POFUT1 Catalyzes the reaction that attaches fucose through an O-glycosidic linkage to a conserved serine or threonine residue found in the consensus sequence C2-X(4,5)-[S/T]-C3 of EGF domains, where C2 and C3 are the second and third conserved cysteines. Specifically uses GDP-fucose as donor substrate and proper disulfide pairing of the substrate EGF domains is required for fucose transfer. Plays a crucial role in NOTCH signaling. Initial fucosylation of NOTCH by POFUT1 generates a substrate for FRINGE/RFNG, an acetylglucosaminyltransferase that can then extend the fucosylation on the NOTCH EGF repeats. This extended fucosylation is required for optimal ligand binding and canonical NOTCH signaling induced by DLL1 or JAGGED1. Fucosylates AGRN and determines its ability to cluster acetylcholine receptors (AChRs). Belongs to the glycosyltransferase 65 family. Highly expressed in heart, brain, placenta, lung, liver, skeletal muscle, kidney and pancreas. 2 alternatively spliced human isoforms have been reported. Note: This description may include information from UniProtKB.
Protein type: EC; Transferase
Chromosomal Location of Human Ortholog: 2|2 H1
Cellular Component:  endoplasmic reticulum; membrane
Molecular Function:  fucosyltransferase activity; peptide-O-fucosyltransferase activity; transferase activity; transferase activity, transferring glycosyl groups
Biological Process:  angiogenesis; carbohydrate metabolic process; fucose metabolic process; heart development; nervous system development; Notch signaling pathway; protein O-linked fucosylation; protein O-linked glycosylation; regulation of Notch signaling pathway; somitogenesis
Reference #:  Q91ZW2 (UniProtKB)
Alt. Names/Synonyms: GDP-fucose protein O-fucosyltransferase 1; KIAA0180; mKIAA0180; O-FucT-1; OFUT1; OTTMUSP00000018129; Peptide-O-fucosyltransferase 1; Pofut1; protein O-fucosyltransferase 1
Gene Symbols: Pofut1
Molecular weight: 44,688 Da
Basal Isoelectric point: 8.67  Predict pI for various phosphorylation states
CST Pathways:  Notch Signaling
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Protein Structure Not Found.

Cross-references to other databases:  STRING  |  Reactome  |  BioGPS  |  Pfam  |  RCSB PDB  |  ENZYME  |  Phospho.ELM  |  NetworKIN  |  UniProtKB  |  Entrez-Gene  |  Ensembl Gene