Glycosaminoglycan metabolism (Homo sapiens)

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1. Schmidt B, Selmer T, Ingendoh A, von Figura K.; ''A novel amino acid modification in sulfatases that is defective in multiple sulfatase deficiency.''; PubMed Europe PMC Scholia
2. Venkatachalam KV, Akita H, Strott CA.; ''Molecular cloning, expression, and characterization of human bifunctional 3'-phosphoadenosine 5'-phosphosulfate synthase and its functional domains.''; PubMed Europe PMC Scholia
3. Lin X, Barber DL.; ''A calcineurin homologous protein inhibits GTPase-stimulated Na-H exchange.''; PubMed Europe PMC Scholia
4. Hästbacka J, de la Chapelle A, Mahtani MM, Clines G, Reeve-Daly MP, Daly M, Hamilton BA, Kusumi K, Trivedi B, Weaver A.; ''The diastrophic dysplasia gene encodes a novel sulfate transporter: positional cloning by fine-structure linkage disequilibrium mapping.''; PubMed Europe PMC Scholia
5. Izumikawa T, Koike T, Shiozawa S, Sugahara K, Tamura J, Kitagawa H.; ''Identification of chondroitin sulfate glucuronyltransferase as chondroitin synthase-3 involved in chondroitin polymerization: chondroitin polymerization is achieved by multiple enzyme complexes consisting of chondroitin synthase family members.''; PubMed Europe PMC Scholia
6. Crawford BE, Olson SK, Esko JD, Pinhal MA.; ''Cloning, Golgi localization, and enzyme activity of the full-length heparin/heparan sulfate-glucuronic acid C5-epimerase.''; PubMed Europe PMC Scholia
7. Guo S, Sato T, Shirane K, Furukawa K.; ''Galactosylation of N-linked oligosaccharides by human beta-1,4-galactosyltransferases I, II, III, IV, V, and VI expressed in Sf-9 cells.''; PubMed Europe PMC Scholia
8. Spicer AP, Olson JS, McDonald JA.; ''Molecular cloning and characterization of a cDNA encoding the third putative mammalian hyaluronan synthase.''; PubMed Europe PMC Scholia
9. Uyama T, Kitagawa H, Tamura Ji J, Sugahara K.; ''Molecular cloning and expression of human chondroitin N-acetylgalactosaminyltransferase: the key enzyme for chain initiation and elongation of chondroitin/dermatan sulfate on the protein linkage region tetrasaccharide shared by heparin/heparan sulfate.''; PubMed Europe PMC Scholia
10. Voglmeir J, Voglauer R, Wilson IB.; ''XT-II, the second isoform of human peptide-O-xylosyltransferase, displays enzymatic activity.''; PubMed Europe PMC Scholia
11. Kreamer BL, Siegel FL, Gourley GR.; ''A novel inhibitor of beta-glucuronidase: L-aspartic acid.''; PubMed Europe PMC Scholia
12. Schulz T, Schumacher U, Prehm P.; ''Hyaluronan export by the ABC transporter MRP5 and its modulation by intracellular cGMP.''; PubMed Europe PMC Scholia
13. O'Dowd BF, Cumming DA, Gravel RA, Mahuran D.; ''Oligosaccharide structure and amino acid sequence of the major glycopeptides of mature human beta-hexosaminidase.''; PubMed Europe PMC Scholia
14. Watanabe K, Yamaguchi Y.; ''Molecular identification of a putative human hyaluronan synthase.''; PubMed Europe PMC Scholia
15. Habuchi H, Miyake G, Nogami K, Kuroiwa A, Matsuda Y, Kusche-Gullberg M, Habuchi O, Tanaka M, Kimata K.; ''Biosynthesis of heparan sulphate with diverse structures and functions: two alternatively spliced forms of human heparan sulphate 6-O-sulphotransferase-2 having different expression patterns and properties.''; PubMed Europe PMC Scholia
16. Alper SL, Sharma AK.; ''The SLC26 gene family of anion transporters and channels.''; PubMed Europe PMC Scholia
17. Götting C, Müller S, Schöttler M, Schön S, Prante C, Brinkmann T, Kuhn J, Kleesiek K.; ''Analysis of the DXD motifs in human xylosyltransferase I required for enzyme activity.''; PubMed Europe PMC Scholia
18. Fan X, Zhang H, Zhang S, Bagshaw RD, Tropak MB, Callahan JW, Mahuran DJ.; ''Identification of the gene encoding the enzyme deficient in mucopolysaccharidosis IIIC (Sanfilippo disease type C).''; PubMed Europe PMC Scholia
19. Tiedemann K, Larsson T, Heinegård D, Malmström A.; ''The glucuronyl C5-epimerase activity is the limiting factor in the dermatan sulfate biosynthesis.''; PubMed Europe PMC Scholia
20. McCormick C, Duncan G, Goutsos KT, Tufaro F.; ''The putative tumor suppressors EXT1 and EXT2 form a stable complex that accumulates in the Golgi apparatus and catalyzes the synthesis of heparan sulfate.''; PubMed Europe PMC Scholia
21. Duncan MB, Liu M, Fox C, Liu J.; ''Characterization of the N-deacetylase domain from the heparan sulfate N-deacetylase/N-sulfotransferase 2.''; PubMed Europe PMC Scholia
22. Scott HS, Blanch L, Guo XH, Freeman C, Orsborn A, Baker E, Sutherland GR, Morris CP, Hopwood JJ.; ''Cloning of the sulphamidase gene and identification of mutations in Sanfilippo A syndrome.''; PubMed Europe PMC Scholia
23. Girard JP, Baekkevold ES, Amalric F.; ''Sulfation in high endothelial venules: cloning and expression of the human PAPS synthetase.''; PubMed Europe PMC Scholia
24. Iwai T, Inaba N, Naundorf A, Zhang Y, Gotoh M, Iwasaki H, Kudo T, Togayachi A, Ishizuka Y, Nakanishi H, Narimatsu H.; ''Molecular cloning and characterization of a novel UDP-GlcNAc:GalNAc-peptide beta1,3-N-acetylglucosaminyltransferase (beta 3Gn-T6), an enzyme synthesizing the core 3 structure of O-glycans.''; PubMed Europe PMC Scholia
25. Beesley CE, Jackson M, Young EP, Vellodi A, Winchester BG.; ''Molecular defects in Sanfilippo syndrome type B (mucopolysaccharidosis IIIB).''; PubMed Europe PMC Scholia
26. Funderburgh JL.; ''Keratan sulfate: structure, biosynthesis, and function.''; PubMed Europe PMC Scholia
27. Culty M, Miyake K, Kincade PW, Sikorski E, Butcher EC, Underhill C.; ''The hyaluronate receptor is a member of the CD44 (H-CAM) family of cell surface glycoproteins.''; PubMed Europe PMC Scholia
28. Robertson DA, Freeman C, Nelson PV, Morris CP, Hopwood JJ.; ''Human glucosamine-6-sulfatase cDNA reveals homology with steroid sulfatase.''; PubMed Europe PMC Scholia
29. Shyjan AM, Heldin P, Butcher EC, Yoshino T, Briskin MJ.; ''Functional cloning of the cDNA for a human hyaluronan synthase.''; PubMed Europe PMC Scholia
30. Pacheco B, Maccarana M, Malmström A.; ''Dermatan 4-O-sulfotransferase 1 is pivotal in the formation of iduronic acid blocks in dermatan sulfate.''; PubMed Europe PMC Scholia
31. Kang HG, Evers MR, Xia G, Baenziger JU, Schachner M.; ''Molecular cloning and expression of an N-acetylgalactosamine-4-O-sulfotransferase that transfers sulfate to terminal and non-terminal beta 1,4-linked N-acetylgalactosamine.''; PubMed Europe PMC Scholia
32. Fransson LA, Belting M, Jönsson M, Mani K, Moses J, Oldberg A.; ''Biosynthesis of decorin and glypican.''; PubMed Europe PMC Scholia
33. Kitagawa H, Tone Y, Tamura J, Neumann KW, Ogawa T, Oka S, Kawasaki T, Sugahara K.; ''Molecular cloning and expression of glucuronyltransferase I involved in the biosynthesis of the glycosaminoglycan-protein linkage region of proteoglycans.''; PubMed Europe PMC Scholia
34. Okajima T, Fukumoto S, Furukawa K, Urano T.; ''Molecular basis for the progeroid variant of Ehlers-Danlos syndrome. Identification and characterization of two mutations in galactosyltransferase I gene.''; PubMed Europe PMC Scholia
35. Shworak NW, Liu J, Petros LM, Zhang L, Kobayashi M, Copeland NG, Jenkins NA, Rosenberg RD.; ''Multiple isoforms of heparan sulfate D-glucosaminyl 3-O-sulfotransferase. Isolation, characterization, and expression of human cdnas and identification of distinct genomic loci.''; PubMed Europe PMC Scholia
36. Almeida R, Levery SB, Mandel U, Kresse H, Schwientek T, Bennett EP, Clausen H.; ''Cloning and expression of a proteoglycan UDP-galactose:beta-xylose beta1,4-galactosyltransferase I. A seventh member of the human beta4-galactosyltransferase gene family.''; PubMed Europe PMC Scholia
37. Müller S, Schöttler M, Schön S, Prante C, Brinkmann T, Kuhn J, Götting C, Kleesiek K.; ''Human xylosyltransferase I: functional and biochemical characterization of cysteine residues required for enzymic activity.''; PubMed Europe PMC Scholia
38. Aikawa J, Esko JD.; ''Molecular cloning and expression of a third member of the heparan sulfate/heparin GlcNAc N-deacetylase/ N-sulfotransferase family.''; PubMed Europe PMC Scholia
39. Bernard MA, Hall CE, Hogue DA, Cole WG, Scott A, Snuggs MB, Clines GA, Lüdecke HJ, Lovett M, Van Winkle WB, Hecht JT.; ''Diminished levels of the putative tumor suppressor proteins EXT1 and EXT2 in exostosis chondrocytes.''; PubMed Europe PMC Scholia
40. Asp NG, Dahlqvist A, Koldovský O.; ''Human small-intestinal beta-galactosidases. Separation and characterization of one lactase and one hetero beta-galactosidase.''; PubMed Europe PMC Scholia
41. Kobayashi M, Sugumaran G, Liu J, Shworak NW, Silbert JE, Rosenberg RD.; ''Molecular cloning and characterization of a human uronyl 2-sulfotransferase that sulfates iduronyl and glucuronyl residues in dermatan/chondroitin sulfate.''; PubMed Europe PMC Scholia
42. Valstar MJ, Bertoli-Avella AM, Wessels MW, Ruijter GJ, de Graaf B, Olmer R, Elfferich P, Neijs S, Kariminejad R, Suheyl Ezgü F, Tokatli A, Czartoryska B, Bosschaart AN, van den Bos-Terpstra F, Puissant H, Bürger F, Omran H, Eckert D, Filocamo M, Simeonov E, Willems PJ, Wevers RA, Niermeijer MF, Halley DJ, Poorthuis BJ, van Diggelen OP.; ''Mucopolysaccharidosis type IIID: 12 new patients and 15 novel mutations.''; PubMed Europe PMC Scholia
43. Basu SS, Basu M, Li Z, Basu S.; ''Characterization of two glycolipid: alpha 2-3sialyltransferases, SAT-3 (CMP-NeuAc:nLcOse4Cer alpha 2-3sialyltransferase) and SAT-4 (CMP-NeuAc:GgOse4Cer alpha 2-3sialyltransferase), from human colon carcinoma (Colo 205) cell line.''; PubMed Europe PMC Scholia
44. Kamiyama S, Suda T, Ueda R, Suzuki M, Okubo R, Kikuchi N, Chiba Y, Goto S, Toyoda H, Saigo K, Watanabe M, Narimatsu H, Jigami Y, Nishihara S.; ''Molecular cloning and identification of 3'-phosphoadenosine 5'-phosphosulfate transporter.''; PubMed Europe PMC Scholia
45. Huang C, Zhou J, Wu S, Shan Y, Teng S, Yu L.; ''Cloning and tissue distribution of the human B3GALT7 gene, a member of the beta1,3-Glycosyltransferase family.''; PubMed Europe PMC Scholia
46. Harris EN, Weigel JA, Weigel PH.; ''Endocytic function, glycosaminoglycan specificity, and antibody sensitivity of the recombinant human 190-kDa hyaluronan receptor for endocytosis (HARE).''; PubMed Europe PMC Scholia
47. Oshima A, Yoshida K, Shimmoto M, Fukuhara Y, Sakuraba H, Suzuki Y.; ''Human beta-galactosidase gene mutations in morquio B disease.''; PubMed Europe PMC Scholia
48. Aikawa J, Grobe K, Tsujimoto M, Esko JD.; ''Multiple isozymes of heparan sulfate/heparin GlcNAc N-deacetylase/GlcN N-sulfotransferase. Structure and activity of the fourth member, NDST4.''; PubMed Europe PMC Scholia
49. Rong J, Habuchi H, Kimata K, Lindahl U, Kusche-Gullberg M.; ''Expression of heparan sulphate L-iduronyl 2-O-sulphotransferase in human kidney 293 cells results in increased D-glucuronyl 2-O-sulphation.''; PubMed Europe PMC Scholia
50. Ishida N, Kuba T, Aoki K, Miyatake S, Kawakita M, Sanai Y.; ''Identification and characterization of human Golgi nucleotide sugar transporter SLC35D2, a novel member of the SLC35 nucleotide sugar transporter family.''; PubMed Europe PMC Scholia
51. Kim YJ, Kim KS, Kim SH, Kim CH, Ko JH, Choe IS, Tsuji S, Lee YC.; ''Molecular cloning and expression of human Gal beta 1,3GalNAc alpha 2,3-sialytransferase (hST3Gal II).''; PubMed Europe PMC Scholia
52. Chruszcz M, Laidler P, Monkiewicz M, Ortlund E, Lebioda L, Lewinski K.; ''Crystal structure of a covalent intermediate of endogenous human arylsulfatase A.''; PubMed Europe PMC Scholia
53. Yada T, Gotoh M, Sato T, Shionyu M, Go M, Kaseyama H, Iwasaki H, Kikuchi N, Kwon YD, Togayachi A, Kudo T, Watanabe H, Narimatsu H, Kimata K.; ''Chondroitin sulfate synthase-2. Molecular cloning and characterization of a novel human glycosyltransferase homologous to chondroitin sulfate glucuronyltransferase, which has dual enzymatic activities.''; PubMed Europe PMC Scholia
54. Hossler P, Goh LT, Lee MM, Hu WS.; ''GlycoVis: visualizing glycan distribution in the protein N-glycosylation pathway in mammalian cells.''; PubMed Europe PMC Scholia
55. Scott HS, Litjens T, Nelson PV, Thompson PR, Brooks DA, Hopwood JJ, Morris CP.; ''Identification of mutations in the alpha-L-iduronidase gene (IDUA) that cause Hurler and Scheie syndromes.''; PubMed Europe PMC Scholia
