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Namehelp_outline
3-O-[β-D-Xyl-(1→4)-Rib-ol-P-Rib-ol-P-3-β-D-GalNAc-(1→3)-β-D-GlcNAc-(1→4)-(O-6-P-α-D-Man)]-Thr-[protein]
Identifier
RHEA-COMP:15023
Reactive part
help_outline
- Name help_outline 3-O-[β-D-xylosyl-(1→4)-(D-ribitylphospho)2-3-N-acetyl-β-D-galactosaminyl-(1→3)-N-acetyl-β-D-glucosaminyl-(1→4)-O-6-phospho-α-D-mannosyl]-L-threonine residue Identifier CHEBI:142405 Charge -4 Formula C41H70N3O38P3 SMILEShelp_outline O([C@@H]1[C@H]([C@H]([C@@H]([C@H](O1)COP([O-])([O-])=O)O[C@H]2[C@@H]([C@H]([C@@H]([C@H](O2)CO)O)O[C@H]3[C@@H]([C@H]([C@H]([C@H](O3)CO)O)OP(OC[C@H]([C@H]([C@H](COP(OC[C@H]([C@H]([C@H](CO)O)O)O[C@H]4[C@@H]([C@H]([C@@H](CO4)O)O)O)([O-])=O)O)O)O)([O-])=O)NC(C)=O)NC(C)=O)O)O)[C@@H]([C@@H](C(*)=O)N*)C 2D coordinates Mol file for the small molecule Search links Involved in 2 reaction(s) Find molecules that contain or resemble this structure Find proteins in UniProtKB for this molecule
- 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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Namehelp_outline
3-O-[β-D-GlcA-(1→3)-β-D-Xyl-(1→4)-Rib-ol-P-Rib-ol-P-3-β-D-GalNAc-(1→3)-β-D-GlcNAc-(1→4)-(O-6-P-α-D-Man)]-Thr-[protein]
Identifier
RHEA-COMP:17482
Reactive part
help_outline
- Name help_outline 3-O-[β-D-GlcA-(1→3)-β-D-Xyl-(1→4)-Rib-ol-P-Rib-ol-P-3-β-D-GalNAc-(1→3)-β-D-GlcNAc-(1→4)-O-6-P-α-D-Man]-L-threonine residue Identifier CHEBI:177336 Charge -5 Formula C47H77N3O44P3 SMILEShelp_outline O([C@@H]1[C@H]([C@H]([C@@H]([C@H](O1)COP([O-])([O-])=O)O[C@H]2[C@@H]([C@H]([C@@H]([C@H](O2)CO)O)O[C@H]3[C@@H]([C@H]([C@H]([C@H](O3)CO)O)OP(OC[C@H]([C@H]([C@H](COP(OC[C@H]([C@H]([C@H](CO)O)O)O[C@H]4[C@@H]([C@H]([C@@H](CO4)O[C@H]5[C@@H]([C@H]([C@@H]([C@H](O5)C([O-])=O)O)O)O)O)O)([O-])=O)O)O)O)([O-])=O)NC(C)=O)NC(C)=O)O)O)[C@@H]([C@@H](C(*)=O)N*)C 2D coordinates Mol file for the small molecule Search links Involved in 2 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 637 reaction(s) Find molecules that contain or resemble this structure Find proteins in UniProtKB for this molecule
- 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 9,932 reaction(s) Find molecules that contain or resemble this structure Find proteins in UniProtKB for this molecule
Cross-references
| RHEA:46860 | RHEA:46861 | RHEA:46862 | RHEA:46863 | |
|---|---|---|---|---|
| Reaction direction help_outline | undefined | left-to-right | right-to-left | bidirectional |
| UniProtKB help_outline |
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| KEGG help_outline | ||||
| MetaCyc help_outline |
Publications
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The glucuronyltransferase B4GAT1 is required for initiation of LARGE-mediated alpha-dystroglycan functional glycosylation.
Willer T., Inamori K.I., Venzke D., Harvey C., Morgensen G., Hara Y., Beltran Valero de Bernabe D., Yu L., Wright K.M., Campbell K.P.
Dystroglycan is a cell membrane receptor that organizes the basement membrane by binding ligands in the extracellular matrix. Proper glycosylation of the α-dystroglycan (α-DG) subunit is essential for these activities, and lack thereof results in neuromuscular disease. Currently, neither the glyca ... >> More
Dystroglycan is a cell membrane receptor that organizes the basement membrane by binding ligands in the extracellular matrix. Proper glycosylation of the α-dystroglycan (α-DG) subunit is essential for these activities, and lack thereof results in neuromuscular disease. Currently, neither the glycan synthesis pathway nor the roles of many known or putative glycosyltransferases that are essential for this process are well understood. Here we show that FKRP, FKTN, TMEM5 and B4GAT1 (formerly known as B3GNT1) localize to the Golgi and contribute to the O-mannosyl post-phosphorylation modification of α-DG. Moreover, we assigned B4GAT1 a function as a xylose β1,4-glucuronyltransferase. Nuclear magnetic resonance studies confirmed that a glucuronic acid β1,4-xylose disaccharide synthesized by B4GAT1 acts as an acceptor primer that can be elongated by LARGE with the ligand-binding heteropolysaccharide. Our findings greatly broaden the understanding of α-DG glycosylation and provide mechanistic insight into why mutations in B4GAT1 disrupt dystroglycan function and cause disease. << Less