Notch Signaling in the Mammalian Central Nervous System: Insights From Mouse Mutants

Nat Neurosci. 2005 Jun;8(6):709-15. doi: 10.1038/nn1475.


The Notch pathway, although originally identified in fruit flies, is now among the most heavily studied in mammalian biology. In mice, loss-of-function and gain-of-function work has demonstrated that Notch signaling is essential both during development and in the adult in a multitude of tissues. Prominent among these is the CNS, where Notch has been implicated in processes ranging from neural stem cell regulation to learning and memory. Here we review the role of Notch in the mammalian CNS by focusing specifically on mutations generated in mice. These mutations have provided critical insight into Notch function in the CNS and have led to the identification of promising new directions that are likely to generate important discoveries in the future.

Publication types

  • Research Support, N.I.H., Extramural
  • Research Support, Non-U.S. Gov't
  • Research Support, U.S. Gov't, P.H.S.
  • Review

MeSH terms

  • Animals
  • Cell Differentiation / genetics*
  • Central Nervous System / embryology*
  • Central Nervous System / growth & development
  • Central Nervous System / metabolism
  • Gene Expression Regulation, Developmental / genetics*
  • Humans
  • Mice
  • Mice, Mutant Strains / abnormalities
  • Mice, Mutant Strains / genetics
  • Mice, Mutant Strains / metabolism
  • Mutation / genetics
  • Nervous System Malformations / genetics*
  • Nervous System Malformations / metabolism
  • Nervous System Malformations / physiopathology
  • Receptor, Notch1
  • Receptors, Cell Surface / genetics
  • Receptors, Cell Surface / metabolism*
  • Signal Transduction / genetics
  • Stem Cells / cytology
  • Stem Cells / metabolism
  • Transcription Factors / genetics
  • Transcription Factors / metabolism*


  • NOTCH1 protein, human
  • Notch1 protein, mouse
  • Receptor, Notch1
  • Receptors, Cell Surface
  • Transcription Factors