Glycosylation and related congenital defects (Homo sapiens)

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218, 191, 79Pathological roleof MAGT1 related tomental retardationis questionable.2dolichyl phosphateUDPbeta-D-glucoseUDP-N-acetyl-α-D-glucosamine dolichyl beta-D-mannosyl phosphate1215, 1612EndoplasmicReticulumGolgiApparatusDolichyl phosphateGMPPBGDP30.34Man5-GlcNac2-PP-DolDPM212, 14Man8-GlcNac2-PP-DolMan7-GlcNac2-PP-DolMan6-GlcNac2-PP-DolMan9-GlcNac2-PP-DolGlc3Man9-GlcNac2-PP-DolGlc2Man9-GlcNac2-PP-DolGlcMan9-GlcNac2-PP-DolGlc2Man9-GlcNac2-ProtGlc3Man9-GlcNac2-ProtMan3-GlcNac4-ProtMan3-GlcNac3-ProtSia2Gal2Man3-GlcNac4-ProtGal2Man3-GlcNac4-ProtSiaGalMan3-GlcNac3-ProtGDP-mannoseDol-P-ManMannose 6-PhosphateMannose 1-phosphateFructose 6-phosphate4420, 2130.131730.1430.1530.1630.630.330.1030.530.1030.3330.430.230.130.3130.3212Mannosyltransferase-1-deficiency-CDG-IkMannosyltransferase-2-deficiency-CDG-IiSteroid 5 alpha-reductase 2 deficiencyMannosyltransferase4-5 deficiency (ALG11-CDG-Ip)UDP-GlcNac:Dol-P-GlcNac-P transferase deficiency - IjDolichol kinase deficiency - CDG - ImMan5GIcNAc2-PP-DolDolichol-PPDolichol phosphateDolichol-PPPolyprenol reductase Chitobiosyldiphosphodolichol beta-mannosyltransferase6, 8, 10UDP-N-acetylglucosamine-dolichyl-phosphate N-acetylglucosaminephosphotransferaseGDP-Man:Man(3)GlcNAc(2)-PP-Dol alpha-1,2-mannosyltransferaseMan3GIcNAc2-PP-DolMan4GIcNAc2-PP-DolDolicholPolyprenolMan2GIcNAc2PP-DolMevalonate3ManGlcNAc2-PP-DolAlpha-1,3/1,6-mannosyltransferase ALG2GlcNAcGlcNAc2-PP-DolMannosyltransferase4-5 deficiency (ALG11-CDG-Ip)Mannosyltransferase6 deficiency (CDG-Ic)5Mannosyltransferase 7-9 deficiency (CDG-II)dolichyl phosphate11, 13dolichyl beta-D-mannosyl phosphateGlucosyltransferase 2deficiency (CDG-Ih)Magnesium transporter 1deficiency (MAGT1-CDG)N-acetylglucosaminyltransferase 2deficiency (CDG-IIa)


Description

Glycosylation is the most common postranslational modification. Congenital disorders of glycosylation (CDG) are mainly autosomal recessive defects in such a pathway. Generally, they count to metabolic disorders. Up to date about 50 CDGs have been identified and more are expected to be discovered. The majority of CDG cases show also neurological diseases. However, CDG commonly affect multiple organs. CDGs can be divided into four basic groups depending on where the glycosylation process occurs on the molecule. The group applicable to defects in N-glycosylation, which is depicted in this pathway, takes on 16 out of these 50 known CDGs. N-glycosylation processes spread over three cellular compartments - cytosol, endoplasmic reticulum and Golgi apparatus - wheres other groups are restricted to one. Moreover, the mortality rate of this specific group accounts for 20% within the first year. This, in combination with the limited treatment options for CDG, points out the relevance for further investigations of this pathway.