56. Gotoh M, Sato T, Akashima T, Iwasaki H, Kameyama A, Mochizuki H, Yada T, Inaba N, Zhang Y, Kikuchi N, Kwon YD, Togayachi A, Kudo T, Nishihara S, Watanabe H, Kimata K, Narimatsu H.; ''Enzymatic synthesis of chondroitin with a novel chondroitin sulfate N-acetylgalactosaminyltransferase that transfers N-acetylgalactosamine to glucuronic acid in initiation and elongation of chondroitin sulfate synthesis.''; PubMed Europe PMC Scholia
57. Shworak NW, Liu J, Fritze LM, Schwartz JJ, Zhang L, Logeart D, Rosenberg RD.; ''Molecular cloning and expression of mouse and human cDNAs encoding heparan sulfate D-glucosaminyl 3-O-sulfotransferase.''; PubMed Europe PMC Scholia
58. Kitagawa H, Paulson JC.; ''Cloning and expression of human Gal beta 1,3(4)GlcNAc alpha 2,3-sialyltransferase.''; PubMed Europe PMC Scholia
59. Bourguignon LY, Singleton PA, Diedrich F, Stern R, Gilad E.; ''CD44 interaction with Na+-H+ exchanger (NHE1) creates acidic microenvironments leading to hyaluronidase-2 and cathepsin B activation and breast tumor cell invasion.''; PubMed Europe PMC Scholia
60. Kitagawa H, Uyama T, Sugahara K.; ''Molecular cloning and expression of a human chondroitin synthase.''; PubMed Europe PMC Scholia
61. Kolbinger F, Streiff MB, Katopodis AG.; ''Cloning of a human UDP-galactose:2-acetamido-2-deoxy-D-glucose 3beta-galactosyltransferase catalyzing the formation of type 1 chains.''; PubMed Europe PMC Scholia
62. Erickson M, Stern R.; ''Chain gangs: new aspects of hyaluronan metabolism.''; PubMed Europe PMC Scholia
63. Kitagawa H, Fujita M, Ito N, Sugahara K.; ''Molecular cloning and expression of a novel chondroitin 6-O-sulfotransferase.''; PubMed Europe PMC Scholia
64. Pang T, Su X, Wakabayashi S, Shigekawa M.; ''Calcineurin homologous protein as an essential cofactor for Na+/H+ exchangers.''; PubMed Europe PMC Scholia
65. Lukatela G, Krauss N, Theis K, Selmer T, Gieselmann V, von Figura K, Saenger W.; ''Crystal structure of human arylsulfatase A: the aldehyde function and the metal ion at the active site suggest a novel mechanism for sulfate ester hydrolysis.''; PubMed Europe PMC Scholia
66. Shiraishi N, Natsume A, Togayachi A, Endo T, Akashima T, Yamada Y, Imai N, Nakagawa S, Koizumi S, Sekine S, Narimatsu H, Sasaki K.; ''Identification and characterization of three novel beta 1,3-N-acetylglucosaminyltransferases structurally related to the beta 1,3-galactosyltransferase family.''; PubMed Europe PMC Scholia
67. Weber B, Guo XH, Wraith JE, Cooper A, Kleijer WJ, Bunge S, Hopwood JJ.; ''Novel mutations in Sanfilippo A syndrome: implications for enzyme function.''; PubMed Europe PMC Scholia
68. Togayachi A, Akashima T, Ookubo R, Kudo T, Nishihara S, Iwasaki H, Natsume A, Mio H, Inokuchi J, Irimura T, Sasaki K, Narimatsu H.; ''Molecular cloning and characterization of UDP-GlcNAc:lactosylceramide beta 1,3-N-acetylglucosaminyltransferase (beta 3Gn-T5), an essential enzyme for the expression of HNK-1 and Lewis X epitopes on glycolipids.''; PubMed Europe PMC Scholia
69. Pang J, Zhang S, Yang P, Hawkins-Lee B, Zhong J, Zhang Y, Ochoa B, Agundez JA, Voelckel MA, Fisher RB, Gu W, Xiong WC, Mei L, She JX, Wang CY.; ''Loss-of-function mutations in HPSE2 cause the autosomal recessive urofacial syndrome.''; PubMed Europe PMC Scholia
70. Kakuda S, Shiba T, Ishiguro M, Tagawa H, Oka S, Kajihara Y, Kawasaki T, Wakatsuki S, Kato R.; ''Structural basis for acceptor substrate recognition of a human glucuronyltransferase, GlcAT-P, an enzyme critical in the biosynthesis of the carbohydrate epitope HNK-1.''; PubMed Europe PMC Scholia
71. Tumova S, Woods A, Couchman JR.; ''Heparan sulfate proteoglycans on the cell surface: versatile coordinators of cellular functions.''; PubMed Europe PMC Scholia
72. Fukuta M, Inazawa J, Torii T, Tsuzuki K, Shimada E, Habuchi O.; ''Molecular cloning and characterization of human keratan sulfate Gal-6-sulfotransferase.''; PubMed Europe PMC Scholia
73. Assmann V, Marshall JF, Fieber C, Hofmann M, Hart IR.; ''The human hyaluronan receptor RHAMM is expressed as an intracellular protein in breast cancer cells.''; PubMed Europe PMC Scholia
74. Banerji S, Ni J, Wang SX, Clasper S, Su J, Tammi R, Jones M, Jackson DG.; ''LYVE-1, a new homologue of the CD44 glycoprotein, is a lymph-specific receptor for hyaluronan.''; PubMed Europe PMC Scholia
75. Pang T, Hisamitsu T, Mori H, Shigekawa M, Wakabayashi S.; ''Role of calcineurin B homologous protein in pH regulation by the Na+/H+ exchanger 1: tightly bound Ca2+ ions as important structural elements.''; PubMed Europe PMC Scholia
76. McKenzie E, Tyson K, Stamps A, Smith P, Turner P, Barry R, Hircock M, Patel S, Barry E, Stubberfield C, Terrett J, Page M.; ''Cloning and expression profiling of Hpa2, a novel mammalian heparanase family member.''; PubMed Europe PMC Scholia
77. Lopez LC, Youakim A, Evans SC, Shur BD.; ''Evidence for a molecular distinction between Golgi and cell surface forms of beta 1,4-galactosyltransferase.''; PubMed Europe PMC Scholia
78. Harris EN, Kyosseva SV, Weigel JA, Weigel PH.; ''Expression, processing, and glycosaminoglycan binding activity of the recombinant human 315-kDa hyaluronic acid receptor for endocytosis (HARE).''; PubMed Europe PMC Scholia
79. Scott HS, Anson DS, Orsborn AM, Nelson PV, Clements PR, Morris CP, Hopwood JJ.; ''Human alpha-L-iduronidase: cDNA isolation and expression.''; PubMed Europe PMC Scholia
80. Habuchi H, Kobayashi M, Kimata K.; ''Molecular characterization and expression of heparan-sulfate 6-sulfotransferase. Complete cDNA cloning in human and partial cloning in Chinese hamster ovary cells.''; PubMed Europe PMC Scholia
81. Li JP, Gong F, El Darwish K, Jalkanen M, Lindahl U.; ''Characterization of the D-glucuronyl C5-epimerase involved in the biosynthesis of heparin and heparan sulfate.''; PubMed Europe PMC Scholia
82. Goldshmidt O, Nadav L, Aingorn H, Irit C, Feinstein N, Ilan N, Zamir E, Geiger B, Vlodavsky I, Katz BZ.; ''Human heparanase is localized within lysosomes in a stable form.''; PubMed Europe PMC Scholia
83. Ishida H, Togayachi A, Sakai T, Iwai T, Hiruma T, Sato T, Okubo R, Inaba N, Kudo T, Gotoh M, Shoda J, Tanaka N, Narimatsu H.; ''A novel beta1,3-N-acetylglucosaminyltransferase (beta3Gn-T8), which synthesizes poly-N-acetyllactosamine, is dramatically upregulated in colon cancer.''; PubMed Europe PMC Scholia
84. Yoshida K, Oshima A, Shimmoto M, Fukuhara Y, Sakuraba H, Yanagisawa N, Suzuki Y.; ''Human beta-galactosidase gene mutations in GM1-gangliosidosis: a common mutation among Japanese adult/chronic cases.''; PubMed Europe PMC Scholia
85. Cantz M, Gehler J.; ''The mucopolysaccharidoses: inborn errors of glycosaminoglycan catabolism.''; PubMed Europe PMC Scholia
86. Marcos I, Galán JJ, Borrego S, Antiñolo G.; ''Cloning, characterization, and chromosome mapping of the human GlcAT-S gene.''; PubMed Europe PMC Scholia
87. Asher R, Bignami A.; ''Hyaluronate binding and CD44 expression in human glioblastoma cells and astrocytes.''; PubMed Europe PMC Scholia
88. Shental-Bechor D, Levy Y.; ''Folding of glycoproteins: toward understanding the biophysics of the glycosylation code.''; PubMed Europe PMC Scholia
89. Stern R.; ''Devising a pathway for hyaluronan catabolism: are we there yet?''; PubMed Europe PMC Scholia
90. 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
91. Regeer RR, Lee A, Markovich D.; ''Characterization of the human sulfate anion transporter (hsat-1) protein and gene (SAT1; SLC26A1).''; PubMed Europe PMC Scholia
92. Suda T, Kamiyama S, Suzuki M, Kikuchi N, Nakayama K, Narimatsu H, Jigami Y, Aoki T, Nishihara S.; ''Molecular cloning and characterization of a human multisubstrate specific nucleotide-sugar transporter homologous to Drosophila fringe connection.''; PubMed Europe PMC Scholia
93. Goossens D, Van Gestel S, Claes S, De Rijk P, Souery D, Massat I, Van den Bossche D, Backhovens H, Mendlewicz J, Van Broeckhoven C, Del-Favero J.; ''A novel CpG-associated brain-expressed candidate gene for chromosome 18q-linked bipolar disorder.''; PubMed Europe PMC Scholia
94. Zhou D, Dinter A, Gutiérrez Gallego R, Kamerling JP, Vliegenthart JF, Berger EG, Hennet T.; ''A beta-1,3-N-acetylglucosaminyltransferase with poly-N-acetyllactosamine synthase activity is structurally related to beta-1,3-galactosyltransferases.''; PubMed Europe PMC Scholia
95. Knudson W, Chow G, Knudson CB.; ''CD44-mediated uptake and degradation of hyaluronan.''; PubMed Europe PMC Scholia
96. Ohtake S, Kimata K, Habuchi O.; ''A unique nonreducing terminal modification of chondroitin sulfate by N-acetylgalactosamine 4-sulfate 6-o-sulfotransferase.''; PubMed Europe PMC Scholia
97. Levy-Adam F, Miao HQ, Heinrikson RL, Vlodavsky I, Ilan N.; ''Heterodimer formation is essential for heparanase enzymatic activity.''; PubMed Europe PMC Scholia
98. Tsutsumi K, Shimakawa H, Kitagawa H, Sugahara K.; ''Functional expression and genomic structure of human chondroitin 6-sulfotransferase.''; PubMed Europe PMC Scholia
99. Wang C, Entwistle J, Hou G, Li Q, Turley EA.; ''The characterization of a human RHAMM cDNA: conservation of the hyaluronan-binding domains.''; PubMed Europe PMC Scholia
100. Ouzzine M, Gulberti S, Netter P, Magdalou J, Fournel-Gigleux S.; ''Structure/function of the human Ga1beta1,3-glucuronosyltransferase. Dimerization and functional activity are mediated by two crucial cysteine residues.''; PubMed Europe PMC Scholia
101. Kang HG, Evers MR, Xia G, Baenziger JU, Schachner M.; ''Molecular cloning and characterization of chondroitin-4-O-sulfotransferase-3. A novel member of the HNK-1 family of sulfotransferases.''; PubMed Europe PMC Scholia
102. Oshima A, Kyle JW, Miller RD, Hoffmann JW, Powell PP, Grubb JH, Sly WS, Tropak M, Guise KS, Gravel RA.; ''Cloning, sequencing, and expression of cDNA for human beta-glucuronidase.''; PubMed Europe PMC Scholia
103. Wuyts W, Van Hul W, De Boulle K, Hendrickx J, Bakker E, Vanhoenacker F, Mollica F, Lüdecke HJ, Sayli BS, Pazzaglia UE, Mortier G, Hamel B, Conrad EU, Matsushita M, Raskind WH, Willems PJ.; ''Mutations in the EXT1 and EXT2 genes in hereditary multiple exostoses.''; PubMed Europe PMC Scholia
104. Stern R.; ''Hyaluronan catabolism: a new metabolic pathway.''; PubMed Europe PMC Scholia
105. Takagaki K, Nakamura T, Endo M.; ''Demonstration of an endo-beta-galactosidase and an endo-beta-xylosidase that degrade the proteoglycan linkage region.''; PubMed Europe PMC Scholia
106. 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
107. Lo NW, Shaper JH, Pevsner J, Shaper NL.; ''The expanding beta 4-galactosyltransferase gene family: messages from the databanks.''; PubMed Europe PMC Scholia
108. Okuda T, Mita S, Yamauchi S, Matsubara T, Yagi F, Yamamori D, Fukuta M, Kuroiwa A, Matsuda Y, Habuchi O.; ''Molecular cloning, expression, and chromosomal mapping of human chondroitin 4-sulfotransferase, whose expression pattern in human tissues is different from that of chondroitin 6-sulfotransferase.''; PubMed Europe PMC Scholia
109. Hiraoka N, Nakagawa H, Ong E, Akama TO, Fukuda MN, Fukuda M.; ''Molecular cloning and expression of two distinct human chondroitin 4-O-sulfotransferases that belong to the HNK-1 sulfotransferase gene family.''; PubMed Europe PMC Scholia
110. Frost GI, Csóka AB, Wong T, Stern R.; ''Purification, cloning, and expression of human plasma hyaluronidase.''; PubMed Europe PMC Scholia
111. Shang J, Qiu R, Wang J, Liu J, Zhou R, Ding H, Yang S, Zhang S, Jin C.; ''Molecular cloning and expression of Galbeta1,3GalNAc alpha2, 3-sialyltransferase from human fetal liver.''; PubMed Europe PMC Scholia
112. Kamiyama S, Sasaki N, Goda E, Ui-Tei K, Saigo K, Narimatsu H, Jigami Y, Kannagi R, Irimura T, Nishihara S.; ''Molecular cloning and characterization of a novel 3'-phosphoadenosine 5'-phosphosulfate transporter, PAPST2.''; PubMed Europe PMC Scholia
113. Akama TO, Misra AK, Hindsgaul O, Fukuda MN.; ''Enzymatic synthesis in vitro of the disulfated disaccharide unit of corneal keratan sulfate.''; PubMed Europe PMC Scholia
114. Yoshida H, Nagaoka A, Kusaka-Kikushima A, Tobiishi M, Kawabata K, Sayo T, Sakai S, Sugiyama Y, Enomoto H, Okada Y, Inoue S.; ''KIAA1199, a deafness gene of unknown function, is a new hyaluronan binding protein involved in hyaluronan depolymerization.''; PubMed Europe PMC Scholia
