UniprotKB/SwissProt ID: Q9H9S5 (Q9H9S5)
Gene Name:
FKRP
Organism: Homo sapiens (Human)
Function: 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) (PubMed:26923585, PubMed:27194101, PubMed:29477842, PubMed:31949166). 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 (PubMed:25279699, PubMed:26923585, PubMed:27194101, PubMed:29477842, PubMed:31949166)
Other Modifications: View all modification sites in dbPTM
Protein Subcellular Localization: Golgi apparatus membrane. Secreted. Cell membrane, sarcolemma. Rough endoplasmic reticulum. Cytoplasm
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