712PubMedCrossRefGoogle Scholar
  27. Kerscher S, Albert S, Wucherpfennig D, Heisenberg M, Schneuwly S (1995) Molecular and genetic analysis of the Drosophila mas-1 (mannosidase-1) gene which encodes a glycoprotein processing alphα-1,2-mannosidase. Dev Biol 168:613–626PubMedCrossRefGoogle Scholar
  28. Leeb T, Kriegesmann B, Baumgartner BG, Klett C, Yerle M, Hameister H, Brenig B (1997) Molecular cloning of the porcine betα-1,2-N-acetylglucosaminyltransferase II gene and assignment to chromosome 1q23-q27. BBA Gen Subj 1336:361–366CrossRefGoogle Scholar
  29. März L, Altmann F, Staudacher E, Kubelka V (1995) Protein glycosylation in insects. In: Montreuil J, Vliegenthart JFG, Schachter H (eds) Glycoproteins. Elsevier, Amsterdam, pp 543–563CrossRefGoogle Scholar
  30. McEver RP (1997) Selectin-carbohydrate interactions during inflammation and metastasis. Glycoconj J 14:585–591PubMedCrossRefGoogle Scholar
  31. Mendicino J, Chandrasekaran EV, Anumula KR, Davila M (1981) Isolation and properties of α-D-mannose:β-1,2-N-acetylglucosaminyltransferase from trachea mucosa. Biochemistry 20:967–976PubMedCrossRefGoogle Scholar
  32. Metzler M, Gertz A, Sarkar M, Schachter H, Schrader JW, Marth JD (1994) Complex asparagine-linked oligosaccharides are required for morphogenic events during postimplantation development. EMBO J 13:2056–2065PubMedGoogle Scholar
  33. Mucha J, Kappel S, Schachter H, Hane W, Glössl J (1995) Molecular cloning and characterization of cDNAs coding for N-acetylglucosaminyltransferases I and II from Xenopus laevis ovary. Glycoconj J 12:473Google Scholar
  34. Mulder H, Dideberg F, Schachter H, Spronk BA, De Jong-Brink M, Kamerling JP, Vliegenthart JFG (1995) In the biosynthesis of N-glycans in connective tissue of the snail Lymnaea stagnalis, incorporation of GlcNAc by beta-2-GlcNAc-transferase I is an essential prerequisite for the action of beta-2-GlcNAc-transferase II and beta-2-Xyl-transferase. Eur J Biochem 232:272–283PubMedCrossRefGoogle Scholar
  35. Narasimhan S, Stanley P, Schachter H (1977) Control of glycoprotein synthesis. II. Lectinresistant mutant containing only one of two distinct N-acetylglucosaminyltransferase activities present in wild type Chinese hamster ovary cells. J Biol Chem 252:3926–3933PubMedGoogle Scholar
  36. Narasimhan S, Harpaz N, Longmore G, Carver JP, Grey AA, Schachter H (1980) Control of glycoprotein synthesis. The purification by preparative high voltage electrophoresis in borate of glycopeptides containing high mannose and complex oligosaccharide chains linked to asparagine. J Biol Chem 255:4876–4884PubMedGoogle Scholar
  37. Oppenheimer CL, Eckhardt AE, Hill RL (1981) The non-identity of porcine N-acetylglucosaminyltransferases I and II. J Biol Chem 256:11477–11482PubMedGoogle Scholar
  38. Palcic MM, Heerze LD, Pierce M, Hindsgaul O (1988) The use of hydrophobic synthetic glycosides as acceptors in glycosyltransferase assays. Glycoconj J 5:49–63CrossRefGoogle Scholar
  39. Rabouille C, Kuntz DA, Lockyer A, Watson R, Signorelli T, Rose DR, van den Heuvel M, Roberts DB (1999) The Drosophila GMII gene encodes a Golgi α-mannosidase II. J Cell Sci 112:3319–3330PubMedGoogle Scholar
  40. Reck F (1995) Synthesis of uridine-5-propylamine derivatives and their use in affinity chromatography of N-acetylglucosaminyltransferases I and II. Carbohydr Res 276:321–335PubMedCrossRefGoogle Scholar