115. Faiyaz ul Haque M, King LM, Krakow D, Cantor RM, Rusiniak ME, Swank RT, Superti-Furga A, Haque S, Abbas H, Ahmad W, Ahmad M, Cohn DH.; ''Mutations in orthologous genes in human spondyloepimetaphyseal dysplasia and the brachymorphic mouse.''; PubMed Europe PMC Scholia
116. Tian J, Ling L, Shboul M, Lee H, O'Connor B, Merriman B, Nelson SF, Cool S, Ababneh OH, Al-Hadidy A, Masri A, Hamamy H, Reversade B.; ''Loss of CHSY1, a secreted FRINGE enzyme, causes syndromic brachydactyly in humans via increased NOTCH signaling.''; PubMed Europe PMC Scholia
117. Sakaguchi H, Kitagawa H, Sugahara K.; ''Functional expression and genomic structure of human N-acetylglucosamine-6-O-sulfotransferase that transfers sulfate to beta-N-acetylglucosamine at the nonreducing end of an N-acetyllactosamine sequence.''; PubMed Europe PMC Scholia
118. Clarke LA.; ''The mucopolysaccharidoses: a success of molecular medicine.''; PubMed Europe PMC Scholia
119. Daly SB, Urquhart JE, Hilton E, McKenzie EA, Kammerer RA, Lewis M, Kerr B, Stuart H, Donnai D, Long DA, Burgu B, Aydogdu O, Derbent M, Garcia-Minaur S, Reardon W, Gener B, Shalev S, Smith R, Woolf AS, Black GC, Newman WG.; ''Mutations in HPSE2 cause urofacial syndrome.''; PubMed Europe PMC Scholia
120. Smeds E, Feta A, Kusche-Gullberg M.; ''Target selection of heparan sulfate hexuronic acid 2-O-sulfotransferase.''; PubMed Europe PMC Scholia
121. Habuchi H, Tanaka M, Habuchi O, Yoshida K, Suzuki H, Ban K, Kimata K.; ''The occurrence of three isoforms of heparan sulfate 6-O-sulfotransferase having different specificities for hexuronic acid adjacent to the targeted N-sulfoglucosamine.''; PubMed Europe PMC Scholia
122. Ozeran JD, Westley J, Schwartz NB.; ''Identification and partial purification of PAPS translocase.''; PubMed Europe PMC Scholia
123. Lee-Chen GJ, Lin SP, Tang YF, Chin YW.; ''Mucopolysaccharidosis type I: characterization of novel mutations affecting alpha-L-iduronidase activity.''; PubMed Europe PMC Scholia
124. Pacheco B, Malmström A, Maccarana M.; ''Two dermatan sulfate epimerases form iduronic acid domains in dermatan sulfate.''; PubMed Europe PMC Scholia
125. Wilson PJ, Morris CP, Anson DS, Occhiodoro T, Bielicki J, Clements PR, Hopwood JJ.; ''Hunter syndrome: isolation of an iduronate-2-sulfatase cDNA clone and analysis of patient DNA.''; PubMed Europe PMC Scholia
126. Li Y, Laue K, Temtamy S, Aglan M, Kotan LD, Yigit G, Canan H, Pawlik B, Nürnberg G, Wakeling EL, Quarrell OW, Baessmann I, Lanktree MB, Yilmaz M, Hegele RA, Amr K, May KW, Nürnberg P, Topaloglu AK, Hammerschmidt M, Wollnik B.; ''Temtamy preaxial brachydactyly syndrome is caused by loss-of-function mutations in chondroitin synthase 1, a potential target of BMP signaling.''; PubMed Europe PMC Scholia
127. Zheng H, Li Y, Ji C, Li J, Zhang J, Yin G, Xu J, Ye X, Wu M, Zou X, Gu S, Xie Y, Mao Y.; ''Characterization of a cDNA encoding a protein with limited similarity to beta1, 3-N-acetylglucosaminyltransferase.''; PubMed Europe PMC Scholia
128. Fukuda S, Tomatsu S, Masue M, Sukegawa K, Iwata H, Ogawa T, Nakashima Y, Hori T, Yamagishi A, Hanyu Y.; ''Mucopolysaccharidosis type IVA. N-acetylgalactosamine-6-sulfate sulfatase exonic point mutations in classical Morquio and mild cases.''; PubMed Europe PMC Scholia
129. Silbert JE, Sugumaran G.; ''Biosynthesis of chondroitin/dermatan sulfate.''; PubMed Europe PMC Scholia
130. Dixon J, Loftus SK, Gladwin AJ, Scambler PJ, Wasmuth JJ, Dixon MJ.; ''Cloning of the human heparan sulfate-N-deacetylase/N-sulfotransferase gene from the Treacher Collins syndrome candidate region at 5q32-q33.1.''; PubMed Europe PMC Scholia
131. Bai X, Zhou D, Brown JR, Crawford BE, Hennet T, Esko JD.; ''Biosynthesis of the linkage region of glycosaminoglycans: cloning and activity of galactosyltransferase II, the sixth member of the beta 1,3-galactosyltransferase family (beta 3GalT6).''; PubMed Europe PMC Scholia
132. Toyoshima M, Nakajima M.; ''Human heparanase. Purification, characterization, cloning, and expression.''; PubMed Europe PMC Scholia
133. Mishima M, Wakabayashi S, Kojima C.; ''Solution structure of the cytoplasmic region of Na+/H+ exchanger 1 complexed with essential cofactor calcineurin B homologous protein 1.''; PubMed Europe PMC Scholia
134. Okajima T, Fukumoto S, Miyazaki H, Ishida H, Kiso M, Furukawa K, Urano T, Furukawa K.; ''Molecular cloning of a novel alpha2,3-sialyltransferase (ST3Gal VI) that sialylates type II lactosamine structures on glycoproteins and glycolipids.''; PubMed Europe PMC Scholia
135. Maccarana M, Olander B, Malmström J, Tiedemann K, Aebersold R, Lindahl U, Li JP, Malmström A.; ''Biosynthesis of dermatan sulfate: chondroitin-glucuronate C5-epimerase is identical to SART2.''; PubMed Europe PMC Scholia
136. 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
137. Jedrzejas MJ, Stern R.; ''Structures of vertebrate hyaluronidases and their unique enzymatic mechanism of hydrolysis.''; PubMed Europe PMC Scholia
138. Yada T, Sato T, Kaseyama H, Gotoh M, Iwasaki H, Kikuchi N, Kwon YD, Togayachi A, Kudo T, Watanabe H, Narimatsu H, Kimata K.; ''Chondroitin sulfate synthase-3. Molecular cloning and characterization.''; PubMed Europe PMC Scholia
139. Evers MR, Xia G, Kang HG, Schachner M, Baenziger JU.; ''Molecular cloning and characterization of a dermatan-specific N-acetylgalactosamine 4-O-sulfotransferase.''; PubMed Europe PMC Scholia
140. Harris EN, Weigel JA, Weigel PH.; ''The human hyaluronan receptor for endocytosis (HARE/Stabilin-2) is a systemic clearance receptor for heparin.''; PubMed Europe PMC Scholia
141. Durand S, Feldhammer M, Bonneil E, Thibault P, Pshezhetsky AV.; ''Analysis of the biogenesis of heparan sulfate acetyl-CoA:alpha-glucosaminide N-acetyltransferase provides insights into the mechanism underlying its complete deficiency in mucopolysaccharidosis IIIC.''; PubMed Europe PMC Scholia
142. Gorham SD, Cantz M.; ''Arylsulphatase B, an exo-sulphatase for chondroitin 4-sulphate tetrasaccharide.''; PubMed Europe PMC Scholia
143. Winchester B.; ''Lysosomal metabolism of glycoproteins.''; PubMed Europe PMC Scholia
144. Xia G, Chen J, Tiwari V, Ju W, Li JP, Malmstrom A, Shukla D, Liu J.; ''Heparan sulfate 3-O-sulfotransferase isoform 5 generates both an antithrombin-binding site and an entry receptor for herpes simplex virus, type 1.''; PubMed Europe PMC Scholia
145. Weber B, Blanch L, Clements PR, Scott HS, Hopwood JJ.; ''Cloning and expression of the gene involved in Sanfilippo B syndrome (mucopolysaccharidosis III B).''; PubMed Europe PMC Scholia
146. Gotoh M, Yada T, Sato T, Akashima T, Iwasaki H, Mochizuki H, Inaba N, Togayachi A, Kudo T, Watanabe H, Kimata K, Narimatsu H.; ''Molecular cloning and characterization of a novel chondroitin sulfate glucuronyltransferase that transfers glucuronic acid to N-acetylgalactosamine.''; PubMed Europe PMC Scholia
147. Lee JK, Bhakta S, Rosen SD, Hemmerich S.; ''Cloning and characterization of a mammalian N-acetylglucosamine-6-sulfotransferase that is highly restricted to intestinal tissue.''; PubMed Europe PMC Scholia
148. Masue M, Sukegawa K, Orii T, Hashimoto T.; ''N-acetylgalactosamine-6-sulfate sulfatase in human placenta: purification and characteristics.''; PubMed Europe PMC Scholia
149. Robertson DA, Freeman C, Morris CP, Hopwood JJ.; ''A cDNA clone for human glucosamine-6-sulphatase reveals differences between arylsulphatases and non-arylsulphatases.''; PubMed Europe PMC Scholia
150. Lederkremer GZ.; ''Glycoprotein folding, quality control and ER-associated degradation.''; PubMed Europe PMC Scholia
151. Hrebícek M, Mrázová L, Seyrantepe V, Durand S, Roslin NM, Nosková L, Hartmannová H, Ivánek R, Cízkova A, Poupetová H, Sikora J, Urinovská J, Stranecký V, Zeman J, Lepage P, Roquis D, Verner A, Ausseil J, Beesley CE, Maire I, Poorthuis BJ, van de Kamp J, van Diggelen OP, Wevers RA, Hudson TJ, Fujiwara TM, Majewski J, Morgan K, Kmoch S, Pshezhetsky AV.; ''Mutations in TMEM76* cause mucopolysaccharidosis IIIC (Sanfilippo C syndrome).''; PubMed Europe PMC Scholia
152. Lim CT, Horwitz AL.; ''Purification and properties of human N-acetylgalactosamine-6-sulfate sulfatase.''; PubMed Europe PMC Scholia
153. Götting C, Kuhn J, Zahn R, Brinkmann T, Kleesiek K.; ''Molecular cloning and expression of human UDP-d-Xylose:proteoglycan core protein beta-d-xylosyltransferase and its first isoform XT-II.''; PubMed Europe PMC Scholia
154. Lepperdinger G, Strobl B, Kreil G.; ''HYAL2, a human gene expressed in many cells, encodes a lysosomal hyaluronidase with a novel type of specificity.''; PubMed Europe PMC Scholia

History

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Compare	Revision	Action	Time	User	Comment
	114622	view	16:08, 25 January 2021	ReactomeTeam	Reactome version 75
	113070	view	11:13, 2 November 2020	ReactomeTeam	Reactome version 74
	112305	view	15:22, 9 October 2020	ReactomeTeam	Reactome version 73
	101203	view	11:10, 1 November 2018	ReactomeTeam	reactome version 66
	100741	view	20:34, 31 October 2018	ReactomeTeam	reactome version 65
	100285	view	19:11, 31 October 2018	ReactomeTeam	reactome version 64
	99831	view	15:55, 31 October 2018	ReactomeTeam	reactome version 63
	99388	view	14:33, 31 October 2018	ReactomeTeam	reactome version 62 (2nd attempt)
	93758	view	13:34, 16 August 2017	ReactomeTeam	reactome version 61
	93280	view	11:19, 9 August 2017	ReactomeTeam	reactome version 61
	87154	view	18:58, 18 July 2016	Mkutmon	Ontology Term : 'glycosaminoglycan metabolic pathway' added !
	86359	view	09:16, 11 July 2016	ReactomeTeam	reactome version 56
	83349	view	10:56, 18 November 2015	ReactomeTeam	Version54
	81509	view	13:02, 21 August 2015	ReactomeTeam	Version53
	76981	view	08:27, 17 July 2014	ReactomeTeam	Fixed remaining interactions
	76686	view	12:05, 16 July 2014	ReactomeTeam	Fixed remaining interactions
	76013	view	10:07, 11 June 2014	ReactomeTeam	Re-fixing comment source
	75721	view	11:08, 10 June 2014	ReactomeTeam	Reactome 48 Update
	74718	view	08:47, 30 April 2014	ReactomeTeam	New pathway

External references

DataNodes

View all...

Name	Type	Database reference	Comment
(HA)2		R-ALL-2160866 (Reactome)
(HA)50		R-ALL-2160864 (Reactome)
(HA)50		R-ALL-2160894 (Reactome)
ABCC5	Protein	O15440 (Uniprot-TrEMBL)
ACAN	Protein	P16112 (Uniprot-TrEMBL)
ADP	Metabolite	CHEBI:16761 (ChEBI)
AGRN(30-2045)	Protein	O00468 (Uniprot-TrEMBL)
APS	Metabolite	CHEBI:17709 (ChEBI)
ARSB:Ca2+	Complex	R-HSA-1606792 (Reactome)
ATP	Metabolite	CHEBI:15422 (ChEBI)
Ac-CoA	Metabolite	CHEBI:15351 (ChEBI)
Asparagine N-linked glycosylation	Pathway	R-HSA-446203 (Reactome)	N-linked glycosylation is the most important form of post-translational modification for proteins synthesized and folded in the Endoplasmic Reticulum (Stanley 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 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 & Levy 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 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 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 et al. 2003), and classified as CDG type I for the genes in the LLO synthesis pathway, and CDG type II for the others.
B3GALT6	Protein	Q96L58 (Uniprot-TrEMBL)
B3GAT dimers	Complex	R-HSA-1889954 (Reactome)
B3GAT1	Protein	Q9P2W7 (Uniprot-TrEMBL)
B3GAT2	Protein	Q9NPZ5 (Uniprot-TrEMBL)
B3GAT3	Protein	O94766 (Uniprot-TrEMBL)
B3GNT1	Protein	O43505 (Uniprot-TrEMBL)
B3GNT1,2,3,4,7	Complex	R-HSA-2046221 (Reactome)
B3GNT2	Protein	Q9NY97 (Uniprot-TrEMBL)
B3GNT3	Protein	Q9Y2A9 (Uniprot-TrEMBL)
B3GNT4	Protein	Q9C0J1 (Uniprot-TrEMBL)
B3GNT7	Protein	Q8NFL0 (Uniprot-TrEMBL)
B4GALT1	Protein	P15291 (Uniprot-TrEMBL)
B4GALT1-6 homodimers	Complex	R-HSA-975898 (Reactome)
B4GALT2	Protein	O60909 (Uniprot-TrEMBL)
B4GALT3	Protein	O60512 (Uniprot-TrEMBL)
B4GALT4	Protein	O60513 (Uniprot-TrEMBL)
B4GALT5	Protein	O43286 (Uniprot-TrEMBL)
B4GALT6	Protein	Q9UBX8 (Uniprot-TrEMBL)
B4GALT7	Protein	Q9UBV7 (Uniprot-TrEMBL)
BCAN	Protein	Q96GW7 (Uniprot-TrEMBL)
BGAL	Complex	R-HSA-3229251 (Reactome)	This CandidateSet contains sequences identified by William Pearson's analysis of Reactome catalyst entities. Catalyst entity sequences were used to identify analagous sequences that shared overall homology and active site homology. Sequences in this Candidate set were identified in an April 24, 2012 analysis.