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Bibliography

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  1. Kadirvelraj R, Yang JY, Sanders JH, Liu L, Ramiah A, Prabhakar PK, Boons GJ, Wood ZA, Moremen KW; ''HumanN-acetylglucosaminyltransferase II substrate recognition uses a modular architecture that includes a convergent exosite.''; Proc Natl Acad Sci U S A, 2018 PubMed Europe PMC Scholia
  2. Tan J, D'Agostaro AF, Bendiak B, Reck F, Sarkar M, Squire JA, Leong P, Schachter H; ''The human UDP-N-acetylglucosamine: alpha-6-D-mannoside-beta-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, 1995 PubMed Europe PMC Scholia
  3. Piton A, Redin C, Mandel JL; ''XLID-causing mutations and associated genes challenged in light of data from large-scale human exome sequencing.''; Am J Hum Genet, 2013 PubMed Europe PMC Scholia
  4. Uemura M, Tamura K, Chung S, Honma S, Okuyama A, Nakamura Y, Nakagawa H; ''Novel 5 alpha-steroid reductase (SRD5A3, type-3) is overexpressed in hormone-refractory prostate cancer.''; Cancer Sci, 2008 PubMed Europe PMC Scholia
  5. Stagljar I, te Heesen S, Aebi M; ''New phenotype of mutations deficient in glucosylation of the lipid-linked oligosaccharide: cloning of the ALG8 locus.''; Proc Natl Acad Sci U S A, 1994 PubMed Europe PMC Scholia
  6. Buczkowska A, Swiezewska E, Lefeber DJ; ''Genetic defects in dolichol metabolism.''; J Inherit Metab Dis, 2015 PubMed Europe PMC Scholia
  7. Körner C, Knauer R, Stephani U, Marquardt T, Lehle L, von Figura K; ''Carbohydrate deficient glycoprotein syndrome type IV: deficiency of dolichyl-P-Man:Man(5)GlcNAc(2)-PP-dolichyl mannosyltransferase.''; EMBO J, 1999 PubMed Europe PMC Scholia
  8. 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.''; J Biol Chem, 2005 PubMed Europe PMC Scholia
  9. Schachter H, Narasimhan S, Gleeson P, Vella G; ''Glycosyltransferases involved in elongation of N-glycosidically linked oligosaccharides of the complex or N-acetyllactosamine type.''; Methods Enzymol, 1983 PubMed Europe PMC Scholia
  10. Miller BS, Freeze HH, Hoffmann GF, Sarafoglou K; ''Pubertal development in ALG6 deficiency (congenital disorder of glycosylation type Ic).''; Mol Genet Metab, 2011 PubMed Europe PMC Scholia
  11. Matthijs G, Schollen E, Bjursell C, Erlandson A, Freeze H, Imtiaz F, Kjaergaard S, Martinsson T, Schwartz M, Seta N, Vuillaumier-Barrot S, Westphal V, Winchester B; ''Mutations in PMM2 that cause congenital disorders of glycosylation, type Ia (CDG-Ia).''; Hum Mutat, 2000 PubMed Europe PMC Scholia
  12. Frank CG, Aebi M; ''ALG9 mannosyltransferase is involved in two different steps of lipid-linked oligosaccharide biosynthesis.''; Glycobiology, 2005 PubMed Europe PMC Scholia
  13. Watanabe R, Murakami Y, Marmor MD, Inoue N, Maeda Y, Hino J, Kangawa K, Julius M, Kinoshita T; ''Initial enzyme for glycosylphosphatidylinositol biosynthesis requires PIG-P and is regulated by DPM2.''; EMBO J, 2000 PubMed Europe PMC Scholia
  14. Lefeber DJ, Schönberger J, Morava E, Guillard M, Huyben KM, Verrijp K, Grafakou O, Evangeliou A, Preijers FW, Manta P, Yildiz J, Grünewald S, Spilioti M, van den Elzen C, Klein D, Hess D, Ashida H, Hofsteenge J, Maeda Y, van den Heuvel L, Lammens M, Lehle L, Wevers RA; ''Deficiency of Dol-P-Man synthase subunit DPM3 bridges the congenital disorders of glycosylation with the dystroglycanopathies.''; Am J Hum Genet, 2009 PubMed Europe PMC Scholia
