Asparagine N-linked glycosylation (Homo sapiens)

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1-646, 4769, 7184-86110-11734-3790-926650-5238, 39103-1095420, 2197-10224-2661-63551528-3132794042, 4348, 4934-37118, 11915, 1966-686513, 1444118, 11973, 7469, 70110-11715, 1687-8958-6033118-1207111, 122717-1993-9675-7879118-1206422, 237-105572110-1177956982, 834145245356, 57118, 11980, 81cytosolendoplasmic reticulum quality control compartmentendoplasmic reticulum lumenGolgi lumen(Glc)1 (GlcNAc)2 (Man)9 (PP-Dol)1UDP-N-acetyl-D-glucosamineRFT1Sec23p:Sec24p Complexunfolded protein:(Glc)1 (GlcNAc)2 (Man)9 (Asn)1Dolichyl phosphateGMPPA/BALG2Sar1p:GTP:Sec23p:Sec24p:Sec13p:Sec31p ComplexUDP-D-galactoseunfolded protein:(Glc)1 (GlcNAc)2 (Man)9(GlcNAc)2 (Man)3 (PP-Dol)1dolichyl phosphate D-mannose(GlcNAc)2 (Man)5 (PP-Dol)1unfolded protein:(Glc)2 (GlcNAc)2 (Man)9 (Asn)1:malectinOrthophosphateMalectinGDPcalnexin/calreticulinOrthophosphateSec12Sar1p:GDP ComplexSar1p:GTP ComplexD-Mannose 1-phosphateL-GlutamateDolichol kinaseunfolded protein:(GlcNAc)2 (Man)7aa(GlcNAc)2 (Man)8 (Asn)1unfolded protein:(Glc)3 (GlcNAc)2 (Man)9 (Asn)1Dolichyl phosphate D-mannoseunfolded protein:(GlcNAc)2 (Man)8cSar1p:GTP:Sec23p:Sec24p(un)folded protein:(GlcNAc)2 (Man)9UDP-N-acetyl-D-glucosamineunfolded protein:(GlcNAc)2 (Man)7aaunfolded protein:glycan:chaperone:ERp57GDPD-glucosamine 6-phosphateAlpha-1,6-fucosyltransferase 8UDP-glucoseUDPDolichyl phosphateDolichyl diphosphate(GlcNAc)2 (Man)9 (PP-Dol)1CTPN-Acetyl-D-glucosamine 1-phosphate(Glc)2 (GlcNAc)2 (Man)9 (PP-Dol)1PGM3N-Acetyl-D-glucosamine 6-phosphateGNPNAT1 homodimerunfolded protein:(GlcNAc)2 (Man)8a(GlcNAc)4 (Man)3 (Asn)1Sec24ALG1GDP-alpha-D-mannoseGlycoprotein with bifurcating GlcNAc in position 3MPIDolichyl beta-D-glucosyl phosphateunfolded protein:(GlcNAc)2 (Man)8aGlycoprotein with sialic acidDolichyl phosphatealpha-D-glucoseunfolded protein:(Glc)2 (GlcNAc)2 (Man)9 (Asn)1N,N'-diacetylchitobiosyldiphosphodolicholB4GALT1-6 homodimersGlycoprotein with GlcNAc in position 5UGGT1/2GTPGDP-L-fucoseSAR1B GTP-binding proteinGDP(GlcNAc)3 (Man)5 (Asn)1SEC13-related proteinSar1p:Sec23p:Sec24p:Sec13p:Sec31p Complexpyrophosphate(GlcNAc)2 (Man)9cytidine 5'-diphosphateUMPcalreticulin/calnexinGlycoproteins with Man8 N-glycansUAP1 homodimerDolichyl beta-D-glucosyl phosphateCoA-SHGlycoproteins with Man8 N-glycans(GlcNAc)2 (Man)8 (PP-Dol)1GFPT1/2Dolichyl phosphateunfolded protein:glycan (no glucose)(GlcNAc)2 (Man)5 (Asn)1N-glycoproteinGlycoprotein with GlcNAc in position 4MAN1A1/A2/C1(GlcNAc)3 (Man)3 (Asn)1uridine 5'-diphosphateUDP-N-acetyl-D-glucosamine(GlcNAc)2 (Man)8 glycansdolichyl-phosphate mannosyltransferaseD-Fructose 6-phosphateALG6UDP-N-acetyl-D-glucosamineProtein transport protein Sec23AMGAT3ST8SIAsProtein transport protein Sec31Aglucosidase IIunfolded protein:(Glc)1 (GlcNAc)2 (Man)9 (Asn)1:chaperone:ERp57(Glc)3 (GlcNAc)2 (Man)9 (Asn)1DOLPP1ALG11OST complexDolichyl phosphateunfolded protein:(GlcNAc)2 (Man)8b(un)folded protein:(GlcNAc)2 (Man)9ALG99-O-acetylneuraminic acid(GlcNAc)2 (Man)6 (PP-Dol)1MANEAERp57LMAN1:MCFD2(un)folded protein:(GlcNAc)2 (Man)9Dolichyl phosphateGTPALG8GDPGlycoprotein with fucosyl alpha-1,6-GlcNAc(GlcNAc)2 (Man)7 (PP-Dol)1(GlcNAc)2 (Man)7bcALG12GDPST3GAL4MOGSD-mannose(GlcNAc)2 (Man)2 (PP-Dol)1MGAT5Dolichyl beta-D-glucosyl phosphateD-mannoseDolichyl beta-D-glucosyl phosphateEDEMD-Mannose 6-phosphateMAN1B1GDPL-Glutamine(Glc)3 (GlcNAc)2 (Man)9 (PP-Dol)1ALG3(GlcNAc)2 (Man)5 (PP-Dol)1alpha-D-glucoseGDP-alpha-D-mannoseOrthophosphateMGAT2Dolichyl phosphateMGAT4sMGAT1H2OALG10 homologueGlycoprotein with galactoseALG5unfolded proteinDPAGT1DolicholPMM1/2UTPN-acetyl-D-glucosaminyl-diphosphodolicholDolichyl phosphateunfolded protein:(Glc)1 (GlcNAc)2 (Man)9 (Asn)1:chaperoneALG13:ALG14 complexAcetyl-CoAbeta-D-mannosyldiacetylchitobiosyldiphosphodolicholST6GAL1MAN2:Zn2+glucosidase IIpyrophosphate


