Enzymes
| UniProtKB help_outline | 12 proteins |
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Name help_outline
O3-(N-acetyl-α-D-glucosaminyl-poly[(1→4)-β-D-glucuronosyl-(1→4)-N-acetyl-α-D-glucosaminyl]-(1→4)-β-D-glucuronosyl-(1→3)-β-D-galactosyl-(1→3)-β-D-galactosyl-(1→4)-β-D-xylosyl)-L-serine residue
Identifier
CHEBI:132416
Charge
-2
Formula
(C14H20NO11)n.C34H53N2O27
Search links
Involved in 2 reaction(s)
Find proteins in UniProtKB for this molecule
Form(s) in this reaction:
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Identifier: RHEA-COMP:12623Polymer name: 3-O-{α-D-GlcNAc-[(1→4)-β-D-GlcA-(1→4)-α-D-GlcNAc](n)-(1→4)-β-D-GlcA-(1→3)-β-D-Gal-(1→3)-β-D-Gal-(1→4)-β-D-Xyl}-L-seryl-[protein]Polymerization index help_outline nFormula C34H53N2O27(C14H20NO11)nCharge (-1)(-1)nMol File for the polymer
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- Name help_outline UDP-α-D-glucuronate Identifier CHEBI:58052 Charge -3 Formula C15H19N2O18P2 InChIKeyhelp_outline HDYANYHVCAPMJV-LXQIFKJMSA-K SMILEShelp_outline O[C@@H]1[C@@H](COP([O-])(=O)OP([O-])(=O)O[C@H]2O[C@@H]([C@@H](O)[C@H](O)[C@H]2O)C([O-])=O)O[C@H]([C@@H]1O)n1ccc(=O)[nH]c1=O 2D coordinates Mol file for the small molecule Search links Involved in 107 reaction(s) Find molecules that contain or resemble this structure Find proteins in UniProtKB for this molecule
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Name help_outline
O3-(poly[(1→4)-β-D-glucuronosyl-(1→4)-N-acetyl-α-D-glucosaminyl]-(1→4)-β-D-glucuronosyl-(1→3)-β-D-galactosyl-(1→3)-β-D-galactosyl-(1→4)-β-D-xylosyl)-L-serine residue
Identifier
CHEBI:132415
Charge
Formula
(C14H20NO11)n.C26H40NO22
Search links
Involved in 2 reaction(s)
Find proteins in UniProtKB for this molecule
Form(s) in this reaction:
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Identifier: RHEA-COMP:14295Polymer name: 3-O-{[(1→4)-β-D-GlcA-(1→4)-α-D-GlcNAc](n+1)-(1→4)-β-D-GlcA-(1→3)-β-D-Gal-(1→3)-β-D-Gal-(1→4)-β-D-Xyl}-L-seryl-[protein]Polymerization index help_outline n+1Formula C26H40NO22(C14H20NO11)n+1Charge (-1)(-1)n+1Mol File for the polymer
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- Name help_outline H+ Identifier CHEBI:15378 Charge 1 Formula H InChIKeyhelp_outline GPRLSGONYQIRFK-UHFFFAOYSA-N SMILEShelp_outline [H+] 2D coordinates Mol file for the small molecule Search links Involved in 9,431 reaction(s) Find molecules that contain or resemble this structure Find proteins in UniProtKB for this molecule
- Name help_outline UDP Identifier CHEBI:58223 Charge -3 Formula C9H11N2O12P2 InChIKeyhelp_outline XCCTYIAWTASOJW-XVFCMESISA-K SMILEShelp_outline O[C@@H]1[C@@H](COP([O-])(=O)OP([O-])([O-])=O)O[C@H]([C@@H]1O)n1ccc(=O)[nH]c1=O 2D coordinates Mol file for the small molecule Search links Involved in 576 reaction(s) Find molecules that contain or resemble this structure Find proteins in UniProtKB for this molecule
Cross-references
| RHEA:20908 | RHEA:20909 | RHEA:20910 | RHEA:20911 | |
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| Reaction direction help_outline | undefined | left-to-right | right-to-left | bidirectional |
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Publications
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The EXT1/EXT2 tumor suppressors: catalytic activities and role in heparan sulfate biosynthesis.
Senay C., Lind T., Muguruma K., Tone Y., Kitagawa H., Sugahara K., Lidholt K., Lindahl U., Kusche-Gullberg M.
The D-glucuronyltransferase and N-acetyl-D-glucosaminyltransferase reactions in heparan sulfate biosynthesis have been associated with two genes, EXT1 and EXT2, which are also implicated in the inherited bone disorder, multiple exostoses. Since the cell systems used to express recombinant EXT prot ... >> More
The D-glucuronyltransferase and N-acetyl-D-glucosaminyltransferase reactions in heparan sulfate biosynthesis have been associated with two genes, EXT1 and EXT2, which are also implicated in the inherited bone disorder, multiple exostoses. Since the cell systems used to express recombinant EXT proteins synthesize endogenous heparan sulfate, and the EXT proteins tend to associate, it has not been possible to define the functional roles of the individual protein species. We therefore expressed EXT1 and EXT2 in yeast, which does not synthesize heparan sulfate. The recombinant EXT1 and EXT2 were both found to catalyze both glycosyltransferase reactions in vitro. Coexpression of the two proteins, but not mixing of separately expressed recombinant EXT1 and EXT2, yields hetero-oligomeric complexes in yeast and mammalian cells, with augmented glycosyltransferase activities. This stimulation does not depend on the membrane-bound state of the proteins. << Less
EMBO Rep 1:282-286(2000) [PubMed] [EuropePMC]
This publication is cited by 1 other entry.
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The putative tumor suppressors EXT1 and EXT2 are glycosyltransferases required for the biosynthesis of heparan sulfate.
Lind T., Tufaro F., McCormick C., Lindahl U., Lidholt K.
Hereditary multiple exostoses, characterized by multiple cartilaginous tumors, is ascribed to mutations at three distinct loci, denoted EXT1-3. Here, we report the purification of a protein from bovine serum that harbored the D-glucuronyl (GlcA) and N-acetyl-D-glucosaminyl (GlcNAc) transferase act ... >> More
Hereditary multiple exostoses, characterized by multiple cartilaginous tumors, is ascribed to mutations at three distinct loci, denoted EXT1-3. Here, we report the purification of a protein from bovine serum that harbored the D-glucuronyl (GlcA) and N-acetyl-D-glucosaminyl (GlcNAc) transferase activities required for biosynthesis of the glycosaminoglycan, heparan sulfate (HS). This protein was identified as EXT2. Expression of EXT2 yielded a protein with both glycosyltransferase activities. Moreover, EXT1, previously found to rescue defective HS biosynthesis (McCormick, C., Leduc, Y., Martindale, D., Mattison, K., Esford, L. E., Dyer, A. P., and Tufaro, F. (1998) Nat. Genet. 19, 158-161), was shown to elevate the low GlcA and GlcNAc transferase levels of mutant cells. Thus at least two members of the EXT family of tumor suppressors encode glycosyltransferases involved in the chain elongation step of HS biosynthesis. << Less
J. Biol. Chem. 273:26265-26268(1998) [PubMed] [EuropePMC]
This publication is cited by 1 other entry.