  15. Kranz C, Denecke J, Lehrman MA, Ray S, Kienz P, Kreissel G, Sagi D, Peter-Katalinic J, Freeze HH, Schmid T, Jackowski-Dohrmann S, Harms E, Marquardt T; ''A mutation in the human MPDU1 gene causes congenital disorder of glycosylation type If (CDG-If).''; J Clin Invest, 2001 PubMed Europe PMC Scholia
  16. 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.''; J Clin Invest, 2002 PubMed Europe PMC Scholia
  17. Wu X, Rush JS, Karaoglu D, Krasnewich D, Lubinsky MS, Waechter CJ, Gilmore R, Freeze HH; ''Deficiency of UDP-GlcNAc:Dolichol Phosphate N-Acetylglucosamine-1 Phosphate Transferase (DPAGT1) causes a novel congenital disorder of Glycosylation Type Ij.''; Hum Mutat, 2003 PubMed Europe PMC Scholia
  18. Weinstein M, Schollen E, Matthijs G, Neupert C, Hennet T, Grubenmann CE, Frank CG, Aebi M, Clarke JT, Griffiths A, Seargeant L, Poplawski N; ''CDG-IL: an infant with a novel mutation in the ALG9 gene and additional phenotypic features.''; Am J Med Genet A, 2005 PubMed Europe PMC Scholia
  19. Cormier-Daire V, Amiel J, Vuillaumier-Barrot S, Tan J, Durand G, Munnich A, Le Merrer M, Seta N; ''Congenital disorders of glycosylation IIa cause growth retardation, mental retardation, and facialdysmorphism.''; J Med Genet, 2000 PubMed Europe PMC Scholia
  20. Garshasbi M, Hadavi V, Habibi H, Kahrizi K, Kariminejad R, Behjati F, Tzschach A, Najmabadi H, Ropers HH, Kuss AW; ''A defect in the TUSC3 gene is associated with autosomal recessive mental retardation.''; Am J Hum Genet, 2008 PubMed Europe PMC Scholia
  21. Quelhas D, Quental R, Vilarinho L, Amorim A, Azevedo L; ''Congenital disorder of glycosylation type Ia: searching for the origin of common mutations in PMM2.''; Ann Hum Genet, 2007 PubMed Europe PMC Scholia
  22. 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.''; Biochemistry, 2006 PubMed Europe PMC Scholia
  23. Mohorko E, Owen RL, Malojčić G, Brozzo MS, Aebi M, Glockshuber R; ''Structural basis of substrate specificity of human oligosaccharyl transferase subunit N33/Tusc3 and its role in regulating protein N-glycosylation.''; Structure, 2014 PubMed Europe PMC Scholia
  24. Molinari F, Foulquier F, Tarpey PS, Morelle W, Boissel S, Teague J, Edkins S, Futreal PA, Stratton MR, Turner G, Matthijs G, Gecz J, Munnich A, Colleaux L; ''Oligosaccharyltransferase-subunit mutations in nonsyndromic mental retardation.''; Am J Hum Genet, 2008 PubMed Europe PMC Scholia
  25. 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).''; Hum Mutat, 2000 PubMed Europe PMC Scholia
  26. Tan J, Dunn J, Jaeken J, Schachter H; ''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, 1996 PubMed Europe PMC Scholia
  27. Kranz C, Denecke J, Lehle L, Sohlbach K, Jeske S, Meinhardt F, Rossi R, Gudowius S, Marquardt T; ''''; , PubMed Europe PMC Scholia
  28. Rush ET, Baker CV, Rizzo WB; ''Dolichol kinase deficiency (DOLK-CDG): Two new cases and expansion of phenotype.''; Am J Med Genet A, 2017 PubMed Europe PMC Scholia
  29. Yoo J, Mashalidis EH, Kuk ACY, Yamamoto K, Kaeser B, Ichikawa S, Lee SY; ''GlcNAc-1-P-transferase-tunicamycin complex structure reveals basis for inhibition of N-glycosylation.''; Nat Struct Mol Biol, 2018 PubMed Europe PMC Scholia
  30. Sagami H, Swiezewska E, Shidoji Y; ''The history and recent advances in research of polyprenol and its derivatives.''; Biosci Biotechnol Biochem, 2018 PubMed Europe PMC Scholia
  31. Chantret I, Dancourt J, Dupré 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; ''A deficiency in dolichyl-P-glucose:Glc1Man9GlcNAc2-PP-dolichyl alpha3-glucosyltransferase defines a new subtype of congenital disorders of glycosylation.''; J Biol Chem, 2003 PubMed Europe PMC Scholia