Description

N-linked glycosylation is the most important form of post-translational modification for proteins synthesized and folded in the Endoplasmic Reticulum (Stanley P et al, 2009). An early study in 1999 revealed that about 50% of the proteins in the Swiss-Prot database at the time were N-glycosylated (Apweiler R et al, 1999). It is now established that the majority of the proteins in the secretory pathway require glycosylation in order to achieve proper folding.
The addition of an N-glycan to a protein can have several roles (Shental-Bechor D and Levy Y, 2009). First, glycans enhance the solubility and stability of the proteins in the ER, the golgi and on the outside of the cell membrane, where the composition of the medium is strongly hydrophilic and where proteins, that are mostly hydrophobic, have difficulty folding properly. Second, N-glycans are used as signal molecules during the folding and transport process of the protein: they have the role of labels to determine when a protein must interact with a chaperon, be transported to the golgi, or targeted for degradation in case of major folding defects. Third, and most importantly, N-glycans on completely folded proteins are involved in a wide range of processes: they help determine the specificity of membrane receptors in innate immunity or in cell-to-cell interactions, they can change the properties of hormones and secreted proteins, or of the proteins in the vesicular system inside the cell.
All N-linked glycans are derived from a common 14-sugar oligosaccharide synthesized in the ER, which is attached co-translationally to a protein while this is being translated inside the reticulum. The process of the synthesis of this glycan, known as Synthesis of the N-glycan precursor or LLO, constitutes one of the most conserved pathways in eukaryotes, and has been also observed in some eubacteria. The attachment usually happens on an asparagine residue within the consensus sequence asparagine-X-threonine by an complex called oligosaccharyl transferase (OST).
After being attached to an unfolded protein, the glycan is used as a label molecule in the folding process (also known as Calnexin/Calreticulin cycle) (Lederkremer GZ, 2009). The majority of the glycoproteins in the ER require at least one glycosylated residue in order to achieve proper folding, even if it has been shown that a smaller portion of the proteins in the ER can be folded without this modification.
Once the glycoprotein has achieved proper folding, it is transported via the Cis-golgi through all the Golgi compartments, where the glycan is further modified according to the properties of the glycoprotein. This process involves relatively few enzymes but due to its combinatorial nature, can lead to several millions of different possible modifications. The exact topography of this network of reactions has not been established yet, representing one of the major challenges after the sequencing of the human genome (Hossler P et al, 2006).
Since N-glycosylation is involved in an great number of different processes, from cell-cell interaction to folding control, mutations in one of the genes involved in glycan assembly and/or modification can lead to severe development problems (often affecting the central nervous system). All the diseases in genes involved in glycosylation are collectively known as Congenital Disorders of Glycosylation (CDG) (Sparks SE et al, 2003), and classified as CDG type I for the genes in the LLO synthesis pathway, and CDG type II for the others.