  41. Reck F, Springer M, Paulsen H, Brockhausen I, Sarkar M, Schachter H (1994a) Synthesis of tetrasaccharide analogues of the N-glycan substrate of beta-(1→2)-N-acetylglucosaminyltransferase II using trisaccharide precursors and recombinant beta-(1→2)-N-acetylglucosaminyltransferase I. Carbohydr Res 259:93–101PubMedCrossRefGoogle Scholar
  42. Reck F, Meinjohanns E, Springer M, Wilkens R, Vandorst JALM, Paulsen H, Moller G, Brockhausen I, Schachter H (1994b) Synthetic substrate analogues for UDP-GlcNAcMan alpha-1-6-R beta(1-2)-n-acetylglucosaminyltransferase II. Substrate specificity and inhibitors for the enzyme. Glycoconj J 11:210–216PubMedCrossRefGoogle Scholar
  43. Reck F, Meinjohanns E, Tan J, Grey AA, Paulsen H, Schachter H (1995) Synthesis of pentasaccharide analogues of the N-glycan substrates of N-acetylglucosaminyltrans-ferases III, IV and V using tetrasaccharide precursors and recombinant beta-(1→2)-N-acetylglucosaminyltransferase II. Carbohydr Res 275:221–229PubMedCrossRefGoogle Scholar
  44. Roberts DB, Mulvany WJ, Dwek RA, Rudd PM (1998) Mutant analysis reveals an alternative pathway for N-linked glycosylation in Drosophila melanogaster. Eur J Biochem 253:494–498PubMedCrossRefGoogle Scholar
  45. Schachter H (1986) Biosynthetic controls that determine the branching and microheterogeneity of protein-bound oligosaccharides. Biochem Cell Biol 64:163–181PubMedCrossRefGoogle Scholar
  46. Schachter H (1991) The “yellow brick road” to branched complex N-glycans. Glycobiology 1:453–461PubMedCrossRefGoogle Scholar
  47. Schachter H, Narasimhan S, Gleeson P, Vella G (1983) Control of branching during the biosynthesis of asparagine-linked oligosaccharides. Can J Biochem Cell Biol 61:1049–1066PubMedCrossRefGoogle Scholar
  48. Segel IH (1975) Enzyme kinetics. Wiley, New YorkGoogle Scholar
  49. Selleck SB (2000) Proteoglycans and pattern formation. Sugar chemistry meets developmental genetics. Trends in Genetics 16:206–212PubMedCrossRefGoogle Scholar
  50. Stanley P, Caillibot V, Siminovitch L (1975a) Selection and characterization of eight phenotypically distinct lines of lectin-resistant Chinese hamster ovary cells. Cell 6:121–128PubMedCrossRefGoogle Scholar
  51. Stanley P, Narasimhan S, Siminovitch L, Schachter H (1975b) Chinese hamster ovary cells selected for resistance to the cytotoxicity of phytohemagglutinin are deficient in a UDP-N-acetylglucosamine: glycoprotein N-acetylglucosaminyltransferase activity. Proc Natl Acad Sci USA 72:3323–3327PubMedCrossRefGoogle Scholar
  52. Tan J, D’Agostaro GAF, Bendiak B, Reck F, Sarkar M, Squire JA, Leong P, Schachter H (1995) The human UDP-N-acetylglucosamine: alpha-6-D-mannoside-betα-1,2-N-acetylglucosaminyltransferase II gene (MGAT2)—cloning of genomic DNA, localization to chromosome 14q21, expression in insect cells and purification of the recombinant protein. Eur J Biochem 231:317–328PubMedCrossRefGoogle Scholar
  53. Tan J, Dunn J, Jaeken J, Schachter H (1996) Mutations in the MGAT2 gene controlling complex N-glycan synthesis cause carbohydrate-deficient glycoprotein syndrome type II, an autosomal recessive disease with defective brain development. Am J Hum Genet 59:810–817PubMedGoogle Scholar
  54. Zhang W, Revers L, Pierce M, Schachter H (2000) Regulation of expression of the human betα-1,2-N-acetylglucosaminyltransferase II gene (MGAT2) by Ets transcription factors. Biochem J 347:511–518PubMedCrossRefGoogle Scholar

Copyright information

© Springer Japan 2002

Authors and Affiliations

  • Harry Schachter
    • 1
  1. 1.Program in Structural Biology and BiochemistryHospital for Sick ChildrenTorontoCanada

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