  32. Dick HM, Strelioff MG, McMurchy KA; ''Pitfalls in early diagnosis of oral epidermoid carcinoma.''; J Can Dent Assoc (Tor), 1970 PubMed Europe PMC Scholia
  33. Blau, Nenad, Duran, Marinus, Gibson, K, Micheal, Dionsi-Vici, Carlo; ''Physician's Guide to the Diagnosis, Treatment, and Follow-Up of inherited Metabolic Diseases''; ISBN 978-3-642400337-8, 2014
  34. De Praeter CM, Gerwig GJ, Bause E, Nuytinck LK, Vliegenthart JF, Breuer W, Kamerling JP, Espeel MF, Martin JJ, De Paepe AM, Chan NW, Dacremont GA, Van Coster RN; ''A novel disorder caused by defective biosynthesis of N-linked oligosaccharides due to glucosidase I deficiency.''; Am J Hum Genet, 2000 PubMed Europe PMC Scholia
  35. 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.''; Am J Hum Genet, 2004 PubMed Europe PMC Scholia
  36. Rind N, Schmeiser V, Thiel C, Absmanner B, Lübbehusen J, Hocks J, Apeshiotis N, Wilichowski E, Lehle L, Körner C; ''A severe human metabolic disease caused by deficiency of the endoplasmatic mannosyltransferase hALG11 leads to congenital disorder of glycosylation-Ip.''; Hum Mol Genet, 2010 PubMed Europe PMC Scholia
  37. Grubenmann CE, Frank CG, Kjaergaard S, Berger EG, Aebi M, Hennet T; ''ALG12 mannosyltransferase defect in congenital disorder of glycosylation type lg.''; Hum Mol Genet, 2002 PubMed Europe PMC Scholia
  38. Niehues R, Hasilik M, Alton G, Körner C, Schiebe-Sukumar M, Koch HG, Zimmer KP, Wu R, Harms E, Reiter K, von Figura K, Freeze HH, Harms HK, Marquardt T; ''Carbohydrate-deficient glycoprotein syndrome type Ib. Phosphomannose isomerase deficiency and mannose therapy.''; J Clin Invest, 1998 PubMed Europe PMC Scholia
  39. Rodríguez Cruz PM, Palace J, Beeson D; ''The Neuromuscular Junction and Wide Heterogeneity of Congenital Myasthenic Syndromes.''; Int J Mol Sci, 2018 PubMed Europe PMC Scholia
  40. Maeda Y, Tanaka S, Hino J, Kangawa K, Kinoshita T; ''''; , PubMed Europe PMC Scholia
  41. Würde AE, Reunert J, Rust S, Hertzberg C, Haverkämper S, Nürnberg G, Nürnberg P, Lehle L, Rossi R, Marquardt T; ''Congenital disorder of glycosylation type Ij (CDG-Ij, DPAGT1-CDG): extending the clinical and molecular spectrum of a rare disease.''; Mol Genet Metab, 2012 PubMed Europe PMC Scholia
  42. Thiel C, Schwarz M, Peng J, Grzmil M, Hasilik M, Braulke T, Kohlschütter A, von Figura K, Lehle L, Körner C; ''''; , PubMed Europe PMC Scholia
  43. 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.''; Cell, 2010 PubMed Europe PMC Scholia
  44. Makridakis NM, di Salle E, Reichardt JK; ''Biochemical and pharmacogenetic dissection of human steroid 5 alpha-reductase type II.''; Pharmacogenetics, 2000 PubMed Europe PMC Scholia
  45. Frank CG, Grubenmann CE, Eyaid W, Berger EG, Aebi M, Hennet T; ''''; , PubMed Europe PMC Scholia
  46. Kelleher DJ, Karaoglu D, Mandon EC, Gilmore R; ''Oligosaccharyltransferase isoforms that contain different catalytic STT3 subunits have distinct enzymatic properties.''; Mol Cell, 2003 PubMed Europe PMC Scholia
  47. 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.''; Am J Hum Genet, 2007 PubMed Europe PMC Scholia
  48. Matthijs G, Schollen E, Heykants L, Grünewald S; ''Phosphomannomutase deficiency: the molecular basis of the classical Jaeken syndrome (CDGS type Ia).''; Mol Genet Metab, 1999 PubMed Europe PMC Scholia
  49. Parentini I, Cavallini G, Donati A, Gori Z, Bergamini E; ''Accumulation of dolichol in older tissues satisfies the proposed criteria to be qualified a biomarker of aging.''; J Gerontol A Biol Sci Med Sci, 2005 PubMed Europe PMC Scholia