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  85. Matthijs G, Schollen E, Pirard M, Budarf ML, Van Schaftingen E, Cassiman JJ.; ''PMM (PMM1), the human homologue of SEC53 or yeast phosphomannomutase, is localized on chromosome 22q13.''; PubMed Europe PMC Scholia
  86. Schallus T, Jaeckh C, Fehér K, Palma AS, Liu Y, Simpson JC, Mackeen M, Stier G, Gibson TJ, Feizi T, Pieler T, Muhle-Goll C.; ''Malectin: a novel carbohydrate-binding protein of the endoplasmic reticulum and a candidate player in the early steps of protein N-glycosylation.''; PubMed Europe PMC Scholia
  87. Angata K, Fukuda M.; ''Polysialyltransferases: major players in polysialic acid synthesis on the neural cell adhesion molecule.''; PubMed Europe PMC Scholia
  88. Kirchhausen T.; ''Three ways to make a vesicle.''; PubMed Europe PMC Scholia
  89. Grubenmann CE, Frank CG, Hülsmeier AJ, Schollen E, Matthijs G, Mayatepek E, Berger EG, Aebi M, Hennet T.; ''Deficiency of the first mannosylation step in the N-glycosylation pathway causes congenital disorder of glycosylation type Ik.''; PubMed Europe PMC Scholia
  90. Wang L, Liang Y, Li Z, Cai X, Zhang W, Wu G, Jin J, Fang Z, Yang Y, Zha X.; ''Increase in beta1-6 GlcNAc branching caused by N-acetylglucosaminyltransferase V directs integrin beta1 stability in human hepatocellular carcinoma cell line SMMC-7721.''; PubMed Europe PMC Scholia
  91. Wedgwood JF, Strominger JL.; ''Enzymatic activities in cultured human lymphocytes that dephosphorylate dolichyl pyrophosphate and dolichyl phosphate.''; PubMed Europe PMC Scholia
  92. Hull E, Sarkar M, Spruijt MP, Höppener JW, Dunn R, Schachter H.; ''Organization and localization to chromosome 5 of the human UDP-N-acetylglucosamine:alpha-3-D-mannoside beta-1,2-N-acetylglucosaminyltransferase I gene.''; PubMed Europe PMC Scholia
  93. Ohyama C, Smith PL, Angata K, Fukuda MN, Lowe JB, Fukuda M.; ''Molecular cloning and expression of GDP-D-mannose-4,6-dehydratase, a key enzyme for fucose metabolism defective in Lec13 cells.''; PubMed Europe PMC Scholia
  94. Kranz C, Jungeblut C, Denecke J, Erlekotte A, Sohlbach C, Debus V, Kehl HG, Harms E, Reith A, Reichel S, Grobe H, Hammersen G, Schwarzer U, Marquardt T.; ''A defect in dolichol phosphate biosynthesis causes a new inherited disorder with death in early infancy.''; PubMed Europe PMC Scholia
  95. Hardt B, Völker C, Mundt S, Salska-Navarro M, Hauptmann M, Bause E.; ''Human endo-alpha1,2-mannosidase is a Golgi-resident type II membrane protein.''; PubMed Europe PMC Scholia
  96. Schwarz M, Thiel C, Lübbehusen J, Dorland B, de Koning T, von Figura K, Lehle L, Körner C.; ''Deficiency of GDP-Man:GlcNAc2-PP-dolichol mannosyltransferase causes congenital disorder of glycosylation type Ik.''; PubMed Europe PMC Scholia
  97. Frank CG, Grubenmann CE, Eyaid W, Berger EG, Aebi M, Hennet T.; ''Identification and functional analysis of a defect in the human ALG9 gene: definition of congenital disorder of glycosylation type IL.''; PubMed Europe PMC Scholia
  98. Cantagrel V, Lefeber DJ, Ng BG, Guan Z, Silhavy JL, Bielas SL, Lehle L, Hombauer H, Adamowicz M, Swiezewska E, De Brouwer AP, Blümel P, Sykut-Cegielska J, Houliston S, Swistun D, Ali BR, Dobyns WB, Babovic-Vuksanovic D, van Bokhoven H, Wevers RA, Raetz CR, Freeze HH, Morava E, Al-Gazali L, Gleeson JG.; ''SRD5A3 is required for converting polyprenol to dolichol and is mutated in a congenital glycosylation disorder.''; PubMed Europe PMC Scholia
  99. Quirk S, Seley KL.; ''Substrate discrimination by the human GTP fucose pyrophosphorylase.''; PubMed Europe PMC Scholia
  100. Christianson JC, Shaler TA, Tyler RE, Kopito RR.; ''OS-9 and GRP94 deliver mutant alpha1-antitrypsin to the Hrd1-SEL1L ubiquitin ligase complex for ERAD.''; PubMed Europe PMC Scholia
  101. Züchner S, Dallman J, Wen R, Beecham G, Naj A, Farooq A, Kohli MA, Whitehead PL, Hulme W, Konidari I, Edwards YJ, Cai G, Peter I, Seo D, Buxbaum JD, Haines JL, Blanton S, Young J, Alfonso E, Vance JM, Lam BL, Peričak-Vance MA.; ''Whole-exome sequencing links a variant in DHDDS to retinitis pigmentosa.''; PubMed Europe PMC Scholia
  102. Lederkremer GZ.; ''Glycoprotein folding, quality control and ER-associated degradation.''; PubMed Europe PMC Scholia
  103. Thiel C, Schwarz M, Peng J, Grzmil M, Hasilik M, Braulke T, Kohlschütter A, von Figura K, Lehle L, Körner C.; ''A new type of congenital disorders of glycosylation (CDG-Ii) provides new insights into the early steps of dolichol-linked oligosaccharide biosynthesis.''; PubMed Europe PMC Scholia
  104. Wada Y, Sakamoto M.; ''Isolation of the human phosphomannomutase gene (PMM1) and assignment to chromosome 22q13.''; PubMed Europe PMC Scholia
  105. Montiel MD, Krzewinski-Recchi MA, Delannoy P, Harduin-Lepers A.; ''Molecular cloning, gene organization and expression of the human UDP-GalNAc:Neu5Acalpha2-3Galbeta-R beta1,4-N-acetylgalactosaminyltransferase responsible for the biosynthesis of the blood group Sda/Cad antigen: evidence for an unusual extended cytoplasmic domain.''; PubMed Europe PMC Scholia
  106. Burda P, Aebi M.; ''The ALG10 locus of Saccharomyces cerevisiae encodes the alpha-1,2 glucosyltransferase of the endoplasmic reticulum: the terminal glucose of the lipid-linked oligosaccharide is required for efficient N-linked glycosylation.''; PubMed Europe PMC Scholia
  107. Kranz C, Denecke J, Lehle L, Sohlbach K, Jeske S, Meinhardt F, Rossi R, Gudowius S, Marquardt T.; ''Congenital disorder of glycosylation type Ik (CDG-Ik): a defect of mannosyltransferase I.''; PubMed Europe PMC Scholia
  108. Intra J, Perotti ME, Pavesi G, Horner D.; ''Comparative and phylogenetic analysis of alpha-L-fucosidase genes.''; PubMed Europe PMC Scholia
  109. Song BL, Sever N, DeBose-Boyd RA.; ''Gp78, a membrane-anchored ubiquitin ligase, associates with Insig-1 and couples sterol-regulated ubiquitination to degradation of HMG CoA reductase.''; PubMed Europe PMC Scholia
  110. Wickramasinghe S, Medrano JF.; ''Primer on genes encoding enzymes in sialic acid metabolism in mammals.''; PubMed Europe PMC Scholia
  111. Aronson NN, Kuranda MJ.; ''Lysosomal degradation of Asn-linked glycoproteins.''; PubMed Europe PMC Scholia
  112. Kudo T, Nakagawa H, Takahashi M, Hamaguchi J, Kamiyama N, Yokoo H, Nakanishi K, Nakagawa T, Kamiyama T, Deguchi K, Nishimura S, Todo S.; ''N-glycan alterations are associated with drug resistance in human hepatocellular carcinoma.''; PubMed Europe PMC Scholia
  113. Nauseef WM, McCormick SJ, Clark RA.; ''Calreticulin functions as a molecular chaperone in the biosynthesis of myeloperoxidase.''; PubMed Europe PMC Scholia
  114. Furukawa T, Youssef EM, Yatsuoka T, Yokoyama T, Makino N, Inoue H, Fukushige S, Hoshi M, Hayashi Y, Sunamura M, Horii A.; ''Cloning and characterization of the human UDP-N-acetylglucosamine: alpha-1,3-D-mannoside beta-1,4-N-acetylglucosaminyltransferase IV-homologue (hGnT-IV-H) gene.''; PubMed Europe PMC Scholia
  115. Vleugels W, Schollen E, Foulquier F, Matthijs G.; ''Screening for OST deficiencies in unsolved CDG-I patients.''; PubMed Europe PMC Scholia
