The VHL tumour-suppressor gene paradigm

Trends Genet. 1998 Oct;14(10):423-6. doi: 10.1016/s0168-9525(98)01558-3.


The VHL tumour-suppressor gene (TSG) has a critical 'gatekeeper' role in regulating growth and differentiation of human kidney cells, and inactivation of the VHL gene is the most frequent genetic event in human kidney cancer. There are many similarities between the genetics of the VHL and retinoblastoma TSGs, but the VHL tumourigenesis model is more complex. Here, we examine the current knowledge of the genetics and functional aspects of the VHL TSG, and emphasize how the VHL gene provides a paradigm that illustrates many aspects of TSG biology.

Publication types

  • Research Support, Non-U.S. Gov't
  • Review

MeSH terms

  • Genes, Tumor Suppressor*
  • Humans
  • Ligases*
  • Neoplasms / genetics
  • Neovascularization, Pathologic
  • Proteins / genetics*
  • Tumor Suppressor Proteins*
  • Ubiquitin-Protein Ligases*
  • Von Hippel-Lindau Tumor Suppressor Protein
  • von Hippel-Lindau Disease / genetics*


  • Proteins
  • Tumor Suppressor Proteins
  • Ubiquitin-Protein Ligases
  • Von Hippel-Lindau Tumor Suppressor Protein
  • Ligases
  • VHL protein, human