History

View all...
CompareRevisionActionTimeUserComment
128247view23:49, 29 January 2024EweitzSoften disease color, standardize case
128246view23:46, 29 January 2024EweitzModified description
124518view09:51, 4 November 2022DeSlAdded missing Rhea IDs
124517view09:46, 4 November 2022DeSlFinalized book laebl conversion to gene names
124516view09:39, 4 November 2022DeSlStarted updating book labels to gene names for proteins
124515view09:35, 4 November 2022DeSlConverted several side metabolites connections to lines into anchor; increased mim-conversion width for interactions missing a Rhea ID
124514view09:24, 4 November 2022DeSlUpdated OMIM IDs to one structure
119311view13:14, 23 June 2021FinterlyAdded ISBN for book citation
108084view11:51, 28 November 2019FehrhartOntology Term : 'disease pathway' added !
107292view11:41, 19 September 2019EgonwRemove an empty ChEBI identifier
107174view14:26, 17 September 2019MaintBotChEBI identifier normalization
104484view05:30, 29 May 2019EgonwHarmonized the OMIM href URL pattern.
104277view12:19, 15 May 2019Fehrhartremoved "." from title
104247view18:48, 11 May 2019EgonwRemoved the RHEA: prefix from the identifier.
104032view17:44, 25 April 2019IreneHemelModified description
103562view09:54, 15 March 2019EviSchoenmakerALG14 changed from GeneProduct to Protein
103536view10:21, 13 March 2019EviSchoenmakerAdded alternative abbreviations under comments for the diseases
103388view15:22, 25 February 2019EviSchoenmakerLayout change
103387view14:31, 25 February 2019DeSlRemoved weird signs in lit. ref, part 2
103386view14:26, 25 February 2019DeSlRemoved weird signs in lit. ref, part 1
103374view17:56, 24 February 2019EviSchoenmakerCorrected OMIM links
103346view13:04, 22 February 2019DeSlUpdated Rhea for reaction connected to 30.11 (was undirected), added lit.refs for disease and protein.
103316view09:11, 22 February 2019EviSchoenmakerChanged identifers to primary ones as indicated by Jenkins
103291view15:47, 21 February 2019L DupuisAddition of ALG14 gene modulation to ALG13 based on literature
103273view16:46, 20 February 2019EviSchoenmakerCorrection of Rhea's on left side
103269view11:03, 20 February 2019EviSchoenmakerCorrection and addition of identifiers as well as enzymes
103254view15:37, 18 February 2019EviSchoenmakerDescription of some enzymes
103240view09:36, 18 February 2019EviSchoenmakerCorrected location of first two diseases
103239view09:28, 18 February 2019EviSchoenmakerAddition of general reference, and adjustement of comment in 30.1
103237view09:11, 18 February 2019EviSchoenmakerOntology Term : 'N-acetylglucosamine, N-acetylmannosamine and N-acetylneuraminic acid dissimilation pathway' added !
103236view09:08, 18 February 2019EviSchoenmakerOntology Term : 'altered carbohydrate metabolic pathway' added !
103235view09:07, 18 February 2019EviSchoenmakerOntology Term : 'autosomal recessive disease' added !
103234view09:06, 18 February 2019EviSchoenmakerOntology Term : 'inherited metabolic disorder' added !
103232view09:02, 18 February 2019EviSchoenmakerModified description
103230view08:53, 18 February 2019EviSchoenmakerColor change of disease boxes
103216view14:49, 17 February 2019DeSlTyped alpha to avoid weird characters.
103215view14:48, 17 February 2019DeSlChanged arrows for diseases to graphical lines, small layout changes
103180view15:01, 15 February 2019BrittPietersModified description
103178view14:55, 15 February 2019BrittPietersAdded Rhea descriptions
103162view14:23, 15 February 2019EviSchoenmakerAdjusted arrows and boxes
103161view14:21, 15 February 2019EviSchoenmakerMore layout changes
103160view14:20, 15 February 2019EviSchoenmakerEnzyme label into number and general layout
103155view13:19, 15 February 2019EviSchoenmakerAddition of diseases to the right section
103146view09:43, 15 February 2019EviSchoenmakerAddition of diseases right part
103140view08:56, 15 February 2019EviSchoenmakerAdjusted failed literature annotation
103137view08:48, 15 February 2019DeSlAdded info for litref.
103131view14:14, 14 February 2019EviSchoenmakerFurther literature addition to the left part
103130view13:05, 14 February 2019EviSchoenmakerAddition of references to cytosolic part
103129view12:31, 14 February 2019EviSchoenmakerOntology Term : 'congenital disorder of glycosylation' added !
103128view12:29, 14 February 2019EviSchoenmakerModified description