  116. Lubas WA, Spiro RG.; ''Evaluation of the role of rat liver Golgi endo-alpha-D-mannosidase in processing N-linked oligosaccharides.''; PubMed Europe PMC Scholia
  117. Kasapkara CS, Tümer L, Ezgü FS, Hasanoğlu A, Race V, Matthijs G, Jaeken J.; ''SRD5A3-CDG: a patient with a novel mutation.''; PubMed Europe PMC Scholia
  118. McKnight GL, Mudri SL, Mathewes SL, Traxinger RR, Marshall S, Sheppard PO, O'Hara PJ.; ''Molecular cloning, cDNA sequence, and bacterial expression of human glutamine:fructose-6-phosphate amidotransferase.''; PubMed Europe PMC Scholia
  119. Karaveg K, Siriwardena A, Tempel W, Liu ZJ, Glushka J, Wang BC, Moremen KW.; ''Mechanism of class 1 (glycosylhydrolase family 47) {alpha}-mannosidases involved in N-glycan processing and endoplasmic reticulum quality control.''; PubMed Europe PMC Scholia
  120. Tremblay LO, Herscovics A.; ''Characterization of a cDNA encoding a novel human Golgi alpha 1, 2-mannosidase (IC) involved in N-glycan biosynthesis.''; PubMed Europe PMC Scholia
  121. Angata K, Suzuki M, McAuliffe J, Ding Y, Hindsgaul O, Fukuda M.; ''Differential biosynthesis of polysialic acid on neural cell adhesion molecule (NCAM) and oligosaccharide acceptors by three distinct alpha 2,8-sialyltransferases, ST8Sia IV (PST), ST8Sia II (STX), and ST8Sia III.''; PubMed Europe PMC Scholia
  122. Schollen E, Dorland L, de Koning TJ, Van Diggelen OP, Huijmans JG, Marquardt T, Babovic-Vuksanovic D, Patterson M, Imtiaz F, Winchester B, Adamowicz M, Pronicka E, Freeze H, Matthijs G.; ''Genomic organization of the human phosphomannose isomerase (MPI) gene and mutation analysis in patients with congenital disorders of glycosylation type Ib (CDG-Ib).''; PubMed Europe PMC Scholia
  123. Sun L, Eklund EA, Chung WK, Wang C, Cohen J, Freeze HH.; ''Congenital disorder of glycosylation id presenting with hyperinsulinemic hypoglycemia and islet cell hyperplasia.''; PubMed Europe PMC Scholia
  124. Suzuki T, Yano K, Sugimoto S, Kitajima K, Lennarz WJ, Inoue S, Inoue Y, Emori Y.; ''Endo-beta-N-acetylglucosaminidase, an enzyme involved in processing of free oligosaccharides in the cytosol.''; PubMed Europe PMC Scholia
  125. Timson DJ, Reece RJ.; ''Identification and characterisation of human aldose 1-epimerase.''; PubMed Europe PMC Scholia
  126. Senderek J, Müller JS, Dusl M, Strom TM, Guergueltcheva V, Diepolder I, Laval SH, Maxwell S, Cossins J, Krause S, Muelas N, Vilchez JJ, Colomer J, Mallebrera CJ, Nascimento A, Nafissi S, Kariminejad A, Nilipour Y, Bozorgmehr B, Najmabadi H, Rodolico C, Sieb JP, Steinlein OK, Schlotter B, Schoser B, Kirschner J, Herrmann R, Voit T, Oldfors A, Lindbergh C, Urtizberea A, von der Hagen M, Hübner A, Palace J, Bushby K, Straub V, Beeson D, Abicht A, Lochmüller H.; ''Hexosamine biosynthetic pathway mutations cause neuromuscular transmission defect.''; PubMed Europe PMC Scholia
  127. Suzuki T, Huang C, Fujihira H.; ''The cytoplasmic peptide:N-glycanase (NGLY1) - Structure, expression and cellular functions.''; PubMed Europe PMC Scholia
  128. Gao XD, Tachikawa H, Sato T, Jigami Y, Dean N.; ''Alg14 recruits Alg13 to the cytoplasmic face of the endoplasmic reticulum to form a novel bipartite UDP-N-acetylglucosamine transferase required for the second step of N-linked glycosylation.''; PubMed Europe PMC Scholia
  129. Sasaki N, Manya H, Okubo R, Kobayashi K, Ishida H, Toda T, Endo T, Nishihara S.; ''beta4GalT-II is a key regulator of glycosylation of the proteins involved in neuronal development.''; PubMed Europe PMC Scholia
  130. Takahashi S, Hori K, Takahashi K, Ogasawara H, Tomatsu M, Saito K.; ''Effects of nucleotides on N-acetyl-d-glucosamine 2-epimerases (renin-binding proteins): comparative biochemical studies.''; PubMed Europe PMC Scholia
  131. Yamaguchi Y, Ikeda Y, Takahashi T, Ihara H, Tanaka T, Sasho C, Uozumi N, Yanagidani S, Inoue S, Fujii J, Taniguchi N.; ''Genomic structure and promoter analysis of the human alpha1, 6-fucosyltransferase gene (FUT8).''; PubMed Europe PMC Scholia
  132. Wolf MJ, Rush JS, Waechter CJ.; ''Golgi-enriched membrane fractions from rat brain and liver contain long-chain polyisoprenyl pyrophosphate phosphatase activity.''; PubMed Europe PMC Scholia
  133. Clarke LA.; ''The mucopolysaccharidoses: a success of molecular medicine.''; PubMed Europe PMC Scholia
  134. Petrescu AJ, Milac AL, Petrescu SM, Dwek RA, Wormald MR.; ''Statistical analysis of the protein environment of N-glycosylation sites: implications for occupancy, structure, and folding.''; PubMed Europe PMC Scholia
  135. Harduin-Lepers A, Vallejo-Ruiz V, Krzewinski-Recchi MA, Samyn-Petit B, Julien S, Delannoy P.; ''The human sialyltransferase family.''; PubMed Europe PMC Scholia
  136. Cameron HS, Szczepaniak D, Weston BW.; ''Expression of human chromosome 19p alpha(1,3)-fucosyltransferase genes in normal tissues. Alternative splicing, polyadenylation, and isoforms.''; PubMed Europe PMC Scholia
  137. Matthijs G, Schollen E, Pardon E, Veiga-Da-Cunha M, Jaeken J, Cassiman JJ, Van Schaftingen E.; ''Mutations in PMM2, a phosphomannomutase gene on chromosome 16p13, in carbohydrate-deficient glycoprotein type I syndrome (Jaeken syndrome).''; PubMed Europe PMC Scholia
  138. Bergfeld AK, Pearce OM, Diaz SL, Pham T, Varki A.; ''Metabolism of vertebrate amino sugars with N-glycolyl groups: elucidating the intracellular fate of the non-human sialic acid N-glycolylneuraminic acid.''; PubMed Europe PMC Scholia
  139. Harada Y, Masahara-Negishi Y, Suzuki T.; ''Cytosolic-free oligosaccharides are predominantly generated by the degradation of dolichol-linked oligosaccharides in mammalian cells.''; PubMed Europe PMC Scholia
  140. Winchester B.; ''Lysosomal metabolism of glycoproteins.''; PubMed Europe PMC Scholia
  141. O'Reilly MK, Zhang G, Imperiali B.; ''In vitro evidence for the dual function of Alg2 and Alg11: essential mannosyltransferases in N-linked glycoprotein biosynthesis.''; PubMed Europe PMC Scholia
  142. Pang H, Koda Y, Soejima M, Kimura H.; ''Identification of human phosphoglucomutase 3 (PGM3) as N-acetylglucosamine-phosphate mutase (AGM1).''; PubMed Europe PMC Scholia
  143. Alanen HI, Williamson RA, Howard MJ, Hatahet FS, Salo KE, Kauppila A, Kellokumpu S, Ruddock LW.; ''ERp27, a new non-catalytic endoplasmic reticulum-located human protein disulfide isomerase family member, interacts with ERp57.''; PubMed Europe PMC Scholia
  144. Kumar R, Yang J, Larsen RD, Stanley P.; ''Cloning and expression of N-acetylglucosaminyltransferase I, the medial Golgi transferase that initiates complex N-linked carbohydrate formation.''; PubMed Europe PMC Scholia
  145. Akama TO, Nakagawa H, Wong NK, Sutton-Smith M, Dell A, Morris HR, Nakayama J, Nishimura S, Pai A, Moremen KW, Marth JD, Fukuda MN.; ''Essential and mutually compensatory roles of {alpha}-mannosidase II and {alpha}-mannosidase IIx in N-glycan processing in vivo in mice.''; PubMed Europe PMC Scholia