BGN	Protein	P21810 (Uniprot-TrEMBL)
C4S chain	Metabolite	CHEBI:63513 (ChEBI)
C4S-BCAN	Protein	Q96GW7 (Uniprot-TrEMBL)
C4S-BGN	Protein	P21810 (Uniprot-TrEMBL)
C4S-CSPG4	Protein	Q6UVK1 (Uniprot-TrEMBL)
C4S-CSPG5	Protein	O95196 (Uniprot-TrEMBL)
C4S-DCN	Protein	P07585 (Uniprot-TrEMBL)
C4S-NCAN	Protein	O14594 (Uniprot-TrEMBL)
C4S-PG		R-HSA-2064226 (Reactome)
C4S-PG		R-HSA-2065134 (Reactome)
C4S-PG	Complex	R-HSA-2064226 (Reactome)
C4S-PGs		R-HSA-2063982 (Reactome)
C4S-VCAN	Protein	P13611 (Uniprot-TrEMBL)
C4S/C6S chains	Complex	R-HSA-R-ALL-2065251 (Reactome)
C6S chain	Metabolite	CHEBI:63512 (ChEBI)
C6S-BCAN	Protein	Q96GW7 (Uniprot-TrEMBL)
C6S-BGN	Protein	P21810 (Uniprot-TrEMBL)
C6S-CSPG4	Protein	Q6UVK1 (Uniprot-TrEMBL)
C6S-CSPG5	Protein	O95196 (Uniprot-TrEMBL)
C6S-DCN	Protein	P07585 (Uniprot-TrEMBL)
C6S-NCAN	Protein	O14594 (Uniprot-TrEMBL)
C6S-PG		R-HSA-2064219 (Reactome)
C6S-PG		R-HSA-2065083 (Reactome)
C6S-PG	Complex	R-HSA-2064219 (Reactome)
C6S-PGs		R-HSA-2064078 (Reactome)
C6S-VCAN	Protein	P13611 (Uniprot-TrEMBL)
CD44	Protein	P16070 (Uniprot-TrEMBL)
CEMIP	Protein	Q8WUJ3 (Uniprot-TrEMBL)
CH3COO-	Metabolite	CHEBI:15366 (ChEBI)
CHEBI:63868 chain	Metabolite	CHEBI:63868 (ChEBI)
CHPF	Protein	Q8IZ52 (Uniprot-TrEMBL)
CHPF,CHPF2,CHSY1,CHSY3	Complex	R-HSA-1971467 (Reactome)
CHPF,CHSY1,CHSY3	Complex	R-HSA-1971425 (Reactome)
CHPF2	Protein	Q9P2E5 (Uniprot-TrEMBL)
CHST11	Protein	Q9NPF2 (Uniprot-TrEMBL)
CHST12	Protein	Q9NRB3 (Uniprot-TrEMBL)
CHST13	Protein	Q8NET6 (Uniprot-TrEMBL)
CHST14 products	Complex	R-HSA-5607524 (Reactome)
CHST14 substrates	Complex	R-HSA-5607518 (Reactome)
CHST14	Protein	Q8NCH0 (Uniprot-TrEMBL)
CHST15	Protein	Q7LFX5 (Uniprot-TrEMBL)
CHST1	Protein	O43916 (Uniprot-TrEMBL)
CHST2	Protein	Q9Y4C5 (Uniprot-TrEMBL)
CHST2,5,6	Complex	R-HSA-2046158 (Reactome)
CHST3	Protein	Q7LGC8 (Uniprot-TrEMBL)
CHST3,7	Complex	R-HSA-2018661 (Reactome)
CHST5	Protein	Q9GZS9 (Uniprot-TrEMBL)
CHST6	Protein	Q9GZX3 (Uniprot-TrEMBL)
CHST7	Protein	Q9NS84 (Uniprot-TrEMBL)
CHST9,11,12,13	Complex	R-HSA-1971481 (Reactome)
CHST9-2	Protein	Q7L1S5-2 (Uniprot-TrEMBL)
CHSY1	Protein	Q86X52 (Uniprot-TrEMBL)
CHSY3	Protein	Q70JA7 (Uniprot-TrEMBL)
CMP-Neu5Ac	Metabolite	CHEBI:16556 (ChEBI)
CMP	Metabolite	CHEBI:17361 (ChEBI)
CS/DS core proteins	Complex	R-HSA-2065248 (Reactome)
CS/HS precursor	Metabolite	CHEBI:63505 (ChEBI)
CSE-BCAN	Protein	Q96GW7 (Uniprot-TrEMBL)
CSE-BGN	Protein	P21810 (Uniprot-TrEMBL)
CSE-CSPG4	Protein	Q6UVK1 (Uniprot-TrEMBL)
CSE-CSPG5	Protein	O95196 (Uniprot-TrEMBL)
CSE-DCN	Protein	P07585 (Uniprot-TrEMBL)
CSE-NCAN	Protein	O14594 (Uniprot-TrEMBL)
CSE-PG		R-HSA-2064101 (Reactome)
CSE-PG		R-HSA-2065112 (Reactome)
CSE-PG	Complex	R-HSA-2064101 (Reactome)
CSE-PGs		R-HSA-2064081 (Reactome)
CSE-VCAN	Protein	P13611 (Uniprot-TrEMBL)
CSGALNACT1	Protein	Q8TDX6 (Uniprot-TrEMBL)
CSGALNACT2	Protein	Q8N6G5 (Uniprot-TrEMBL)
CSGALNACT	Complex	R-HSA-1971496 (Reactome)
CSPG4	Protein	Q6UVK1 (Uniprot-TrEMBL)
CSPG5	Protein	O95196 (Uniprot-TrEMBL)
CSPGs	Complex	R-HSA-2024088 (Reactome)
CSPGs	Complex	R-HSA-2064124 (Reactome)
CSPGs	Complex	R-HSA-2065110 (Reactome)
Ca2+	Metabolite	CHEBI:29108 (ChEBI)
ChEBI:63515 chain	Metabolite	CHEBI:63515 (ChEBI)
ChEBI:63515 chain	Metabolite	CHEBI:63515 (ChEBI)
ChEBI:63516 chain	Metabolite	CHEBI:63516 (ChEBI)
ChEBI:63517 chain	Metabolite	CHEBI:63517 (ChEBI)
ChEBI:63519 chain	Metabolite	CHEBI:63519 (ChEBI)
Chondroitin chain	Metabolite	CHEBI:63511 (ChEBI)
Chondroitin chain	Metabolite	CHEBI:63511 (ChEBI)
Chondroitin chains	Complex	R-HSA-R-ALL-2065257 (Reactome)
CoA-SH	Metabolite	CHEBI:15346 (ChEBI)
D-xylose	Metabolite	CHEBI:15936 (ChEBI)
D2,4(S)2-BCAN	Protein	Q96GW7 (Uniprot-TrEMBL)
D2,4(S)2-BGN	Protein	P21810 (Uniprot-TrEMBL)
D2,4(S)2-CSPG4	Protein	Q6UVK1 (Uniprot-TrEMBL)
D2,4(S)2-CSPG5	Protein	O95196 (Uniprot-TrEMBL)
D2,4(S)2-DCN	Protein	P07585 (Uniprot-TrEMBL)
D2,4(S)2-NCAN	Protein	O14594 (Uniprot-TrEMBL)
D2,4(S)2-PG		R-HSA-2065145 (Reactome)
D2,4(S)2-PG	Complex	R-HSA-2065145 (Reactome)
D2,4(S)2-PGs		R-HSA-2065202 (Reactome)
D2,4(S)2-PGs		R-HSA-2065226 (Reactome)
D2,4(S)2-VCAN	Protein	P13611 (Uniprot-TrEMBL)
D2,4,4(S)3-BCAN	Protein	Q96GW7 (Uniprot-TrEMBL)
D2,4,4(S)3-BGN	Protein	P21810 (Uniprot-TrEMBL)
D2,4,4(S)3-CSPG4	Protein	Q6UVK1 (Uniprot-TrEMBL)
D2,4,4(S)3-CSPG5	Protein	O95196 (Uniprot-TrEMBL)
D2,4,4(S)3-DCN	Protein	P07585 (Uniprot-TrEMBL)
D2,4,4(S)3-NCAN	Protein	O14594 (Uniprot-TrEMBL)
D2,4,4(S)3-PGs		R-HSA-2065087 (Reactome)
D2,4,4(S)3-PGs		R-HSA-2065132 (Reactome)
D2,4,4(S)3-PGs		R-HSA-2065138 (Reactome)
D2,4,4(S)3-PGs	Complex	R-HSA-2065138 (Reactome)
D2,4,4(S)3-VCAN	Protein	P13611 (Uniprot-TrEMBL)
D4S-BCAN	Protein	Q96GW7 (Uniprot-TrEMBL)
D4S-BGN	Protein	P21810 (Uniprot-TrEMBL)
D4S-CSPG4	Protein	Q6UVK1 (Uniprot-TrEMBL)
D4S-CSPG5	Protein	O95196 (Uniprot-TrEMBL)
D4S-DCN	Protein	P07585 (Uniprot-TrEMBL)
D4S-NCAN	Protein	O14594 (Uniprot-TrEMBL)
D4S-PGs		R-HSA-2065135 (Reactome)
D4S-PGs		R-HSA-2065207 (Reactome)
D4S-PGs		R-HSA-2065235 (Reactome)
D4S-PGs	Complex	R-HSA-2065135 (Reactome)
D4S-VCAN	Protein	P13611 (Uniprot-TrEMBL)
DCN	Protein	P07585 (Uniprot-TrEMBL)
DSE	Protein	Q9UL01 (Uniprot-TrEMBL)
DSE,DSEL products	Complex	R-HSA-5607545 (Reactome)
DSE,DSEL substrates	Complex	R-HSA-5607547 (Reactome)
DSE,DSEL	Complex	R-HSA-3734047 (Reactome)
DSEL	Protein	Q8IZU8 (Uniprot-TrEMBL)
DSPGs	Complex	R-HSA-2065214 (Reactome)
DSPGs	Complex	R-HSA-2065240 (Reactome)
DSPGs	Complex	R-HSA-2065267 (Reactome)
EXT1	Protein	Q16394 (Uniprot-TrEMBL)
EXT1:EXT2	Complex	R-HSA-2022878 (Reactome)
EXT2	Protein	Q93063 (Uniprot-TrEMBL)
FMOD	Protein	Q06828 (Uniprot-TrEMBL)
GAG core proteins	Complex	R-HSA-2054107 (Reactome)
GALNS oligomer		R-HSA-1630327 (Reactome)
GLB1	Protein	P16278 (Uniprot-TrEMBL)
GLB1L	Protein	Q6UWU2 (Uniprot-TrEMBL)
GLCE	Protein	O94923 (Uniprot-TrEMBL)
GPC1	Protein	P35052 (Uniprot-TrEMBL)
GPC2	Protein	Q8N158 (Uniprot-TrEMBL)
GPC3(25-?)	Protein	P51654 (Uniprot-TrEMBL)
GPC4	Protein	O75487 (Uniprot-TrEMBL)
GPC5(25-?)	Protein	P78333 (Uniprot-TrEMBL)
GPC6	Protein	Q9Y625 (Uniprot-TrEMBL)
GUSB	Protein	P08236 (Uniprot-TrEMBL)
GUSB tetramer	Complex	R-HSA-1678867 (Reactome)
GXYLT1	Protein	Q4G148 (Uniprot-TrEMBL)
GXYLT2	Protein	A0PJZ3 (Uniprot-TrEMBL)
GXYLTs	Complex	R-HSA-1877994 (Reactome)
Gal(S)-GlcNAc(S)-Gal-GlcNAc(S)-Gal	Metabolite	CHEBI:63846 (ChEBI)
Gal-Gal-Xyl-AGRN	Protein	O00468 (Uniprot-TrEMBL)
Gal-Gal-Xyl-BCAN	Protein	Q96GW7 (Uniprot-TrEMBL)
Gal-Gal-Xyl-BGN	Protein	P21810 (Uniprot-TrEMBL)
Gal-Gal-Xyl-CSPG4	Protein	Q6UVK1 (Uniprot-TrEMBL)
Gal-Gal-Xyl-CSPG5	Protein	O95196 (Uniprot-TrEMBL)
Gal-Gal-Xyl-DCN	Protein	P07585 (Uniprot-TrEMBL)
Gal-Gal-Xyl-GPC1	Protein	P35052 (Uniprot-TrEMBL)
Gal-Gal-Xyl-GPC2	Protein	Q8N158 (Uniprot-TrEMBL)
Gal-Gal-Xyl-GPC3	Protein	P51654 (Uniprot-TrEMBL)
Gal-Gal-Xyl-GPC4	Protein	O75487 (Uniprot-TrEMBL)
Gal-Gal-Xyl-GPC5	Protein	P78333 (Uniprot-TrEMBL)
Gal-Gal-Xyl-GPC6	Protein	Q9Y625 (Uniprot-TrEMBL)
Gal-Gal-Xyl-HSPG2	Protein	P98160 (Uniprot-TrEMBL)
Gal-Gal-Xyl-NCAN	Protein	O14594 (Uniprot-TrEMBL)
Gal-Gal-Xyl-SDC1	Protein	P18827 (Uniprot-TrEMBL)
Gal-Gal-Xyl-SDC2	Protein	P34741 (Uniprot-TrEMBL)
Gal-Gal-Xyl-SDC3	Protein	O75056 (Uniprot-TrEMBL)
Gal-Gal-Xyl-SDC4	Protein	P31431 (Uniprot-TrEMBL)
Gal-Gal-Xyl-VCAN	Protein	P13611 (Uniprot-TrEMBL)
Gal-Gal-Xyl-proteins	Complex	R-HSA-2064214 (Reactome)
Gal-GlcNAc(S)-Gal-GlcNAc(S)-Gal	Metabolite	CHEBI:63850 (ChEBI)
Gal-GlcNAc(S)-Gal	Metabolite	CHEBI:63853 (ChEBI)
Gal-Xyl-AGRN	Protein	O00468 (Uniprot-TrEMBL)
Gal-Xyl-BCAN	Protein	Q96GW7 (Uniprot-TrEMBL)
Gal-Xyl-BGN	Protein	P21810 (Uniprot-TrEMBL)
Gal-Xyl-CSPG4	Protein	Q6UVK1 (Uniprot-TrEMBL)
Gal-Xyl-CSPG5	Protein	O95196 (Uniprot-TrEMBL)
Gal-Xyl-DCN	Protein	P07585 (Uniprot-TrEMBL)
Gal-Xyl-GPC1	Protein	P35052 (Uniprot-TrEMBL)
Gal-Xyl-GPC2	Protein	Q8N158 (Uniprot-TrEMBL)
Gal-Xyl-GPC3	Protein	P51654 (Uniprot-TrEMBL)
Gal-Xyl-GPC4	Protein	O75487 (Uniprot-TrEMBL)
Gal-Xyl-GPC5	Protein	P78333 (Uniprot-TrEMBL)
Gal-Xyl-GPC6	Protein	Q9Y625 (Uniprot-TrEMBL)
Gal-Xyl-HSPG2	Protein	P98160 (Uniprot-TrEMBL)
Gal-Xyl-NCAN	Protein	O14594 (Uniprot-TrEMBL)
Gal-Xyl-SDC1	Protein	P18827 (Uniprot-TrEMBL)
Gal-Xyl-SDC2	Protein	P34741 (Uniprot-TrEMBL)
Gal-Xyl-SDC3	Protein	O75056 (Uniprot-TrEMBL)
Gal-Xyl-SDC4	Protein	P31431 (Uniprot-TrEMBL)
Gal-Xyl-VCAN	Protein	P13611 (Uniprot-TrEMBL)
Gal-Xyl-proteins	Complex	R-HSA-2064058 (Reactome)
Gal-glycan ACAN	Protein	P16112 (Uniprot-TrEMBL)