External references

DataNodes

View all...
NameTypeDatabase referenceComment
30.10ProteinQ9H6U8 (Uniprot-TrEMBL) Aka ;mannosyltransferase 7-9
30.13ProteinQ10469 (Uniprot-TrEMBL)
  • AKA MGAT2; N-acetylglucosaminyltransferase 2
  • Function"Plays an essential role in protein N-glycosylation. Catalyzes the transfer of N-acetylglucosamine (GlcNAc) onto the free terminal mannose moiety in the core structure of the nascent N-linked glycan chain, giving rise to the second branch in complex glycans." [https://www.uniprot.org/uniprot/Q10469]
30.14ProteinQ13724 (Uniprot-TrEMBL)
  • AKA GCS1; Glucosidase 1
  • Function:"Cleaves the distal alpha 1,2-linked glucose residue from the Glc3Man9GlcNAc2 oligosaccharide precursor in a highly specific manner." [https://www.uniprot.org/uniprot/Q13724]
30.15ProteinQ13454 (Uniprot-TrEMBL) AKA TUSC3; oligosaccharyltransferase subunit tusc 3
30.16ProteinQ9H0U3 (Uniprot-TrEMBL) Aka MAGT1; magnesium transporter 1
30.1ProteinO15305 (Uniprot-TrEMBL) AKA PMM@, phosphomannomutase 2
30.2ProteinP34949 (Uniprot-TrEMBL) AKA MPI, phosphomannose isomerase
30.31ProteinO60762 (Uniprot-TrEMBL) AKA DPM1, GDP-Man:Dol-P mannosyltransferase subunit 1
30.32ProteinQ9P2X0 (Uniprot-TrEMBL)
  • AKA DPM3, GDP-Man:Dol-P mannosyltransferase 3
  • "Stabilizer subunit of the dolichol-phosphate mannose (DPM) synthase complex; tethers catalytic subunit DPM1 to the ER" [https://www.uniprot.org/uniprot/Q9P2X0]
30.33ProteinO75352 (Uniprot-TrEMBL) AKA MPDU1; Dol-P-Man utilization 1
30.34ProteinP15291 (Uniprot-TrEMBL) AKA B4GALT1; beta-1,4-galactosyltransferase 1
30.3ProteinQ9Y672 (Uniprot-TrEMBL) Aka ALG6; glucosyltransferase 1.
30.4ProteinQ92685 (Uniprot-TrEMBL)
  • AKA ALG3, mannosyltransferase 6
  • Function:"Adds the first Dol-P-Man derived mannose in an alpha-1,3 linkage to Man5GlcNAc2-PP-Dol" [https://www.uniprot.org/uniprot/Q92685]
30.5ProteinQ9BV10 (Uniprot-TrEMBL) AKA ALG12, Mannosyltransferase 8
30.6ProteinQ9BVK2 (Uniprot-TrEMBL) AKA ALG8; Glucosyltransferase 2
Alpha-1,3/1,6- mannosyltransferase ALG2ProteinQ9H553 (Uniprot-TrEMBL)
Chitobiosyldiphosphodolichol beta-mannosyltransferaseProteinQ9BT22 (Uniprot-TrEMBL)
