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Proteins/UNIPROT:Q9H9S5
Catalyzes the transfer of a ribitol 5-phosphate from CDP-L-ribitol to the ribitol 5-phosphate previously attached by FKTN/fukutin to the phosphorylated O-mannosyl trisaccharide (N-acetylgalactosamine-beta-3-N-acetylglucosamine-beta-4-(phosphate-6-)mannose), a carbohydrate structure present in alpha-dystroglycan (DAG1). This constitutes the second step in the formation of the ribose 5-phosphate tandem repeat which links the phosphorylated O-mannosyl trisaccharide to the ligand binding moiety composed of repeats of 3-xylosyl-alpha-1,3-glucuronic acid-beta-1..
Function from UniProtKB (Q9H9S5), read 2026-09-23. CC BY 4.0.
Curated compounds routed: 0
9 rows
13 statements
13 statements · 4 reference databases · Subject: FKRP · Sources: Hetionet, Reactome, Rhea, UniProt
1 reaction
3-O-[Rib-ol-P-3-beta-D-GalNAc-(1->3)-beta-D-GlcNAc-(1->4)-(O-6-P-alpha-D-Man)]-Thr-[protein]+CDP-L-ribitol3-O-[Rib-ol-P-Rib-ol-P-3-beta-D-GalNAc-(1->3)-beta-D-GlcNAc-(1->4)-(O-6-P-alpha-D-Man)]-Thr-[protein]+CMP+hydron
Catalysed by FKRP
Reaction pageLeft to rightBalance undeterminable
Rhea RHEA:39867CC BY 4.0Reference data
2 names
Resolves to: FKRP