Wilms' tumours: about tumour suppressor genes, an oncogene and a chameleon gene

Nat Rev Cancer. 2011 Feb;11(2):111-21. doi: 10.1038/nrc3002. Epub 2011 Jan 20.

Abstract

Genes identified as being mutated in Wilms' tumour include TP53, a classic tumour suppressor gene (TSG); CTNNB1 (encoding β-catenin), a classic oncogene; WTX, which accumulating data indicate is a TSG; and WT1, which is inactivated in some Wilms' tumours, similar to a TSG. However, WT1 does not always conform to the TSG label, and some data indicate that WT1 enhances cell survival and proliferation, like an oncogene. Is WT1 a chameleon, functioning as either a TSG or an oncogene, depending on cellular context? Are these labels even appropriate for describing and understanding the function of WT1?

Publication types

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

MeSH terms

  • Adaptor Proteins, Signal Transducing
  • Genes, Wilms Tumor*
  • Humans
  • Kidney Neoplasms / genetics*
  • Kidney Neoplasms / physiopathology
  • Mutation*
  • Oncogenes / genetics*
  • Tumor Suppressor Proteins / genetics
  • Wilms Tumor / genetics*
  • Wilms Tumor / physiopathology

Substances

  • AMER1 protein, human
  • Adaptor Proteins, Signal Transducing
  • Tumor Suppressor Proteins