DPM2ProteinO94777 (Uniprot-TrEMBL)
Dol-P-ManMetaboliteCHEBI:58211 (ChEBI)
Dolichol phosphateMetaboliteCHEBI:4693 (ChEBI)
Dolichol-PPMetaboliteCHEBI:4691 (ChEBI) In CheBi known as Dolichol diphosphate
DolicholMetaboliteCHEBI:16091 (ChEBI)
Dolichyl phosphateMetaboliteCHEBI:57683 (ChEBI)
Fructose 6-phosphateMetaboliteCHEBI:61527 (ChEBI)
GDP-Man:Man(3)GlcNAc(2)-PP- Dol alpha-1,2-mannosyltransferaseProteinQ2TAA5 (Uniprot-TrEMBL)
GDP-mannoseMetaboliteCHEBI:57527 (ChEBI)
GDPMetaboliteCHEBI:58189 (ChEBI)
GMPPBProteinQ9Y5P6 (Uniprot-TrEMBL) AKA mannose-1-phosphate guanylyltransferase [https://en.wikipedia.org/wiki/Mannose-1-phosphate_guanylyltransferase]
Gal2Man3-GlcNac4-ProtMetabolite
Glc2Man9-GlcNac2-PP-DolMetaboliteCHEBI:132522 (ChEBI)
Glc2Man9-GlcNac2-ProtMetaboliteCHEBI:59082 (ChEBI)
Glc3Man9-GlcNac2-PP-DolMetabolite
Glc3Man9-GlcNac2-ProtMetaboliteCHEBI:132537 (ChEBI)
GlcMan9-GlcNac2-PP-DolMetaboliteCHEBI:132521 (ChEBI)
GlcNAc2-PP-DolMetaboliteCHEBI:57269 (ChEBI) Aka N-acetylglucosamine
GlcNAcMetaboliteCHEBI:506227 (ChEBI) Aka N-acetylglucosamine
Man2GIcNAc2PP-DolMetaboliteCHEBI:132510 (ChEBI)
Man3-GlcNac3-ProtMetaboliteCHEBI:60615 (ChEBI)
Man3-GlcNac4-ProtMetaboliteCHEBI:60615 (ChEBI)
Man3GIcNAc2-PP-DolMetaboliteCHEBI:132511 (ChEBI)
Man4GIcNAc2-PP-DolMetabolite
Man5-GlcNac2-PP-DolMetaboliteCHEBI:132516 (ChEBI)
Man5GIcNAc2-PP-DolMetaboliteCHEBI:132515 (ChEBI)
Man6-GlcNac2-PP-DolMetabolite
Man7-GlcNac2-PP-DolMetaboliteCHEBI:132517 (ChEBI)
Man8-GlcNac2-PP-DolMetaboliteCHEBI:132519 (ChEBI)
Man9-GlcNac2-PP-DolMetaboliteCHEBI:132520 (ChEBI)
ManGlcNAc2-PP-Dol MetaboliteCHEBI:58472 (ChEBI)
Mannose 1-phosphateMetaboliteCHEBI:58409 (ChEBI)
Mannose 6-PhosphateMetaboliteCHEBI:58735 (ChEBI)
MevalonateMetaboliteCHEBI:345 (ChEBI)
Polyprenol reductase ProteinQ9H8P0 (Uniprot-TrEMBL)
PolyprenolMetaboliteCHEBI:26199 (ChEBI)
Sia2Gal2Man3-GlcNac4-ProtMetabolite
SiaGalMan3-GlcNac3-ProtMetabolite
UDP-N-acetyl -α-D-glucosamine MetaboliteCHEBI:57705 (ChEBI)
UDP-N-acetylglucosamine-dolichyl-phosphate N-acetylglucosaminephosphotransferaseProteinQ9H3H5 (Uniprot-TrEMBL)
UDPMetaboliteCHEBI:58223 (ChEBI)
beta-D-glucoseMetaboliteCHEBI:15903 (ChEBI)
dolichyl