Gal-glycan FMOD	Protein	Q06828 (Uniprot-TrEMBL)
Gal-glycan KERA	Protein	O60938 (Uniprot-TrEMBL)
Gal-glycan LUM	Protein	P51884 (Uniprot-TrEMBL)
Gal-glycan OGN	Protein	P20774 (Uniprot-TrEMBL)
Gal-glycan OMD	Protein	Q99983 (Uniprot-TrEMBL)
Gal-glycan PRELP	Protein	P51888 (Uniprot-TrEMBL)
Gal-glycan-protein	Complex	R-HSA-2046271 (Reactome)
Gal	Metabolite	CHEBI:28061 (ChEBI)
GalNAc	Metabolite	CHEBI:28037 (ChEBI)
GlcA-Gal-Gal-Xyl-AGRN	Protein	O00468 (Uniprot-TrEMBL)
GlcA-Gal-Gal-Xyl-BCAN	Protein	Q96GW7 (Uniprot-TrEMBL)
GlcA-Gal-Gal-Xyl-BGN	Protein	P21810 (Uniprot-TrEMBL)
GlcA-Gal-Gal-Xyl-CS proteins	Complex	R-HSA-2064233 (Reactome)
GlcA-Gal-Gal-Xyl-CSPG4	Protein	Q6UVK1 (Uniprot-TrEMBL)
GlcA-Gal-Gal-Xyl-CSPG5	Protein	O95196 (Uniprot-TrEMBL)
GlcA-Gal-Gal-Xyl-DCN	Protein	P07585 (Uniprot-TrEMBL)
GlcA-Gal-Gal-Xyl-GPC1	Protein	P35052 (Uniprot-TrEMBL)
GlcA-Gal-Gal-Xyl-GPC2	Protein	Q8N158 (Uniprot-TrEMBL)
GlcA-Gal-Gal-Xyl-GPC3	Protein	P51654 (Uniprot-TrEMBL)
GlcA-Gal-Gal-Xyl-GPC4	Protein	O75487 (Uniprot-TrEMBL)
GlcA-Gal-Gal-Xyl-GPC5	Protein	P78333 (Uniprot-TrEMBL)
GlcA-Gal-Gal-Xyl-GPC6	Protein	Q9Y625 (Uniprot-TrEMBL)
GlcA-Gal-Gal-Xyl-HS proteins	Complex	R-HSA-2076551 (Reactome)
GlcA-Gal-Gal-Xyl-HSPG2	Protein	P98160 (Uniprot-TrEMBL)
GlcA-Gal-Gal-Xyl-NCAN	Protein	O14594 (Uniprot-TrEMBL)
GlcA-Gal-Gal-Xyl-SDC1	Protein	P18827 (Uniprot-TrEMBL)
GlcA-Gal-Gal-Xyl-SDC2	Protein	P34741 (Uniprot-TrEMBL)
GlcA-Gal-Gal-Xyl-SDC3	Protein	O75056 (Uniprot-TrEMBL)
GlcA-Gal-Gal-Xyl-SDC4	Protein	P31431 (Uniprot-TrEMBL)
GlcA-Gal-Gal-Xyl-VCAN	Protein	P13611 (Uniprot-TrEMBL)
GlcA-Gal-Gal-Xyl-proteins	Complex	R-HSA-2064225 (Reactome)
GlcA-b1,3-GlcNAc	Metabolite	CHEBI:64024 (ChEBI)
GlcA	Metabolite	CHEBI:15748 (ChEBI)
GlcA	Metabolite	CHEBI:4178 (ChEBI)
GlcNAc(S)-Gal-GlcNAc(S)-Gal	Metabolite	CHEBI:63851 (ChEBI)
GlcNAc-Gal-GlcNAc(S)-Gal	Metabolite	CHEBI:63852 (ChEBI)
GlcNAc-GlcA-GlcNAc		R-NUL-2162223 (Reactome)
GlcNAc	Metabolite	CHEBI:17411 (ChEBI)
H+	Metabolite	CHEBI:15378 (ChEBI)
H2O	Metabolite	CHEBI:15377 (ChEBI)
HA		R-ALL-2160848 (Reactome)
HA polymer		R-ALL-2142896 (Reactome)
HA:HAR:HYAL2:SLC9A1:pCHP:Ca2+	Complex	R-HSA-2160891 (Reactome)
HA:HAR:HYAL2	Complex	R-HSA-2160889 (Reactome)
HA		R-ALL-2160848 (Reactome)
HARs	Complex	R-HSA-2160926 (Reactome)
HAS1	Protein	Q92839 (Uniprot-TrEMBL)
HAS1,2,3	Complex	R-HSA-2142875 (Reactome)
HAS2	Protein	Q92819 (Uniprot-TrEMBL)
HAS3	Protein	O00219 (Uniprot-TrEMBL)
HEXA	Protein	P06865 (Uniprot-TrEMBL)
HEXA,B	Complex	R-HSA-3662340 (Reactome)
HEXB(122-311)	Protein	P07686 (Uniprot-TrEMBL)
HEXB(315-556)	Protein	P07686 (Uniprot-TrEMBL)
HGSNAT oligomer		R-HSA-1678773 (Reactome)
HMMR	Protein	O75330 (Uniprot-TrEMBL)
HPSE dimer	Complex	R-HSA-1666976 (Reactome)
HPSE(158-543)	Protein	Q9Y251 (Uniprot-TrEMBL)
HPSE(36-109)	Protein	Q9Y251 (Uniprot-TrEMBL)
HPSE2(1-592)	Protein	Q8WWQ2 (Uniprot-TrEMBL)
HS core proteins	Complex	R-HSA-2090039 (Reactome)
HS core proteins	Complex	R-HSA-2090076 (Reactome)
HS(1)-AGRN	Protein	O00468 (Uniprot-TrEMBL)
HS(1)-GPC1	Protein	P35052 (Uniprot-TrEMBL)
HS(1)-GPC2	Protein	Q8N158 (Uniprot-TrEMBL)
HS(1)-GPC3	Protein	P51654 (Uniprot-TrEMBL)
HS(1)-GPC4	Protein	O75487 (Uniprot-TrEMBL)
HS(1)-GPC5	Protein	P78333 (Uniprot-TrEMBL)
HS(1)-GPC6	Protein	Q9Y625 (Uniprot-TrEMBL)
HS(1)-HSPG2	Protein	P98160 (Uniprot-TrEMBL)
HS(1)-PGs		R-HSA-2076452 (Reactome)
HS(1)-PGs		R-HSA-2076647 (Reactome)
HS(1)-PGs	Complex	R-HSA-2076452 (Reactome)
HS(1)-SDC1	Protein	P18827 (Uniprot-TrEMBL)
HS(1)-SDC2	Protein	P34741 (Uniprot-TrEMBL)
HS(1)-SDC3	Protein	O75056 (Uniprot-TrEMBL)
HS(1)-SDC4	Protein	P31431 (Uniprot-TrEMBL)
HS(2)-AGRN	Protein	O00468 (Uniprot-TrEMBL)
HS(2)-GPC1	Protein	P35052 (Uniprot-TrEMBL)
HS(2)-GPC2	Protein	Q8N158 (Uniprot-TrEMBL)
HS(2)-GPC3	Protein	P51654 (Uniprot-TrEMBL)
HS(2)-GPC4	Protein	O75487 (Uniprot-TrEMBL)
HS(2)-GPC5	Protein	P78333 (Uniprot-TrEMBL)
HS(2)-GPC6	Protein	Q9Y625 (Uniprot-TrEMBL)
HS(2)-HSPG2	Protein	P98160 (Uniprot-TrEMBL)
HS(2)-PGs		R-HSA-2076425 (Reactome)
HS(2)-PGs		R-HSA-2076620 (Reactome)
HS(2)-PGs	Complex	R-HSA-2076425 (Reactome)
HS(2)-SDC1	Protein	P18827 (Uniprot-TrEMBL)
HS(2)-SDC2	Protein	P34741 (Uniprot-TrEMBL)
HS(2)-SDC3	Protein	O75056 (Uniprot-TrEMBL)
HS(2)-SDC4	Protein	P31431 (Uniprot-TrEMBL)
HS(3)-AGRN	Protein	O00468 (Uniprot-TrEMBL)
HS(3)-GPC1	Protein	P35052 (Uniprot-TrEMBL)
HS(3)-GPC2	Protein	Q8N158 (Uniprot-TrEMBL)
HS(3)-GPC3	Protein	P51654 (Uniprot-TrEMBL)
HS(3)-GPC4	Protein	O75487 (Uniprot-TrEMBL)
HS(3)-GPC5	Protein	P78333 (Uniprot-TrEMBL)
HS(3)-GPC6	Protein	Q9Y625 (Uniprot-TrEMBL)
HS(3)-HSPG2	Protein	P98160 (Uniprot-TrEMBL)
HS(3)-PGs		R-HSA-2076297 (Reactome)
HS(3)-PGs		R-HSA-2076690 (Reactome)
HS(3)-PGs	Complex	R-HSA-2076297 (Reactome)
HS(3)-SDC1	Protein	P18827 (Uniprot-TrEMBL)
HS(3)-SDC2	Protein	P34741 (Uniprot-TrEMBL)
HS(3)-SDC3	Protein	O75056 (Uniprot-TrEMBL)
HS(3)-SDC4	Protein	P31431 (Uniprot-TrEMBL)
HS(4)-AGRN	Protein	O00468 (Uniprot-TrEMBL)
HS(4)-GPC1	Protein	P35052 (Uniprot-TrEMBL)
HS(4)-GPC2	Protein	Q8N158 (Uniprot-TrEMBL)
HS(4)-GPC3	Protein	P51654 (Uniprot-TrEMBL)
HS(4)-GPC4	Protein	O75487 (Uniprot-TrEMBL)
HS(4)-GPC5	Protein	P78333 (Uniprot-TrEMBL)
HS(4)-GPC6	Protein	Q9Y625 (Uniprot-TrEMBL)
HS(4)-HSPG2	Protein	P98160 (Uniprot-TrEMBL)
HS(4)-PGs		R-HSA-2076491 (Reactome)
HS(4)-PGs		R-HSA-2076655 (Reactome)
HS(4)-PGs	Complex	R-HSA-2076491 (Reactome)
HS(4)-SDC1	Protein	P18827 (Uniprot-TrEMBL)
HS(4)-SDC2	Protein	P34741 (Uniprot-TrEMBL)
HS(4)-SDC3	Protein	O75056 (Uniprot-TrEMBL)
HS(4)-SDC4	Protein	P31431 (Uniprot-TrEMBL)
HS(5)-AGRN	Protein	O00468 (Uniprot-TrEMBL)
HS(5)-GPC1	Protein	P35052 (Uniprot-TrEMBL)
HS(5)-GPC2	Protein	Q8N158 (Uniprot-TrEMBL)
HS(5)-GPC3	Protein	P51654 (Uniprot-TrEMBL)
HS(5)-GPC4	Protein	O75487 (Uniprot-TrEMBL)
HS(5)-GPC5	Protein	P78333 (Uniprot-TrEMBL)
HS(5)-GPC6	Protein	Q9Y625 (Uniprot-TrEMBL)
HS(5)-HSPG2	Protein	P98160 (Uniprot-TrEMBL)
HS(5)-PGs		R-HSA-2076461 (Reactome)
HS(5)-PGs		R-HSA-2076688 (Reactome)
HS(5)-PGs	Complex	R-HSA-2076461 (Reactome)
HS(5)-SDC1	Protein	P18827 (Uniprot-TrEMBL)
HS(5)-SDC2	Protein	P34741 (Uniprot-TrEMBL)
HS(5)-SDC3	Protein	O75056 (Uniprot-TrEMBL)
HS(5)-SDC4	Protein	P31431 (Uniprot-TrEMBL)
HS(6)-AGRN	Protein	O00468 (Uniprot-TrEMBL)
HS(6)-GPC1	Protein	P35052 (Uniprot-TrEMBL)
HS(6)-GPC2	Protein	Q8N158 (Uniprot-TrEMBL)
HS(6)-GPC3	Protein	P51654 (Uniprot-TrEMBL)
HS(6)-GPC4	Protein	O75487 (Uniprot-TrEMBL)
HS(6)-GPC5	Protein	P78333 (Uniprot-TrEMBL)
HS(6)-GPC6	Protein	Q9Y625 (Uniprot-TrEMBL)
HS(6)-HSPG2	Protein	P98160 (Uniprot-TrEMBL)
HS(6)-SDC1	Protein	P18827 (Uniprot-TrEMBL)
HS(6)-SDC2	Protein	P34741 (Uniprot-TrEMBL)
HS(6)-SDC3	Protein	O75056 (Uniprot-TrEMBL)
HS(6)-SDC4	Protein	P31431 (Uniprot-TrEMBL)
HS/HPIN-PGs		R-HSA-2076357 (Reactome)
HS/HPIN-PGs		R-HSA-2076639 (Reactome)
HS/HPIN-PGs	Complex	R-HSA-2076357 (Reactome)
HS/HPIN-PGs	Complex	R-HSA-2076639 (Reactome)
HS/HPIN-PGs	Complex	R-HSA-2090050 (Reactome)
HS2ST1	Protein	Q7LGA3 (Uniprot-TrEMBL)
HS3ST sulfotransferases	Complex	R-HSA-2869445 (Reactome)	This CandidateSet contains sequences identified by William Pearson's analysis of Reactome catalyst entities. Catalyst entity sequences were used to identify analagous sequences that shared overall homology and active site homology. Sequences in this Candidate set were identified in an April 24, 2012 analysis.
HS3ST1	Protein	O14792 (Uniprot-TrEMBL)
HS3ST1	Protein	O14792 (Uniprot-TrEMBL)
HS3ST2	Protein	Q9Y278 (Uniprot-TrEMBL)
HS3ST3A1	Protein	Q9Y663 (Uniprot-TrEMBL)
HS3ST3B1	Protein	Q9Y662 (Uniprot-TrEMBL)
HS3ST4	Protein	Q9Y661 (Uniprot-TrEMBL)
HS3ST5	Protein	Q8IZT8 (Uniprot-TrEMBL)
HS3ST6	Protein	Q96QI5 (Uniprot-TrEMBL)
HS3STs	Complex	R-HSA-2076438 (Reactome)
HS6ST1	Protein	O60243 (Uniprot-TrEMBL)
HS6ST2	Protein	Q96MM7 (Uniprot-TrEMBL)
HS6ST3	Protein	Q8IZP7 (Uniprot-TrEMBL)
HS6STs	Complex	R-HSA-2076470 (Reactome)
HSPG2(22-4391)	Protein	P98160 (Uniprot-TrEMBL)
HSPGs	Complex	R-HSA-2076618 (Reactome)
HSPGs	Complex	R-HSA-2076619 (Reactome)
HYAL1	Protein	Q12794 (Uniprot-TrEMBL)
HYAL1-like proteins	Complex	R-HSA-3907265 (Reactome)	This CandidateSet contains sequences identified by William Pearson's analysis of Reactome catalyst entities. Catalyst entity sequences were used to identify analagous sequences that shared overall homology and active site homology. Sequences in this Candidate set were identified in an April 24, 2012 analysis.
