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Namehelp_outline
3-O-[β-D-GalNAc-(1→3)-β-D-GlcNAc-(1→4)-(O-6-P-α-D-Man)]-Thr-[protein]
Identifier
RHEA-COMP:13309
Reactive part
help_outline
- Name help_outline N-acetyl-β-D-galactosaminyl-(1→3)-N-acetyl-β-D-glucosaminyl-(1→4)-6-O-phospho-α-D-mannosyl-L-threonine residue Identifier CHEBI:136710 Charge -2 Formula C26H42N3O20P SMILEShelp_outline [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)O)NC(C)=O)NC(C)=O)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 CDP-L-ribitol Identifier CHEBI:57608 Charge -2 Formula C14H23N3O15P2 InChIKeyhelp_outline DPJKHFICSGCNIR-HRENORGGSA-L SMILEShelp_outline C=1N(C(N=C(C1)N)=O)[C@@H]2O[C@@H]([C@H]([C@H]2O)O)COP(OP(OC[C@H]([C@H]([C@H](CO)O)O)O)(=O)[O-])(=O)[O-] 2D coordinates Mol file for the small molecule Search links Involved in 7 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-[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:17480
Reactive part
help_outline
- Name help_outline 3-O-[D-ribitylphospho-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:177331 Charge -3 Formula C31H52N3O27P2 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](CO)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 CMP Identifier CHEBI:60377 Charge -2 Formula C9H12N3O8P InChIKeyhelp_outline IERHLVCPSMICTF-XVFCMESISA-L SMILEShelp_outline Nc1ccn([C@@H]2O[C@H](COP([O-])([O-])=O)[C@@H](O)[C@H]2O)c(=O)n1 2D coordinates Mol file for the small molecule Search links Involved in 193 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:36551 | RHEA:36552 | RHEA:36553 | RHEA:36554 | |
|---|---|---|---|---|
| Reaction direction help_outline | undefined | left-to-right | right-to-left | bidirectional |
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Publications
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Identification of a Post-translational Modification with Ribitol-Phosphate and Its Defect in Muscular Dystrophy.
Kanagawa M., Kobayashi K., Tajiri M., Manya H., Kuga A., Yamaguchi Y., Akasaka-Manya K., Furukawa J.I., Mizuno M., Kawakami H., Shinohara Y., Wada Y., Endo T., Toda T.
Glycosylation is an essential post-translational modification that underlies many biological processes and diseases. α-dystroglycan (α-DG) is a receptor for matrix and synaptic proteins that causes muscular dystrophy and lissencephaly upon its abnormal glycosylation (α-dystroglycanopathies). Here ... >> More
Glycosylation is an essential post-translational modification that underlies many biological processes and diseases. α-dystroglycan (α-DG) is a receptor for matrix and synaptic proteins that causes muscular dystrophy and lissencephaly upon its abnormal glycosylation (α-dystroglycanopathies). Here we identify the glycan unit ribitol 5-phosphate (Rbo5P), a phosphoric ester of pentose alcohol, in α-DG. Rbo5P forms a tandem repeat and functions as a scaffold for the formation of the ligand-binding moiety. We show that enzyme activities of three major α-dystroglycanopathy-causing proteins are involved in the synthesis of tandem Rbo5P. Isoprenoid synthase domain-containing (ISPD) is cytidine diphosphate ribitol (CDP-Rbo) synthase. Fukutin and fukutin-related protein are sequentially acting Rbo5P transferases that use CDP-Rbo. Consequently, Rbo5P glycosylation is defective in α-dystroglycanopathy models. Supplementation of CDP-Rbo to ISPD-deficient cells restored α-DG glycosylation. These findings establish the molecular basis of mammalian Rbo5P glycosylation and provide insight into pathogenesis and therapeutic strategies in α-DG-associated diseases. << Less
Cell Rep. 14:2209-2223(2016) [PubMed] [EuropePMC]
This publication is cited by 1 other entry.