beta-D-mannosyl

phosphate		MetaboliteCHEBI:58211 (ChEBI)
dolichyl phosphateMetaboliteCHEBI:57683 (ChEBI)

Annotated Interactions

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SourceTargetTypeDatabase referenceComment
Fructose 6-phosphateMannose 6-Phosphatemim-conversionRHEA:12358 (Rhea)
GDP-mannoseDol-P-ManArrowRHEA:21185 (Rhea)
Glc3Man9-GlcNac2-ProtGlc2Man9-GlcNac2-Protmim-conversionRHEA:55989 (Rhea)
GlcMan9-GlcNac2-PP-DolGlc2Man9-GlcNac2-PP-Dolmim-conversionRHEA:31308 (Rhea)
GlcNAc2-PP-DolManGlcNAc2-PP-Dol mim-conversionRHEA:13865 (Rhea)
GlcNAc2-PP-Dolmim-conversionRHEA:13289 (Rhea)
Man3-GlcNac3-ProtMan3-GlcNac4-Protmim-conversionRHEA:12942 (Rhea)
Man3GIcNAc2-PP-DolMan4GIcNAc2-PP-Dolmim-conversionRHEA:29523 (Rhea)
Man4GIcNAc2-PP-DolMan5GIcNAc2-PP-Dolmim-conversionRHEA:29523 (Rhea)
Man5GIcNAc2-PP-DolMan5-GlcNac2-PP-Dolmim-conversionRHEA:29528 (Rhea)
Man7-GlcNac2-PP-DolMan8-GlcNac2-PP-Dolmim-conversionRHEA:29536 (Rhea)
Man8-GlcNac2-PP-DolMan9-GlcNac2-PP-Dolmim-conversionRHEA:29540 (Rhea)
Man9-GlcNac2-PP-DolGlcMan9-GlcNac2-PP-Dolmim-conversionRHEA:30636 (Rhea)
ManGlcNAc2-PP-Dol Man2GIcNAc2PP-Dolmim-conversionRHEA:29515 (Rhea)
Mannose 6-PhosphateMannose 1-phosphatemim-conversionRHEA:11142 (Rhea)
MevalonatePolyprenolmim-conversionRHEA:54384 (Rhea)
beta-D-glucosemim-conversionRHEA:55989 (Rhea)
dolichyl

beta-D-mannosyl

phosphate		mim-conversionRHEA:29528 (Rhea)
dolichyl

beta-D-mannosyl

phosphate		mim-conversionRHEA:29540 (Rhea)
dolichyl phosphatemim-conversionRHEA:29528 (Rhea)
dolichyl phosphatemim-conversionRHEA:29540 (Rhea)
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