  146. Brynedal B, Wojcik J, Esposito F, Debailleul V, Yaouanq J, Martinelli-Boneschi F, Edan G, Comi G, Hillert J, Abderrahim H.; ''MGAT5 alters the severity of multiple sclerosis.''; PubMed Europe PMC Scholia
  147. Kelleher DJ, Gilmore R.; ''An evolving view of the eukaryotic oligosaccharyltransferase.''; PubMed Europe PMC Scholia
  148. Hinderlich S, Berger M, Schwarzkopf M, Effertz K, Reutter W.; ''Molecular cloning and characterization of murine and human N-acetylglucosamine kinase.''; PubMed Europe PMC Scholia
  149. Oriol R, Martinez-Duncker I, Chantret I, Mollicone R, Codogno P.; ''Common origin and evolution of glycosyltransferases using Dol-P-monosaccharides as donor substrate.''; PubMed Europe PMC Scholia
  150. Chantret I, Dupré T, Delenda C, Bucher S, Dancourt J, Barnier A, Charollais A, Heron D, Bader-Meunier B, Danos O, Seta N, Durand G, Oriol R, Codogno P, Moore SE.; ''Congenital disorders of glycosylation type Ig is defined by a deficiency in dolichyl-P-mannose:Man7GlcNAc2-PP-dolichyl mannosyltransferase.''; PubMed Europe PMC Scholia
  151. Hansske B, Thiel C, Lübke T, Hasilik M, Höning S, Peters V, Heidemann PH, Hoffmann GF, Berger EG, von Figura K, Körner C.; ''Deficiency of UDP-galactose:N-acetylglucosamine beta-1,4-galactosyltransferase I causes the congenital disorder of glycosylation type IId.''; PubMed Europe PMC Scholia
  152. Granovsky M, Fata J, Pawling J, Muller WJ, Khokha R, Dennis JW.; ''Suppression of tumor growth and metastasis in Mgat5-deficient mice.''; PubMed Europe PMC Scholia
  153. Imbach T, Burda P, Kuhnert P, Wevers RA, Aebi M, Berger EG, Hennet T.; ''A mutation in the human ortholog of the Saccharomyces cerevisiae ALG6 gene causes carbohydrate-deficient glycoprotein syndrome type-Ic.''; PubMed Europe PMC Scholia
  154. Kämpf M, Absmanner B, Schwarz M, Lehle L.; ''Biochemical characterization and membrane topology of Alg2 from Saccharomyces cerevisiae as a bifunctional alpha1,3- and 1,6-mannosyltransferase involved in lipid-linked oligosaccharide biosynthesis.''; PubMed Europe PMC Scholia
  155. Mi Y, Fiete D, Baenziger JU.; ''Ablation of GalNAc-4-sulfotransferase-1 enhances reproduction by altering the carbohydrate structures of luteinizing hormone in mice.''; PubMed Europe PMC Scholia
  156. Tian H, Miyoshi E, Kawaguchi N, Shaker M, Ito Y, Taniguchi N, Tsujimoto M, Matsuura N.; ''The implication of N-acetylglucosaminyltransferase V expression in gastric cancer.''; PubMed Europe PMC Scholia
  157. Serafini-Cessi F, Conte R.; ''Precipitin reaction between Sda-active human Tamm-Horsfall glycoprotein and anti-Sda-serum.''; PubMed Europe PMC Scholia
  158. Takahashi T, Honda R, Nishikawa Y.; ''Cloning of the human cDNA which can complement the defect of the yeast mannosyltransferase I-deficient mutant alg 1.''; PubMed Europe PMC Scholia
  159. Maeda Y, Tanaka S, Hino J, Kangawa K, Kinoshita T.; ''Human dolichol-phosphate-mannose synthase consists of three subunits, DPM1, DPM2 and DPM3.''; PubMed Europe PMC Scholia
  160. Hiraoka N, Misra A, Belot F, Hindsgaul O, Fukuda M.; ''Molecular cloning and expression of two distinct human N-acetylgalactosamine 4-O-sulfotransferases that transfer sulfate to GalNAc beta 1-->4GlcNAc beta 1-->R in both N- and O-glycans.''; PubMed Europe PMC Scholia
  161. Tonetti M, Sturla L, Bisso A, Benatti U, De Flora A.; ''Synthesis of GDP-L-fucose by the human FX protein.''; PubMed Europe PMC Scholia
  162. Gonzalez DS, Karaveg K, Vandersall-Nairn AS, Lal A, Moremen KW.; ''Identification, expression, and characterization of a cDNA encoding human endoplasmic reticulum mannosidase I, the enzyme that catalyzes the first mannose trimming step in mammalian Asn-linked oligosaccharide biosynthesis.''; PubMed Europe PMC Scholia
  163. Arnold SM, Kaufman RJ.; ''The noncatalytic portion of human UDP-glucose: glycoprotein glucosyltransferase I confers UDP-glucose binding and transferase function to the catalytic domain.''; PubMed Europe PMC Scholia
  164. Saxena A, Yik JH, Weigel PH.; ''H2, the minor subunit of the human asialoglycoprotein receptor, trafficks intracellularly and forms homo-oligomers, but does not bind asialo-orosomucoid.''; PubMed Europe PMC Scholia
  165. Clarke JL, Watkins WM.; ''Expression of human alpha-l-fucosyltransferase gene homologs in monkey kidney COS cells and modification of potential fucosyltransferase acceptor substrates by an endogenous glycosidase.''; PubMed Europe PMC Scholia
  166. Ide Y, Miyoshi E, Nakagawa T, Gu J, Tanemura M, Nishida T, Ito T, Yamamoto H, Kozutsumi Y, Taniguchi N.; ''Aberrant expression of N-acetylglucosaminyltransferase-IVa and IVb (GnT-IVa and b) in pancreatic cancer.''; PubMed Europe PMC Scholia
  167. Cipollo JF, Trimble RB, Chi JH, Yan Q, Dean N.; ''The yeast ALG11 gene specifies addition of the terminal alpha 1,2-Man to the Man5GlcNAc2-PP-dolichol N-glycosylation intermediate formed on the cytosolic side of the endoplasmic reticulum.''; PubMed Europe PMC Scholia
  168. Apweiler R, Hermjakob H, Sharon N.; ''On the frequency of protein glycosylation, as deduced from analysis of the SWISS-PROT database.''; PubMed Europe PMC Scholia
  169. Kinoshita T, Inoue N.; ''Dissecting and manipulating the pathway for glycosylphos-phatidylinositol-anchor biosynthesis.''; PubMed Europe PMC Scholia
  170. Lo Presti L, Cabuy E, Chiricolo M, Dall'Olio F.; ''Molecular cloning of the human beta1,4 N-acetylgalactosaminyltransferase responsible for the biosynthesis of the Sd(a) histo-blood group antigen: the sequence predicts a very long cytoplasmic domain.''; PubMed Europe PMC Scholia
  171. Alcock F, Swanton E.; ''Mammalian OS-9 is upregulated in response to endoplasmic reticulum stress and facilitates ubiquitination of misfolded glycoproteins.''; PubMed Europe PMC Scholia
  172. Schaub BE, Berger B, Berger EG, Rohrer J.; ''Transition of galactosyltransferase 1 from trans-Golgi cisterna to the trans-Golgi network is signal mediated.''; PubMed Europe PMC Scholia
  173. Schmid M, Prajczer S, Gruber LN, Bertocchi C, Gandini R, Pfaller W, Jennings P, Joannidis M.; ''Uromodulin facilitates neutrophil migration across renal epithelial monolayers.''; PubMed Europe PMC Scholia
  174. Misago M, Liao YF, Kudo S, Eto S, Mattei MG, Moremen KW, Fukuda MN.; ''Molecular cloning and expression of cDNAs encoding human alpha-mannosidase II and a previously unrecognized alpha-mannosidase IIx isozyme.''; PubMed Europe PMC Scholia
  175. Ciccarelli FD, von Mering C, Suyama M, Harrington ED, Izaurralde E, Bork P.; ''Complex genomic rearrangements lead to novel primate gene function.''; PubMed Europe PMC Scholia
  176. Zhou H, Sun L, Li J, Xu C, Yu F, Liu Y, Ji C, He J.; ''The crystal structure of human GDP-L-fucose synthase.''; PubMed Europe PMC Scholia
  177. Pinho SS, Reis CA, Paredes J, Magalhães AM, Ferreira AC, Figueiredo J, Xiaogang W, Carneiro F, Gärtner F, Seruca R.; ''The role of N-acetylglucosaminyltransferase III and V in the post-transcriptional modifications of E-cadherin.''; PubMed Europe PMC Scholia
  178. Willems PJ, Seo HC, Coucke P, Tonlorenzi R, O'Brien JS.; ''Spectrum of mutations in fucosidosis.''; PubMed Europe PMC Scholia