HYAL2	Protein	Q12891 (Uniprot-TrEMBL)
HYAL2	Protein	Q12891 (Uniprot-TrEMBL)
HYAL3	Protein	O43820 (Uniprot-TrEMBL)
Heparan chain(1)	Metabolite	CHEBI:63811 (ChEBI)
Heparan chain(2)	Metabolite	CHEBI:63645 (ChEBI)
Heparan sulfate chain(1)	Metabolite	CHEBI:63666 (ChEBI)
Heparan sulfate chain(2)	Metabolite	CHEBI:63805 (ChEBI)
Heparan sulfate chain(3)	Metabolite	CHEBI:63806 (ChEBI)
Heparan sulfate chain(4)	Metabolite	CHEBI:63807 (ChEBI)
Heparan sulfate chain(5)	Metabolite	CHEBI:63808 (ChEBI)
Heparan sulfate chain(6)	Metabolite	CHEBI:63809 (ChEBI)
Heparan sulfate chain(7)	Metabolite	CHEBI:63810 (ChEBI)
Heparan sulfate chain	Metabolite	CHEBI:63666 (ChEBI)
Heparan(1)-AGRN	Protein	O00468 (Uniprot-TrEMBL)
Heparan(1)-GPC1	Protein	P35052 (Uniprot-TrEMBL)
Heparan(1)-GPC2	Protein	Q8N158 (Uniprot-TrEMBL)
Heparan(1)-GPC3	Protein	P51654 (Uniprot-TrEMBL)
Heparan(1)-GPC4	Protein	O75487 (Uniprot-TrEMBL)
Heparan(1)-GPC5	Protein	P78333 (Uniprot-TrEMBL)
Heparan(1)-GPC6	Protein	Q9Y625 (Uniprot-TrEMBL)
Heparan(1)-HSPG2	Protein	P98160 (Uniprot-TrEMBL)
Heparan(1)-PGs	Complex	R-HSA-2076416 (Reactome)
Heparan(1)-SDC1	Protein	P18827 (Uniprot-TrEMBL)
Heparan(1)-SDC2	Protein	P34741 (Uniprot-TrEMBL)
Heparan(1)-SDC3	Protein	O75056 (Uniprot-TrEMBL)
Heparan(1)-SDC4	Protein	P31431 (Uniprot-TrEMBL)
Heparan(2)-AGRN	Protein	O00468 (Uniprot-TrEMBL)
Heparan(2)-GPC1	Protein	P35052 (Uniprot-TrEMBL)
Heparan(2)-GPC2	Protein	Q8N158 (Uniprot-TrEMBL)
Heparan(2)-GPC3	Protein	P51654 (Uniprot-TrEMBL)
Heparan(2)-GPC4	Protein	O75487 (Uniprot-TrEMBL)
Heparan(2)-GPC5	Protein	P78333 (Uniprot-TrEMBL)
Heparan(2)-GPC6	Protein	Q9Y625 (Uniprot-TrEMBL)
Heparan(2)-HSPG2	Protein	P98160 (Uniprot-TrEMBL)
Heparan(2)-PGs	Complex	R-HSA-2076412 (Reactome)
Heparan(2)-SDC1	Protein	P18827 (Uniprot-TrEMBL)
Heparan(2)-SDC2	Protein	P34741 (Uniprot-TrEMBL)
Heparan(2)-SDC3	Protein	O75056 (Uniprot-TrEMBL)
Heparan(2)-SDC4	Protein	P31431 (Uniprot-TrEMBL)
Heparan(3)-AGRN	Protein	O00468 (Uniprot-TrEMBL)
Heparan(3)-GPC1	Protein	P35052 (Uniprot-TrEMBL)
Heparan(3)-GPC2	Protein	Q8N158 (Uniprot-TrEMBL)
Heparan(3)-GPC3	Protein	P51654 (Uniprot-TrEMBL)
Heparan(3)-GPC4	Protein	O75487 (Uniprot-TrEMBL)
Heparan(3)-GPC5	Protein	P78333 (Uniprot-TrEMBL)
Heparan(3)-GPC6	Protein	Q9Y625 (Uniprot-TrEMBL)
Heparan(3)-HSPG2	Protein	P98160 (Uniprot-TrEMBL)
Heparan(3)-PGs	Complex	R-HSA-2076535 (Reactome)
Heparan(3)-SDC1	Protein	P18827 (Uniprot-TrEMBL)
Heparan(3)-SDC2	Protein	P34741 (Uniprot-TrEMBL)
Heparan(3)-SDC3	Protein	O75056 (Uniprot-TrEMBL)
Heparan(3)-SDC4	Protein	P31431 (Uniprot-TrEMBL)
Heparan(4)-AGRN	Protein	O00468 (Uniprot-TrEMBL)
Heparan(4)-GPC1	Protein	P35052 (Uniprot-TrEMBL)
Heparan(4)-GPC2	Protein	Q8N158 (Uniprot-TrEMBL)
Heparan(4)-GPC3	Protein	P51654 (Uniprot-TrEMBL)
Heparan(4)-GPC4	Protein	O75487 (Uniprot-TrEMBL)
Heparan(4)-GPC5	Protein	P78333 (Uniprot-TrEMBL)
Heparan(4)-GPC6	Protein	Q9Y625 (Uniprot-TrEMBL)
Heparan(4)-HSPG2	Protein	P98160 (Uniprot-TrEMBL)
Heparan(4)-PGs	Complex	R-HSA-2076346 (Reactome)
Heparan(4)-SDC1	Protein	P18827 (Uniprot-TrEMBL)
Heparan(4)-SDC2	Protein	P34741 (Uniprot-TrEMBL)
Heparan(4)-SDC3	Protein	O75056 (Uniprot-TrEMBL)
Heparan(4)-SDC4	Protein	P31431 (Uniprot-TrEMBL)
Heparan-AGRN	Protein	O00468 (Uniprot-TrEMBL)
Heparan-GPC1	Protein	P35052 (Uniprot-TrEMBL)
Heparan-GPC2	Protein	Q8N158 (Uniprot-TrEMBL)
Heparan-GPC3	Protein	P51654 (Uniprot-TrEMBL)
Heparan-GPC4	Protein	O75487 (Uniprot-TrEMBL)
Heparan-GPC5	Protein	P78333 (Uniprot-TrEMBL)
Heparan-GPC6	Protein	Q9Y625 (Uniprot-TrEMBL)
Heparan-HSPG2	Protein	P98160 (Uniprot-TrEMBL)
Heparan-PGs	Complex	R-HSA-2076465 (Reactome)
Heparan-SDC1	Protein	P18827 (Uniprot-TrEMBL)
Heparan-SDC2	Protein	P34741 (Uniprot-TrEMBL)
Heparan-SDC3	Protein	O75056 (Uniprot-TrEMBL)
Heparan-SDC4	Protein	P31431 (Uniprot-TrEMBL)
IDS dimer	Complex	R-HSA-1678638 (Reactome)
IDS(34-455)	Protein	P22304 (Uniprot-TrEMBL)
IDS(456-550)	Protein	P22304 (Uniprot-TrEMBL)
IDUA	Protein	P35475 (Uniprot-TrEMBL)
IdoA-GalNAc(4S)-GlcA-Gal-Gal-Xyl	Metabolite	CHEBI:63873 (ChEBI)
KERA	Protein	O60938 (Uniprot-TrEMBL)
KS core proteins	Complex	R-HSA-2105011 (Reactome)
KS(1)-ACAN	Protein	P16112 (Uniprot-TrEMBL)
KS(1)-FMOD	Protein	Q06828 (Uniprot-TrEMBL)
KS(1)-KERA	Protein	O60938 (Uniprot-TrEMBL)
KS(1)-LUM	Protein	P51884 (Uniprot-TrEMBL)
KS(1)-OGN	Protein	P20774 (Uniprot-TrEMBL)
KS(1)-OMD	Protein	Q99983 (Uniprot-TrEMBL)
KS(1)-PRELP	Protein	P51888 (Uniprot-TrEMBL)
KS(2)-ACAN	Protein	P16112 (Uniprot-TrEMBL)
KS(2)-FMOD	Protein	Q06828 (Uniprot-TrEMBL)
KS(2)-KERA	Protein	O60938 (Uniprot-TrEMBL)
KS(2)-LUM	Protein	P51884 (Uniprot-TrEMBL)
KS(2)-OGN	Protein	P20774 (Uniprot-TrEMBL)
KS(2)-OMD	Protein	Q99983 (Uniprot-TrEMBL)
KS(2)-PRELP	Protein	P51888 (Uniprot-TrEMBL)
KSPG(1)	Complex	R-HSA-2046166 (Reactome)
KSPG(2)	Complex	R-HSA-2046191 (Reactome)
KSPG(2)	Complex	R-HSA-2046244 (Reactome)
KSPG(2)	Complex	R-HSA-2046288 (Reactome)
Keratan(1)-ACAN	Protein	P16112 (Uniprot-TrEMBL)
Keratan(1)-FMOD	Protein	Q06828 (Uniprot-TrEMBL)
Keratan(1)-KERA	Protein	O60938 (Uniprot-TrEMBL)
Keratan(1)-LUM	Protein	P51884 (Uniprot-TrEMBL)
Keratan(1)-OGN	Protein	P20774 (Uniprot-TrEMBL)
Keratan(1)-OMD	Protein	Q99983 (Uniprot-TrEMBL)
Keratan(1)-PG	Complex	R-HSA-2046187 (Reactome)
Keratan(1)-PRELP	Protein	P51888 (Uniprot-TrEMBL)
Keratan(2)-ACAN	Protein	P16112 (Uniprot-TrEMBL)
Keratan(2)-FMOD	Protein	Q06828 (Uniprot-TrEMBL)
Keratan(2)-KERA	Protein	O60938 (Uniprot-TrEMBL)
Keratan(2)-LUM	Protein	P51884 (Uniprot-TrEMBL)
Keratan(2)-OGN	Protein	P20774 (Uniprot-TrEMBL)
Keratan(2)-OMD	Protein	Q99983 (Uniprot-TrEMBL)
Keratan(2)-PG	Complex	R-HSA-2046206 (Reactome)
Keratan(2)-PRELP	Protein	P51888 (Uniprot-TrEMBL)
Keratan(3)-ACAN	Protein	P16112 (Uniprot-TrEMBL)
Keratan(3)-FMOD	Protein	Q06828 (Uniprot-TrEMBL)
Keratan(3)-KERA	Protein	O60938 (Uniprot-TrEMBL)
Keratan(3)-LUM	Protein	P51884 (Uniprot-TrEMBL)
Keratan(3)-OGN	Protein	P20774 (Uniprot-TrEMBL)
Keratan(3)-OMD	Protein	Q99983 (Uniprot-TrEMBL)
Keratan(3)-PG	Complex	R-HSA-2046314 (Reactome)
Keratan(3)-PRELP	Protein	P51888 (Uniprot-TrEMBL)
Keratan(4)-ACAN	Protein	P16112 (Uniprot-TrEMBL)
Keratan(4)-FMOD	Protein	Q06828 (Uniprot-TrEMBL)
Keratan(4)-KERA	Protein	O60938 (Uniprot-TrEMBL)
Keratan(4)-LUM	Protein	P51884 (Uniprot-TrEMBL)
Keratan(4)-OGN	Protein	P20774 (Uniprot-TrEMBL)
Keratan(4)-OMD	Protein	Q99983 (Uniprot-TrEMBL)
Keratan(4)-PG	Complex	R-HSA-2046203 (Reactome)
Keratan(4)-PRELP	Protein	P51888 (Uniprot-TrEMBL)
L-Asp	Metabolite	CHEBI:17053 (ChEBI)
LUM	Protein	P51884 (Uniprot-TrEMBL)
LYVE1	Protein	Q9Y5Y7 (Uniprot-TrEMBL)
Mn2+	Metabolite	CHEBI:29035 (ChEBI)
N-glycan ACAN	Protein	P16112 (Uniprot-TrEMBL)
N-glycan FMOD	Protein	Q06828 (Uniprot-TrEMBL)
N-glycan KERA	Protein	O60938 (Uniprot-TrEMBL)
N-glycan LUM	Protein	P51884 (Uniprot-TrEMBL)
N-glycan OGN	Protein	P20774 (Uniprot-TrEMBL)
N-glycan OMD	Protein	Q99983 (Uniprot-TrEMBL)
N-glycan PRELP	Protein	P51888 (Uniprot-TrEMBL)
N-glycan-protein	Complex	R-HSA-2046280 (Reactome)
NAGLU(59-743)	Protein	P54802 (Uniprot-TrEMBL)
NCAN	Protein	O14594 (Uniprot-TrEMBL)
NDST1	Protein	P52848 (Uniprot-TrEMBL)
NDST2	Protein	P52849 (Uniprot-TrEMBL)
NDST3	Protein	O95803 (Uniprot-TrEMBL)
NDST4	Protein	Q9H3R1 (Uniprot-TrEMBL)
NDSTs	Complex	R-HSA-2022936 (Reactome)
OGN	Protein	P20774 (Uniprot-TrEMBL)
OMD	Protein	Q99983 (Uniprot-TrEMBL)
OxA-ARSB	Protein	P15848 (Uniprot-TrEMBL)
OxA-GNS	Protein	P15586 (Uniprot-TrEMBL)
PAP	Metabolite	CHEBI:17985 (ChEBI)
PAPS	Metabolite	CHEBI:17980 (ChEBI)
PAPSS1	Protein	O43252 (Uniprot-TrEMBL)
PAPSS1,2	Complex	R-HSA-174400 (Reactome)
PAPSS2	Protein	O95340 (Uniprot-TrEMBL)
PPi	Metabolite	CHEBI:29888 (ChEBI)
PRELP	Protein	P51888 (Uniprot-TrEMBL)
SDC1	Protein	P18827 (Uniprot-TrEMBL)
SDC2	Protein	P34741 (Uniprot-TrEMBL)
SDC3	Protein	O75056 (Uniprot-TrEMBL)
SDC4	Protein	P31431 (Uniprot-TrEMBL)
SGSH	Protein	P51688 (Uniprot-TrEMBL)
SLC26A1	Protein	Q9H2B4 (Uniprot-TrEMBL)
SLC26A1,2	Complex	R-HSA-427632 (Reactome)
SLC26A2	Protein	P50443 (Uniprot-TrEMBL)
SLC35B2	Protein	Q8TB61 (Uniprot-TrEMBL)
SLC35B2,3	Complex	R-HSA-3465611 (Reactome)
SLC35B3	Protein	Q9H1N7 (Uniprot-TrEMBL)
SLC35D2	Protein	Q76EJ3 (Uniprot-TrEMBL)
SLC9A1	Protein	P19634 (Uniprot-TrEMBL)
SLC9A1:p-CHP:Ca2+	Complex	R-HSA-5333682 (Reactome)
SO4(2-)	Metabolite	CHEBI:16189 (ChEBI)
ST3GAL1	Protein	Q11201 (Uniprot-TrEMBL)
ST3GAL1-4,6	Complex	R-HSA-2046170 (Reactome)
ST3GAL2	Protein	Q16842 (Uniprot-TrEMBL)
ST3GAL3	Protein	Q11203 (Uniprot-TrEMBL)
ST3GAL4	Protein	Q11206 (Uniprot-TrEMBL)
ST3GAL6	Protein	Q9Y274 (Uniprot-TrEMBL)
STAB2(1136-2551)	Protein	Q8WWQ8 (Uniprot-TrEMBL)
UDP-Gal	Metabolite	CHEBI:18307 (ChEBI)
UDP-GalNAc	Metabolite	CHEBI:16650 (ChEBI)
UDP-Glc	Metabolite	CHEBI:18066 (ChEBI)
UDP-GlcA	Metabolite	CHEBI:17200 (ChEBI)
UDP-GlcNAc	Metabolite	CHEBI:16264 (ChEBI)
UDP-GlcNAc	Metabolite	CHEBI:16264 (ChEBI)
UDP-sugars	Complex	R-HSA-R-ALL-744229 (Reactome)
UDP-sugars	Complex	R-HSA-R-ALL-744234 (Reactome)
UDP-xyl	Metabolite	CHEBI:16082 (ChEBI)
UDP	Metabolite	CHEBI:17659 (ChEBI)
UMP	Metabolite	CHEBI:16695 (ChEBI)
UST	Protein	Q9Y2C2 (Uniprot-TrEMBL)
VCAN	Protein	P13611 (Uniprot-TrEMBL)
XylS-AGRN(30-2045)	Protein	O00468 (Uniprot-TrEMBL)
XylS-BCAN	Protein	Q96GW7 (Uniprot-TrEMBL)
XylS-BGN	Protein	P21810 (Uniprot-TrEMBL)
XylS-CSPG4	Protein	Q6UVK1 (Uniprot-TrEMBL)
XylS-CSPG5	Protein	O95196 (Uniprot-TrEMBL)
XylS-DCN	Protein	P07585 (Uniprot-TrEMBL)
XylS-GPC1	Protein	P35052 (Uniprot-TrEMBL)
XylS-GPC2	Protein	Q8N158 (Uniprot-TrEMBL)
XylS-GPC3(25-?)	Protein	P51654 (Uniprot-TrEMBL)
XylS-GPC4	Protein	O75487 (Uniprot-TrEMBL)
XylS-GPC5(25-?)	Protein	P78333 (Uniprot-TrEMBL)
XylS-GPC6	Protein	Q9Y625 (Uniprot-TrEMBL)
XylS-HSPG2(22-4391)	Protein	P98160 (Uniprot-TrEMBL)
XylS-NCAN	Protein	O14594 (Uniprot-TrEMBL)
XylS-SDC1	Protein	P18827 (Uniprot-TrEMBL)
XylS-SDC2	Protein	P34741 (Uniprot-TrEMBL)
XylS-SDC3	Protein	O75056 (Uniprot-TrEMBL)
XylS-SDC4	Protein	P31431 (Uniprot-TrEMBL)
XylS-VCAN	Protein	P13611 (Uniprot-TrEMBL)
aldehydo-L-iduronic acid	Metabolite	CHEBI:28481 (ChEBI)
beta-xylosidase		R-HSA-2247521 (Reactome)
chondroitin(1)-BCAN	Protein	Q96GW7 (Uniprot-TrEMBL)
chondroitin(1)-BGN	Protein	P21810 (Uniprot-TrEMBL)
chondroitin(1)-CSPG4	Protein	Q6UVK1 (Uniprot-TrEMBL)
chondroitin(1)-CSPG5	Protein	O95196 (Uniprot-TrEMBL)
chondroitin(1)-DCN	Protein	P07585 (Uniprot-TrEMBL)
chondroitin(1)-NCAN	Protein	O14594 (Uniprot-TrEMBL)
chondroitin(1)-VCAN	Protein	P13611 (Uniprot-TrEMBL)
chondroitin(1)-core proteins	Complex	R-HSA-2064172 (Reactome)
chondroitin(2)-BCAN	Protein	Q96GW7 (Uniprot-TrEMBL)
chondroitin(2)-BGN	Protein	P21810 (Uniprot-TrEMBL)
chondroitin(2)-CSPG4	Protein	Q6UVK1 (Uniprot-TrEMBL)
chondroitin(2)-CSPG5	Protein	O95196 (Uniprot-TrEMBL)
chondroitin(2)-DCN	Protein	P07585 (Uniprot-TrEMBL)
chondroitin(2)-NCAN	Protein	O14594 (Uniprot-TrEMBL)
chondroitin(2)-VCAN	Protein	P13611 (Uniprot-TrEMBL)
chondroitin(2)-core proteins	Complex	R-HSA-2064050 (Reactome)
chondroitin(3)-BCAN	Protein	Q96GW7 (Uniprot-TrEMBL)
chondroitin(3)-BGN	Protein	P21810 (Uniprot-TrEMBL)
chondroitin(3)-CSPG4	Protein	Q6UVK1 (Uniprot-TrEMBL)
chondroitin(3)-CSPG5	Protein	O95196 (Uniprot-TrEMBL)
chondroitin(3)-DCN	Protein	P07585 (Uniprot-TrEMBL)
chondroitin(3)-NCAN	Protein	O14594 (Uniprot-TrEMBL)
chondroitin(3)-VCAN	Protein	P13611 (Uniprot-TrEMBL)
chondroitin(3)-core proteins	Complex	R-HSA-2064075 (Reactome)
chondroitin(3)-core proteins		R-HSA-2064075 (Reactome)
dermatan-core proteins		R-HSA-5607515 (Reactome)
keratan sulfate 1,4-beta-D-galactosidase		R-HSA-1793214 (Reactome)
linker chain(2)	Metabolite	CHEBI:63503 (ChEBI)
p-CHP1	Protein	Q99653 (Uniprot-TrEMBL)
xylosyl-core proteins	Complex	R-HSA-2064201 (Reactome)

Annotated Interactions

View all...

