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Protein O-fucosyltransferase 1 is an essential component of Notch signaling pathways.

Notch receptor signaling regulates cell growth and differentiation, and core components of Notch signaling pathways are conserved from Drosophila to humans. Fringe glycosyltransferases are crucial modulators of Notch signaling that act on epidermal growth factor (EGF)-like repeats in the Notch receptor extracellular domain. The substrate of Fringe is EGF-O-fucose and the transfer of fucose to Notch by protein O-fucosyltransferase 1 is necessary for Fringe to function. O-fucose also occurs on Cripto and on Notch ligands. Here we show that mouse embryos lacking protein O-fucosyltransferase 1 die at midgestation with severe defects in somitogenesis, vasculogenesis, cardiogenesis, and neurogenesis. The phenotype is similar to that of embryos lacking downstream effectors of all Notch signaling pathways such as presenilins or RBP-J kappa, and is different from Cripto, Notch receptor, Notch ligand, or Fringe null phenotypes. Protein O-fucosyltransferase 1 is therefore an essential core member of Notch signaling pathways in mammals.

Pubmed ID: 12697902


  • Shi S
  • Stanley P


Proceedings of the National Academy of Sciences of the United States of America

Publication Data

April 29, 2003

Associated Grants

  • Agency: NCI NIH HHS, Id: CA30645
  • Agency: NCI NIH HHS, Id: CA95022
  • Agency: NCI NIH HHS, Id: P01 CA1333048

Mesh Terms

  • Animals
  • Base Sequence
  • Blood Vessels
  • Blotting, Northern
  • Blotting, Southern
  • Chromosomes, Artificial, Bacterial
  • DNA Primers
  • Fucosyltransferases
  • Gene Targeting
  • In Situ Hybridization
  • Membrane Proteins
  • Mice
  • Receptors, Notch
  • Signal Transduction