History

View all...
CompareRevisionActionTimeUserComment
115049view16:59, 25 January 2021ReactomeTeamReactome version 75
113493view11:57, 2 November 2020ReactomeTeamReactome version 74
112693view16:08, 9 October 2020ReactomeTeamReactome version 73
101610view11:47, 1 November 2018ReactomeTeamreactome version 66
101147view21:33, 31 October 2018ReactomeTeamreactome version 65
100675view20:07, 31 October 2018ReactomeTeamreactome version 64
100225view16:52, 31 October 2018ReactomeTeamreactome version 63
99776view15:17, 31 October 2018ReactomeTeamreactome version 62 (2nd attempt)
99333view12:47, 31 October 2018ReactomeTeamreactome version 62
93956view13:47, 16 August 2017ReactomeTeamreactome version 61
93552view11:26, 9 August 2017ReactomeTeamreactome version 61
86654view09:23, 11 July 2016ReactomeTeamreactome version 56
83351view10:56, 18 November 2015ReactomeTeamVersion54
81512view13:03, 21 August 2015ReactomeTeamVersion53
78300view14:22, 24 December 2014EgonwGave an unlabeled protein a label matching its identifier ("4836523").
76985view08:27, 17 July 2014ReactomeTeamFixed remaining interactions
76690view12:05, 16 July 2014ReactomeTeamFixed remaining interactions
76016view10:07, 11 June 2014ReactomeTeamRe-fixing comment source
75725view11:19, 10 June 2014ReactomeTeamReactome 48 Update
75075view14:02, 8 May 2014AnweshaFixing comment source for displaying WikiPathways description
74857view15:15, 2 May 2014EgonwMarked a metabolite as a DataNode type="Metabolite"...
74722view08:48, 30 April 2014ReactomeTeamReactome46
44964view12:49, 6 October 2011MartijnVanIerselOntology Term : 'protein modification pathway' added !
42008view21:49, 4 March 2011MaintBotAutomatic update
39811view05:50, 21 January 2011MaintBotNew pathway