Source	Target	Type	Database reference	Comment
(HA)2			2162227 (Reactome)
	(HA)2	Arrow	2160874 (Reactome)
(HA)50			2160874 (Reactome)
(HA)50			2160906 (Reactome)
	(HA)50	Arrow	2160892 (Reactome)
	(HA)50	Arrow	2160906 (Reactome)
			1606789 (Reactome)
			1678660 (Reactome)
			1678742 (Reactome)
			1678854 (Reactome)
			174389 (Reactome)
			174392 (Reactome)
			1793176 (Reactome)
			1793182 (Reactome)
			1793186 (Reactome)
			1793207 (Reactome)
			1793209 (Reactome)
			1878002 (Reactome)
			1889955 (Reactome)
			1889978 (Reactome)
			1889981 (Reactome)
			1971482 (Reactome)
			1971483 (Reactome)
			1971487 (Reactome)
			1971491 (Reactome)
			2018659 (Reactome)
			2018682 (Reactome)
			2022052 (Reactome)
			2022056 (Reactome)
			2022061 (Reactome)
			2022063 (Reactome)
			2022065 (Reactome)
			2022851 (Reactome)
			2022856 (Reactome)
			2022860 (Reactome)
			2022887 (Reactome)
			2022911 (Reactome)
			2022919 (Reactome)
			2024084 (Reactome)
			2024100 (Reactome)
			2024108 (Reactome)
			2025723 (Reactome)
			2025724 (Reactome)
			2046175 (Reactome)
			2046180 (Reactome)
			2046222 (Reactome)
			2046239 (Reactome)
			2046265 (Reactome)
			2046285 (Reactome)
			2046298 (Reactome)
			2065233 (Reactome)
			2076371 (Reactome)
			2076383 (Reactome)
			2076392 (Reactome)
			2076419 (Reactome)
			2076508 (Reactome)
			2076611 (Reactome)
			2090037 (Reactome)
			2090038 (Reactome)
			2090043 (Reactome)
			2090079 (Reactome)
			2090085 (Reactome)
			2105001 (Reactome)
			2142859 (Reactome)
			2160851 (Reactome)
			2160874 (Reactome)
			2160884 (Reactome)
			2160892 (Reactome)
			2160906 (Reactome)
			2160915 (Reactome)
			2162225 (Reactome)
			2162226 (Reactome)
			2162227 (Reactome)
			2162229 (Reactome)
			427555 (Reactome)
			5693356 (Reactome)
			741449 (Reactome)
			744231 (Reactome)
ABCC5		mim-catalysis	2142859 (Reactome)
	ADP	Arrow	174389 (Reactome)
APS			174389 (Reactome)
	APS	Arrow	174392 (Reactome)
ARSB:Ca2+		mim-catalysis	1606789 (Reactome)
ARSB:Ca2+		mim-catalysis	1793207 (Reactome)
ATP			174389 (Reactome)
ATP			174392 (Reactome)
Ac-CoA			1678660 (Reactome)
Ac-CoA			2090085 (Reactome)
B3GALT6		mim-catalysis	1889978 (Reactome)
B3GAT dimers		mim-catalysis	1889955 (Reactome)
B3GNT1,2,3,4,7		mim-catalysis	2025724 (Reactome)
B4GALT1-6 homodimers		mim-catalysis	2025723 (Reactome)
B4GALT1-6 homodimers		mim-catalysis	2046265 (Reactome)
B4GALT1-6 homodimers		mim-catalysis	2046298 (Reactome)
B4GALT7		mim-catalysis	1889981 (Reactome)
BGAL		mim-catalysis	2090079 (Reactome)
BGAL		mim-catalysis	R-HSA-1630306 (Reactome)
C4S-PG			2018659 (Reactome)
	C4S-PG	Arrow	1971483 (Reactome)
C4S/C6S chains			1793207 (Reactome)
	C4S/C6S chains	Arrow	2065233 (Reactome)
	C6S-PG	Arrow	2018682 (Reactome)
CEMIP		mim-catalysis	5693356 (Reactome)
	CH3COO-	Arrow	2022887 (Reactome)
	CHEBI:63868 chain	Arrow	1793186 (Reactome)
CHPF,CHPF2,CHSY1,CHSY3		mim-catalysis	1971491 (Reactome)
CHPF,CHSY1,CHSY3		mim-catalysis	1971487 (Reactome)
	CHST14 products	Arrow	2022063 (Reactome)
CHST14 substrates			2022063 (Reactome)
CHST14		mim-catalysis	2022063 (Reactome)
CHST15		mim-catalysis	2018659 (Reactome)
CHST1		mim-catalysis	2046175 (Reactome)
CHST2,5,6		mim-catalysis	2046222 (Reactome)
CHST3,7		mim-catalysis	2018682 (Reactome)
CHST9,11,12,13		mim-catalysis	1971483 (Reactome)
CMP-Neu5Ac			2046285 (Reactome)
	CMP	Arrow	2046285 (Reactome)
	CS/DS core proteins	Arrow	1793176 (Reactome)
	CS/DS core proteins	Arrow	2065233 (Reactome)
CS/HS precursor			1678854 (Reactome)
	CS/HS precursor	Arrow	1793209 (Reactome)
	CS/HS precursor	Arrow	2090038 (Reactome)
	CSE-PG	Arrow	2018659 (Reactome)
CSGALNACT		mim-catalysis	1971482 (Reactome)
CSPGs			2022056 (Reactome)
CSPGs			2022911 (Reactome)
CSPGs			2065233 (Reactome)
	CSPGs	Arrow	2022056 (Reactome)
	CSPGs	Arrow	2022911 (Reactome)
ChEBI:63516 chain			2105001 (Reactome)
	ChEBI:63516 chain	Arrow	1793182 (Reactome)
ChEBI:63517 chain			1793182 (Reactome)
	ChEBI:63517 chain	Arrow	1606789 (Reactome)
ChEBI:63519 chain			1606789 (Reactome)
	ChEBI:63519 chain	Arrow	1793176 (Reactome)
	Chondroitin chain	Arrow	1793207 (Reactome)
Chondroitin chains			1793209 (Reactome)
	CoA-SH	Arrow	1678660 (Reactome)
	CoA-SH	Arrow	2090085 (Reactome)
	D-xylose	Arrow	2090079 (Reactome)
	D2,4(S)2-PG	Arrow	2022061 (Reactome)
D2,4,4(S)3-PGs			1793176 (Reactome)
D4S-PGs			2022061 (Reactome)
	DSE,DSEL products	Arrow	2022052 (Reactome)
DSE,DSEL substrates			2022052 (Reactome)
DSE,DSEL		mim-catalysis	2022052 (Reactome)
DSPGs			2022056 (Reactome)
DSPGs			2022065 (Reactome)
	DSPGs	Arrow	2022056 (Reactome)
	DSPGs	Arrow	2022065 (Reactome)
EXT1:EXT2		mim-catalysis	2022851 (Reactome)
EXT1:EXT2		mim-catalysis	2022856 (Reactome)
EXT1:EXT2		mim-catalysis	2022919 (Reactome)
EXT1:EXT2		mim-catalysis	2076392 (Reactome)
GAG core proteins			1878002 (Reactome)
GALNS oligomer		mim-catalysis	R-HSA-1630304 (Reactome)
GLCE		mim-catalysis	2024100 (Reactome)
GLCE		mim-catalysis	2076371 (Reactome)
GUSB tetramer		mim-catalysis	1678854 (Reactome)
GUSB tetramer		mim-catalysis	2162226 (Reactome)
GUSB tetramer		mim-catalysis	2162227 (Reactome)
GXYLTs		mim-catalysis	1878002 (Reactome)
	Gal(S)-GlcNAc(S)-Gal-GlcNAc(S)-Gal	Arrow	R-HSA-1793217 (Reactome)
Gal(S)-GlcNAc(S)-Gal-GlcNAc(S)-Gal			R-HSA-1630304 (Reactome)
Gal-Gal-Xyl-proteins			1889955 (Reactome)
	Gal-Gal-Xyl-proteins	Arrow	1889978 (Reactome)
	Gal-GlcNAc(S)-Gal-GlcNAc(S)-Gal	Arrow	R-HSA-1630304 (Reactome)
Gal-GlcNAc(S)-Gal-GlcNAc(S)-Gal			R-HSA-1630306 (Reactome)
	Gal-GlcNAc(S)-Gal	Arrow	R-HSA-1638053 (Reactome)
Gal-Xyl-proteins			1889978 (Reactome)
	Gal-Xyl-proteins	Arrow	1889981 (Reactome)
Gal-glycan-protein			2025724 (Reactome)
	Gal-glycan-protein	Arrow	2025723 (Reactome)
	Gal	Arrow	2090079 (Reactome)
	Gal	Arrow	R-HSA-1630306 (Reactome)
	GalNAc	Arrow	2105001 (Reactome)
	GalNAc	Arrow	R-HSA-1638053 (Reactome)
GlcA-Gal-Gal-Xyl-CS proteins			1971482 (Reactome)
GlcA-Gal-Gal-Xyl-HS proteins			2022919 (Reactome)
	GlcA-Gal-Gal-Xyl-proteins	Arrow	1889955 (Reactome)
GlcA-b1,3-GlcNAc			2160851 (Reactome)
GlcA-b1,3-GlcNAc			2162226 (Reactome)
	GlcA-b1,3-GlcNAc	Arrow	2162225 (Reactome)
	GlcA-b1,3-GlcNAc	Arrow	5693356 (Reactome)
GlcA			2162229 (Reactome)
	GlcA	Arrow	1678854 (Reactome)
	GlcA	Arrow	2162226 (Reactome)
	GlcA	Arrow	2162227 (Reactome)
	GlcA	Arrow	2162229 (Reactome)
	GlcNAc(S)-Gal-GlcNAc(S)-Gal	Arrow	R-HSA-1630306 (Reactome)
GlcNAc(S)-Gal-GlcNAc(S)-Gal			R-HSA-1638032 (Reactome)
	GlcNAc-Gal-GlcNAc(S)-Gal	Arrow	R-HSA-1638032 (Reactome)
GlcNAc-Gal-GlcNAc(S)-Gal			R-HSA-1638053 (Reactome)
GlcNAc-GlcA-GlcNAc			2162225 (Reactome)
	GlcNAc-GlcA-GlcNAc	Arrow	2162227 (Reactome)
GlcNAc			2162229 (Reactome)
	GlcNAc	Arrow	1678742 (Reactome)
	GlcNAc	Arrow	2090038 (Reactome)
	GlcNAc	Arrow	2162225 (Reactome)
	GlcNAc	Arrow	2162226 (Reactome)
	GlcNAc	Arrow	2162229 (Reactome)
H+			427555 (Reactome)
	H+	Arrow	427555 (Reactome)
H2O			1606789 (Reactome)
H2O			1678742 (Reactome)
H2O			1678854 (Reactome)
H2O			1793176 (Reactome)
H2O			1793182 (Reactome)
H2O			1793186 (Reactome)
H2O			1793207 (Reactome)
H2O			1793209 (Reactome)
H2O			2022887 (Reactome)
H2O			2065233 (Reactome)
H2O			2090037 (Reactome)
H2O			2090038 (Reactome)
H2O			2090043 (Reactome)
H2O			2090079 (Reactome)
H2O			2105001 (Reactome)
H2O			2162225 (Reactome)
H2O			2162226 (Reactome)
H2O			2162227 (Reactome)
H2O			R-HSA-1630304 (Reactome)
H2O			R-HSA-1630306 (Reactome)
H2O			R-HSA-1638032 (Reactome)
H2O			R-HSA-1638053 (Reactome)
H2O			R-HSA-1667005 (Reactome)
H2O			R-HSA-1678650 (Reactome)
H2O			R-HSA-1678694 (Reactome)
H2O			R-HSA-1678708 (Reactome)
H2O			R-HSA-1678716 (Reactome)
H2O			R-HSA-1793217 (Reactome)
HA polymer			2142859 (Reactome)
	HA polymer	Arrow	2160851 (Reactome)
HA			2160915 (Reactome)
HA			5693356 (Reactome)
HA:HAR:HYAL2			2160884 (Reactome)
HA:HAR:HYAL2:SLC9A1:pCHP:Ca2+			2160892 (Reactome)
	HA:HAR:HYAL2:SLC9A1:pCHP:Ca2+	Arrow	2160884 (Reactome)
	HA:HAR:HYAL2:SLC9A1:pCHP:Ca2+	Arrow	2160892 (Reactome)
HA:HAR:HYAL2:SLC9A1:pCHP:Ca2+		mim-catalysis	2160892 (Reactome)
	HA:HAR:HYAL2	Arrow	2160915 (Reactome)
	HA	Arrow	2142859 (Reactome)
	HA	Arrow	5693356 (Reactome)
HARs			2160915 (Reactome)
HAS1,2,3		mim-catalysis	2160851 (Reactome)
HEXA,B		mim-catalysis	2105001 (Reactome)
HEXA,B		mim-catalysis	2162225 (Reactome)
HEXA,B		mim-catalysis	R-HSA-1638053 (Reactome)
HGSNAT oligomer		mim-catalysis	1678660 (Reactome)
HGSNAT oligomer		mim-catalysis	2090085 (Reactome)
HPSE dimer		mim-catalysis	R-HSA-1667005 (Reactome)
HPSE2(1-592)		mim-catalysis	R-HSA-1678694 (Reactome)
	HS core proteins	Arrow	R-HSA-1667005 (Reactome)
	HS core proteins	Arrow	R-HSA-1678694 (Reactome)
HS(1)-PGs			2024100 (Reactome)
	HS(1)-PGs	Arrow	2022860 (Reactome)
HS(2)-PGs			2076508 (Reactome)
	HS(2)-PGs	Arrow	2024100 (Reactome)
HS(3)-PGs			2076383 (Reactome)
HS(3)-PGs			2076611 (Reactome)
	HS(3)-PGs	Arrow	2076508 (Reactome)
HS(4)-PGs			2076371 (Reactome)
	HS(4)-PGs	Arrow	2076383 (Reactome)
	HS(4)-PGs	Arrow	2076611 (Reactome)
HS(5)-PGs			2076419 (Reactome)
	HS(5)-PGs	Arrow	2076371 (Reactome)
HS/HPIN-PGs			2024084 (Reactome)
	HS/HPIN-PGs	Arrow	2024084 (Reactome)
	HS/HPIN-PGs	Arrow	2076419 (Reactome)
HS/HPIN-PGs			R-HSA-1667005 (Reactome)
HS/HPIN-PGs			R-HSA-1678694 (Reactome)
HS2ST1		mim-catalysis	2076508 (Reactome)
HS3ST sulfotransferases		mim-catalysis	2076383 (Reactome)
HS3STs		mim-catalysis	2076611 (Reactome)
HS6STs		mim-catalysis	2076419 (Reactome)
HSPGs			2024108 (Reactome)
	HSPGs	Arrow	2024108 (Reactome)
HYAL1-like proteins		mim-catalysis	1793209 (Reactome)
HYAL1-like proteins		mim-catalysis	2160874 (Reactome)
HYAL2			2160915 (Reactome)
Heparan chain(1)			2090085 (Reactome)
	Heparan chain(1)	Arrow	2090043 (Reactome)
Heparan chain(2)			2090038 (Reactome)
	Heparan chain(2)	Arrow	2090085 (Reactome)
	Heparan sulfate chain(1)	Arrow	R-HSA-1667005 (Reactome)
Heparan sulfate chain(1)			R-HSA-1678716 (Reactome)
	Heparan sulfate chain(2)	Arrow	R-HSA-1678716 (Reactome)
Heparan sulfate chain(2)			R-HSA-1678708 (Reactome)
Heparan sulfate chain(3)			1678660 (Reactome)
