Neurofibromatosis type 1 - a model for nervous system tumour formation?

Nat Rev Cancer. 2005 Jul;5(7):557-64. doi: 10.1038/nrc1653.


Neurofibromatosis type 1 (NF1) is a common genetic condition in which affected individuals develop benign and malignant nervous system tumours. Genetically engineered mouse (GEM) models of these NF1-associated nervous system tumours recapitulate several of the unique clinical aspects of the disease. Moreover, these Nf1 GEM models allow for a direct examination of the earliest stages of tumour evolution, including the contributions that Nf1(+/-) cellular elements and cooperating genetic changes make to facilitate the transition from the pre-neoplastic to the neoplastic state and, in some cases, to promote malignant progression.

Publication types

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

MeSH terms

  • Animals
  • Genes, Neurofibromatosis 1 / physiology
  • Humans
  • Mice
  • Models, Animal
  • Nervous System Neoplasms / genetics*
  • Neurofibromatosis 1 / genetics*