External references

DataNodes

View all...
NameTypeDatabase referenceComment
(Glc)1 (GlcNAc)2

(Man)9 (PP-Dol)1

Metabolite59081 (ChEBI)
(Glc)2 (GlcNAc)2

(Man)9 (PP-Dol)1

Metabolite53020 (ChEBI)
(Glc)3 (GlcNAc)2

(Man)9 (Asn)1

Metabolite59084 (ChEBI)
(Glc)3 (GlcNAc)2

(Man)9 (PP-Dol)1

Metabolite53019 (ChEBI)
(GlcNAc)2 (Man)2

(PP-Dol)1

Metabolite59085 (ChEBI)
(GlcNAc)2 (Man)3

(PP-Dol)1

Metabolite53742 (ChEBI)
(GlcNAc)2 (Man)5

(Asn)1

Metabolite59087 (ChEBI)
(GlcNAc)2 (Man)5

(PP-Dol)1

Metabolite53022 (ChEBI)
(GlcNAc)2 (Man)6

(PP-Dol)1

Metabolite53023 (ChEBI)
(GlcNAc)2 (Man)7

(PP-Dol)1

Metabolite59088 (ChEBI)
(GlcNAc)2 (Man)7bc Metabolite60637 (ChEBI)
(GlcNAc)2 (Man)8

(Asn)1

Metabolite59089 (ChEBI)
(GlcNAc)2 (Man)8

(PP-Dol)1

Metabolite59091 (ChEBI)
(GlcNAc)2 (Man)8

glycans

MetaboliteREACT_26078 (Reactome)
(GlcNAc)2 (Man)9 Metabolite59092 (ChEBI)
(GlcNAc)2 (Man)9

(PP-Dol)1

Metabolite59093 (ChEBI)
(GlcNAc)3 (Man)3

(Asn)1

Metabolite60615 (ChEBI)
(GlcNAc)3 (Man)5

(Asn)1

Metabolite60625 (ChEBI)
(GlcNAc)4 (Man)3

(Asn)1

Metabolite60651 (ChEBI)
(un)folded protein:

(GlcNAc)2 (Man)9

ComplexREACT_24075 (Reactome)
(un)folded protein:

(GlcNAc)2 (Man)9

ComplexREACT_24185 (Reactome)
(un)folded protein:

(GlcNAc)2 (Man)9

ComplexREACT_24760 (Reactome)
9-O-

acetylneuraminic acid

Metabolite28879 (ChEBI)
ALG1 ProteinQ9BT22 (UniProt)
ALG10 homologue UnknownREACT_22988 (Reactome)
ALG11 ProteinQ2TAA5 (UniProt)
ALG12 ProteinQ9BV10 (UniProt)
ALG13:ALG14

complex

UnknownREACT_22489 (Reactome)
ALG2 ProteinQ9H553 (UniProt)
ALG3 ProteinQ92685 (UniProt)
ALG5 ProteinQ9Y673 (UniProt)
ALG6 ProteinQ9Y672 (UniProt)
ALG8 ProteinQ9BVK2 (UniProt)
ALG9 ProteinQ9H6U8 (UniProt)
Acetyl-CoA Metabolite15351 (ChEBI)
Alpha-1,6-

fucosyltransferase 8

ProteinQ9BYC5 (UniProt)
B4GALT1-6

homodimers

UnknownREACT_26591 (Reactome)
CTP Metabolite17677 (ChEBI)
CoA-SH Metabolite15346 (ChEBI)
D-Fructose 6-

phosphate

Metabolite15946 (ChEBI)
D-Mannose 1-

phosphate

Metabolite35374 (ChEBI)
D-Mannose 6-

phosphate

Metabolite17369 (ChEBI)
D-glucosamine 6-

phosphate

Metabolite15873 (ChEBI)
D-mannose Metabolite4208 (ChEBI)
DOLPP1 ProteinQ86YN1 (UniProt)
DPAGT1 ProteinQ9H3H5 (UniProt)
Dolichol Metabolite16091 (ChEBI)
Dolichol kinase ProteinQ9UPQ8 (UniProt)
Dolichyl

diphosphate

Metabolite15750 (ChEBI)
Dolichyl beta-D-

glucosyl phosphate

Metabolite15812 (ChEBI)
Dolichyl phosphate Metabolite16214 (ChEBI)
Dolichyl phosphate D-

mannose

Metabolite15809 (ChEBI)
EDEM ProteinREACT_27025 (Reactome)
ERp57 ProteinP30101 (UniProt)
GDP Metabolite17552 (ChEBI)
GDP-L-fucose Metabolite17009 (ChEBI)
GDP-alpha-D-

mannose

Metabolite15820 (ChEBI)
GFPT1/2 UnknownREACT_22587 (Reactome)
GMPPA/B UnknownREACT_22600 (Reactome)
GNPNAT1