	Heparan sulfate chain(3)	Arrow	R-HSA-1678708 (Reactome)
Heparan sulfate chain(4)			1678742 (Reactome)
	Heparan sulfate chain(4)	Arrow	1678660 (Reactome)
	Heparan sulfate chain(5)	Arrow	1678742 (Reactome)
Heparan sulfate chain(5)			R-HSA-1678650 (Reactome)
Heparan sulfate chain(6)			2090037 (Reactome)
	Heparan sulfate chain(6)	Arrow	R-HSA-1678650 (Reactome)
Heparan sulfate chain(7)			2090043 (Reactome)
	Heparan sulfate chain(7)	Arrow	2090037 (Reactome)
	Heparan sulfate chain	Arrow	R-HSA-1678694 (Reactome)
Heparan(1)-PGs			2022851 (Reactome)
	Heparan(1)-PGs	Arrow	2022856 (Reactome)
Heparan(2)-PGs			2076392 (Reactome)
	Heparan(2)-PGs	Arrow	2022851 (Reactome)
Heparan(3)-PGs			2022887 (Reactome)
	Heparan(3)-PGs	Arrow	2076392 (Reactome)
Heparan(4)-PGs			2022860 (Reactome)
	Heparan(4)-PGs	Arrow	2022887 (Reactome)
Heparan-PGs			2022856 (Reactome)
	Heparan-PGs	Arrow	2022919 (Reactome)
IDS dimer		mim-catalysis	1793182 (Reactome)
IDS dimer		mim-catalysis	R-HSA-1678650 (Reactome)
IDUA		mim-catalysis	1793186 (Reactome)
IDUA		mim-catalysis	2090037 (Reactome)
IDUA		mim-catalysis	R-HSA-1678716 (Reactome)
IdoA-GalNAc(4S)-GlcA-Gal-Gal-Xyl			1793186 (Reactome)
	IdoA-GalNAc(4S)-GlcA-Gal-Gal-Xyl	Arrow	2105001 (Reactome)
	KS core proteins	Arrow	R-HSA-1793217 (Reactome)
KSPG(1)			2046175 (Reactome)
	KSPG(1)	Arrow	2046222 (Reactome)
KSPG(2)			2046180 (Reactome)
KSPG(2)			2046239 (Reactome)
	KSPG(2)	Arrow	2046175 (Reactome)
	KSPG(2)	Arrow	2046180 (Reactome)
	KSPG(2)	Arrow	2046239 (Reactome)
KSPG(2)			R-HSA-1793217 (Reactome)
Keratan(1)-PG			2046265 (Reactome)
	Keratan(1)-PG	Arrow	2025724 (Reactome)
Keratan(2)-PG			2046298 (Reactome)
	Keratan(2)-PG	Arrow	2046265 (Reactome)
Keratan(3)-PG			2046285 (Reactome)
	Keratan(3)-PG	Arrow	2046298 (Reactome)
Keratan(4)-PG			2046222 (Reactome)
	Keratan(4)-PG	Arrow	2046285 (Reactome)
L-Asp		TBar	1678854 (Reactome)
N-glycan-protein			2025723 (Reactome)
NAGLU(59-743)		mim-catalysis	1678742 (Reactome)
NAGLU(59-743)		mim-catalysis	2090038 (Reactome)
NDSTs		mim-catalysis	2022860 (Reactome)
NDSTs		mim-catalysis	2022887 (Reactome)
OxA-GNS		mim-catalysis	R-HSA-1638032 (Reactome)
	PAP	Arrow	1971483 (Reactome)
	PAP	Arrow	2018659 (Reactome)
	PAP	Arrow	2018682 (Reactome)
	PAP	Arrow	2022061 (Reactome)
	PAP	Arrow	2022063 (Reactome)
	PAP	Arrow	2022860 (Reactome)
	PAP	Arrow	2046175 (Reactome)
	PAP	Arrow	2046222 (Reactome)
	PAP	Arrow	2076383 (Reactome)
	PAP	Arrow	2076419 (Reactome)
	PAP	Arrow	2076508 (Reactome)
	PAP	Arrow	2076611 (Reactome)
PAPS			1971483 (Reactome)
PAPS			2018659 (Reactome)
PAPS			2018682 (Reactome)
PAPS			2022061 (Reactome)
PAPS			2022063 (Reactome)
PAPS			2022860 (Reactome)
PAPS			2046175 (Reactome)
PAPS			2046222 (Reactome)
PAPS			2076383 (Reactome)
PAPS			2076419 (Reactome)
PAPS			2076508 (Reactome)
PAPS			2076611 (Reactome)
PAPS			741449 (Reactome)
	PAPS	Arrow	174389 (Reactome)
	PAPS	Arrow	741449 (Reactome)
PAPSS1,2		mim-catalysis	174389 (Reactome)
PAPSS1,2		mim-catalysis	174392 (Reactome)
	PPi	Arrow	174392 (Reactome)
			R-HSA-1630304 (Reactome)	N-acetylgalactosamine 6-sulfate sulfatase (GALNS) hydrolyses sulfate from galactose 6-sulfate units of keratan sulfate (KS, shown here) and sulfate from N-acetyl-D-galactosamine 6-sulfate units of chondroitin sulfate (CS, not shown) (Lim & Horwitz 1981, Masue et al. 1991). The conversion to 3-oxoalanine (C-formylglycine, FGly) of a cysteine residue in eukaryotes, is critical for catalytic activity, based on similarity to the prototypical arylsulfatase ARSA (Chruszcz et al. 2003, Lukatela et al. 1998). Defects in GALNS cause mucopolysaccharidosis type IVA (MPSIVA, MIM:253000), also called Morquio A syndrome, a lysosomal storage disease characterized by intracellular accumulation of KS and CS (Fukuda et al. 1992).
			R-HSA-1630306 (Reactome)	Beta-galactosidase (GLB1) can cleave terminal galactose residues from glycosaminoglycans such as keratan sulfate (KS) (Asp et al. 1969). Defects in GLB1 cause the lysosomal storage diseases GM1gangliosidosis (Yoshida et al. 1991) and Morquio syndrome type B (Oshima et al. 1991).
			R-HSA-1638032 (Reactome)	N-acetylglucosamine 6-sulfatase (GNS) is a lysosomal enzyme which degrades glycosaminoglycans such as heparan sulfate and keratan sulfate. GNS shows strong sequence similarity to other sulphatases such as the family of arylsulfatases and the conversion to 3-oxo-alanine (formylglycine, FGly) of a cysteine residue is critical for catalytic activity, based on this similarity (Robertson et al. 1992, Robertson et al. 1988). Defects in GNS are the cause of mucopolysaccharidosis type IIID (MPSIIID, MIM:252940), also called Sanfilippo D syndrome (Valstar et al. 2010).
			R-HSA-1638053 (Reactome)	Beta-hexosaminidase (HEX) cleaves the terminal N-acetyl galactosamine (GalNAc) from glucosaminoglycans (GAGs) and any other molecules containing a terminal GalNAc. There are two forms of HEX: HEXA and B. The A form is a trimer of the subunits alpha, beta A and beta B. The B form is a tetramer of 2 beta A and 2 beta B subunits (O'Dowd et al. 1988). Defects in the two subunits cause lysosomal storage diseases marked by the accumulation of GM2 gangliosides in neuronal cells.
			R-HSA-1667005 (Reactome)	Heparanase (HPSE) is an endoglycosidase that cleaves heparan sulfate (HS) from its HS proteoglycan (HSPG) (Toyoshima & Nakajima 1999). The formation of a heterodimer of 8kDa and 50kDa subunits cleaved from the 65kDa form is required for enzyme activity (Levy-Adam et al. 2003) and this proteolytic cleavage occurs in the lysosome (Goldshmidt et al. 2002). Acidic conditions within the lysosome optimises HPSE activity.
			R-HSA-1678650 (Reactome)	Iduronate 2sulfatase (IDS) hydrolyses 2-sulfate groups from Liduronate 2-sulfate units of heparan sulfate. Defects in IDS are the cause of mucopolysaccharidosis type II (MPSII, MIM:309900), also called Hunter syndrome (Wilson et al. 1990).
			R-HSA-1678694 (Reactome)	Heparanase 2 (HPSE2) (McKenzie et al. 2000) is a membrane-bound endoglycosidase that cleaves heparan sulfate (HS) from its HS proteoglycan (HSPG), either in the extracellular matrix or the basement membranes of cells. Defects in HPSE2 are the cause of urofacial syndrome (UFS) (MIM:236730) (Daly et al. 2010, Pang et al. 2010).
			R-HSA-1678708 (Reactome)	N-sulphoglucosamine sulphohydrolase (SGSH) hydrolyses the sulfate group from the terminal N-sulphoglucosamine residue of heparan sulfate (Scott et al. 1995). Defects in SGSH cause mucopolysaccharidosis type IIIA (MPSIIIA, MIM:252900), also called Sanfilippo syndrome A (Weber et al. 1997).
			R-HSA-1678716 (Reactome)	An L-iduronic acid residue can be cleaved from either heparan sulfate or dermatan sulfate by the lysosomal enzyme alpha-L-iduronidase (IDUA) (Scott et al. 1991). Defects in IDUA are the cause of mucopolysaccharidosis type IH (MPS IH, Hurler syndrome, MIM:607014), mucopolysaccharidosis IH/S (MPSIH/S, HurlerScheie syndrome, MIM:607015) and mucopolysaccharidosis type IS (MPSIS, Scheie syndrome, MIM:607016) (LeeChen et al. 1999).
			R-HSA-1793217 (Reactome)	Keratan sulfate (KS) is cleaved from its KS proteoglycan (KSPG) by an as yet unknown beta-galactosidase. It performs a similar function to beta-galactosidase GLB1 (Asp et al. 1969). A simplified version of KS is used to demonstrate cleavage reactions.
SGSH		mim-catalysis	2090043 (Reactome)
SGSH		mim-catalysis	R-HSA-1678708 (Reactome)
SLC26A1,2		mim-catalysis	427555 (Reactome)
SLC35B2,3		mim-catalysis	741449 (Reactome)
SLC35D2		mim-catalysis	744231 (Reactome)
SLC9A1:p-CHP:Ca2+			2160884 (Reactome)
SO4(2-)			174392 (Reactome)
SO4(2-)			427555 (Reactome)
	SO4(2-)	Arrow	1606789 (Reactome)
	SO4(2-)	Arrow	1793182 (Reactome)
	SO4(2-)	Arrow	1793207 (Reactome)
	SO4(2-)	Arrow	2090043 (Reactome)
	SO4(2-)	Arrow	427555 (Reactome)
	SO4(2-)	Arrow	R-HSA-1630304 (Reactome)
	SO4(2-)	Arrow	R-HSA-1638032 (Reactome)
	SO4(2-)	Arrow	R-HSA-1678650 (Reactome)
	SO4(2-)	Arrow	R-HSA-1678708 (Reactome)
ST3GAL1-4,6		mim-catalysis	2046285 (Reactome)
UDP-Gal			1889978 (Reactome)
UDP-Gal			1889981 (Reactome)
UDP-Gal			2025723 (Reactome)
UDP-Gal			2046265 (Reactome)
UDP-Gal			2046298 (Reactome)
UDP-GalNAc			1971482 (Reactome)
UDP-GalNAc			1971487 (Reactome)
UDP-GlcA			1889955 (Reactome)
UDP-GlcA			1971491 (Reactome)
UDP-GlcA			2022856 (Reactome)
UDP-GlcA			2076392 (Reactome)
UDP-GlcNAc			2022851 (Reactome)
UDP-GlcNAc			2022919 (Reactome)
UDP-GlcNAc			2025724 (Reactome)
UDP-sugars			744231 (Reactome)
	UDP-sugars	Arrow	744231 (Reactome)
UDP-xyl			1878002 (Reactome)
	UDP	Arrow	1878002 (Reactome)
	UDP	Arrow	1889955 (Reactome)
	UDP	Arrow	1889978 (Reactome)
	UDP	Arrow	1889981 (Reactome)
	UDP	Arrow	1971482 (Reactome)
	UDP	Arrow	1971487 (Reactome)
	UDP	Arrow	1971491 (Reactome)
	UDP	Arrow	2022851 (Reactome)
	UDP	Arrow	2022856 (Reactome)
	UDP	Arrow	2022919 (Reactome)
	UDP	Arrow	2025723 (Reactome)
	UDP	Arrow	2025724 (Reactome)
	UDP	Arrow	2046265 (Reactome)
	UDP	Arrow	2046298 (Reactome)
	UDP	Arrow	2076392 (Reactome)
UMP			744231 (Reactome)
	UMP	Arrow	744231 (Reactome)
UST		mim-catalysis	2022061 (Reactome)
	aldehydo-L-iduronic acid	Arrow	1793186 (Reactome)
	aldehydo-L-iduronic acid	Arrow	2090037 (Reactome)
	aldehydo-L-iduronic acid	Arrow	R-HSA-1678716 (Reactome)
beta-xylosidase		mim-catalysis	1793176 (Reactome)
beta-xylosidase		mim-catalysis	2065233 (Reactome)
chondroitin(1)-core proteins			1971491 (Reactome)
	chondroitin(1)-core proteins	Arrow	1971482 (Reactome)
chondroitin(2)-core proteins			1971487 (Reactome)
	chondroitin(2)-core proteins	Arrow	1971491 (Reactome)
chondroitin(3)-core proteins			1971483 (Reactome)
chondroitin(3)-core proteins			2018682 (Reactome)
	chondroitin(3)-core proteins	Arrow	1971487 (Reactome)
keratan sulfate 1,4-beta-D-galactosidase		mim-catalysis	R-HSA-1793217 (Reactome)
linker chain(2)			2090079 (Reactome)
	linker chain(2)	Arrow	1678854 (Reactome)
xylosyl-core proteins			1889981 (Reactome)
	xylosyl-core proteins	Arrow	1878002 (Reactome)