homodimer

ComplexREACT_22659 (Reactome)
GTP Metabolite15996 (ChEBI)
Glycoprotein with

GlcNAc in position 4

UnknownREACT_26694 (Reactome)
Glycoprotein with

GlcNAc in position 5

UnknownREACT_26240 (Reactome)
Glycoprotein with

bifurcating GlcNAc in position 3

UnknownREACT_25989 (Reactome)
Glycoprotein with

fucosyl alpha-1,6- GlcNAc

UnknownREACT_26726 (Reactome)
Glycoprotein with

galactose

UnknownREACT_26909 (Reactome)
Glycoprotein with

sialic acid

UnknownREACT_26541 (Reactome)
Glycoproteins with

Man8 N-glycans

ComplexREACT_25793 (Reactome)
Glycoproteins with

Man8 N-glycans

ComplexREACT_26233 (Reactome)
H2O Metabolite15377 (ChEBI)
L-Glutamate Metabolite16015 (ChEBI)
L-Glutamine Metabolite18050 (ChEBI)
LMAN1:MCFD2 ComplexREACT_26123 (Reactome)
MAN1A1/A2/C1 UnknownREACT_25706 (Reactome)
MAN1B1 ProteinQ9UKM7 (UniProt)
MAN2:Zn2+ UnknownREACT_26817 (Reactome)
MANEA ProteinQ5SRI9 (UniProt)
MGAT1 ProteinP26572 (UniProt)
MGAT2 ProteinQ10469 (UniProt)
MGAT3 ProteinQ09327 (UniProt)
MGAT4s UnknownREACT_26701 (Reactome)
MGAT5 ProteinQ09328 (UniProt)
MOGS ProteinQ13724 (UniProt)
MPI ProteinP34949 (UniProt)
Malectin ProteinQ14165 (UniProt)
N,N'-

diacetylchitobiosyldiph osphodolichol

Metabolite18341 (ChEBI)
N-Acetyl-D-

glucosamine 1- phosphate

Metabolite7125 (ChEBI)
N-Acetyl-D-

glucosamine 6- phosphate

Metabolite15784 (ChEBI)
N-acetyl-D-

glucosaminyl- diphosphodolichol

Metabolite18278 (ChEBI)
N-glycoprotein Metabolite59520 (ChEBI)
OST complex ComplexREACT_23228 (Reactome)
Orthophosphate Metabolite18367 (ChEBI)
PGM3 ProteinO95394 (UniProt)
PMM1/2 UnknownREACT_22680 (Reactome)
Protein transport

protein Sec23A

ProteinQ15436 (UniProt)
Protein transport

protein Sec31A

ProteinO94979 (UniProt)
RFT1 ProteinQ96AA3 (UniProt)
SAR1B GTP-binding

protein

ProteinQ9Y6B6 (UniProt)
SEC13-related

protein

ProteinP55735 (UniProt)
ST3GAL4 ProteinQ11206 (UniProt)
ST6GAL1 ProteinP15907 (UniProt)
ST8SIAs ProteinREACT_25611 (Reactome)
Sar1p:GDP Complex ComplexREACT_12715 (Reactome)
Sar1p:GTP Complex ComplexREACT_12713 (Reactome)
Sar1p:GTP:Sec23p:

Sec24p

ComplexREACT_13151 (Reactome)
Sar1p:GTP:Sec23p:

Sec24p:Sec13p: Sec31p Complex

ComplexREACT_13165 (Reactome)
Sar1p:Sec23p:

Sec24p:Sec13p: Sec31p Complex

ComplexREACT_12742 (Reactome)
Sec12 ProteinQ9HCU5 (UniProt)
Sec23p:Sec24p

Complex

ComplexREACT_13290 (Reactome)
Sec24 ProteinREACT_12982 (Reactome)
UAP1 homodimer ComplexREACT_22471 (Reactome)
UDP Metabolite17659 (ChEBI)
UDP-D-galactose Metabolite18307 (ChEBI)
UDP-N-acetyl-D-

glucosamine

Metabolite16264 (ChEBI)
UDP-glucose Metabolite18066 (ChEBI)
UGGT1/2 UnknownREACT_26186 (Reactome)
UMP Metabolite16695 (ChEBI)
UTP Metabolite15713 (ChEBI)
alpha-D-glucose Metabolite17925 (ChEBI)
beta-D-

mannosyldiacetylchitobi osyldiphosphodolichol

Metabolite18396 (ChEBI)
calnexin/calreticulin ComplexREACT_24766 (Reactome)
calreticulin/calnexin ProteinREACT_24126 (Reactome)
cytidine 5'-

diphosphate

Metabolite17239 (ChEBI)
dolichyl phosphate

D-mannose

UnknownC03862 (COMPOUND)
dolichyl-phosphate

mannosyltransferase

ComplexREACT_3881 (Reactome)
glucosidase II ComplexREACT_24609 (Reactome)
pyrophosphate Metabolite29888 (ChEBI)
unfolded protein UnknownREACT_18756 (Reactome)
unfolded protein:

(Glc)1 (GlcNAc)2 (Man)9

ComplexREACT_26238 (Reactome)
unfolded protein:

(Glc)1 (GlcNAc)2 (Man)9 (Asn)1

ComplexREACT_24493 (Reactome)
unfolded protein:

(Glc)1 (GlcNAc)2 (Man)9 (Asn)1: chaperone

ComplexREACT_24814 (Reactome)
unfolded protein:

(Glc)1 (GlcNAc)2 (Man)9 (Asn)1: chaperone:ERp57

ComplexREACT_24059 (Reactome)
unfolded protein:

(Glc)2 (GlcNAc)2 (Man)9 (Asn)1

ComplexREACT_24146 (Reactome)
unfolded protein:

(Glc)2 (GlcNAc)2 (Man)9 (Asn)1: malectin

ComplexREACT_24801 (Reactome)
unfolded protein:

(Glc)3 (GlcNAc)2 (Man)9 (Asn)1

ComplexREACT_22818 (Reactome)
unfolded protein:

(GlcNAc)2 (Man)7aa

ComplexREACT_26126 (Reactome)
unfolded protein:

(GlcNAc)2 (Man)7aa

ComplexREACT_27046 (Reactome)
unfolded protein:

(GlcNAc)2 (Man)8a

ComplexREACT_25760 (Reactome)
unfolded protein:

(GlcNAc)2 (Man)8a

ComplexREACT_26747 (Reactome)
unfolded protein:

(GlcNAc)2 (Man)8b

ComplexREACT_25730 (Reactome)
unfolded protein:

(GlcNAc)2 (Man)8c

ComplexREACT_26221 (Reactome)
unfolded protein:

glycan (no glucose)

ComplexREACT_24309 (Reactome)
unfolded protein:

glycan:chaperone: ERp57

ComplexREACT_24597 (Reactome)
uridine 5'-

diphosphate

Metabolite17659 (ChEBI)

Annotated Interactions

No annotated interactions

Retrieved from "https://classic.wikipathways.org/index.php/Pathway:WP1785"