Wilms tumor suppressor WTX negatively regulates WNT/beta-catenin signaling

Science. 2007 May 18;316(5827):1043-6. doi: 10.1126/science/1141515.

Abstract

Aberrant WNT signal transduction is involved in many diseases. In colorectal cancer and melanoma, mutational disruption of proteins involved in the degradation of beta-catenin, the key effector of the WNT signaling pathway, results in stabilization of beta-catenin and, in turn, activation of transcription. We have used tandem-affinity protein purification and mass spectrometry to define the protein interaction network of the beta-catenin destruction complex. This assay revealed that WTX, a protein encoded by a gene mutated in Wilms tumors, forms a complex with beta-catenin, AXIN1, beta-TrCP2 (beta-transducin repeat-containing protein 2), and APC (adenomatous polyposis coli). Functional analyses in cultured cells, Xenopus, and zebrafish demonstrate that WTX promotes beta-catenin ubiquitination and degradation, which antagonize WNT/beta-catenin signaling. These data provide a possible mechanistic explanation for the tumor suppressor activity of WTX.

Publication types

  • Research Support, N.I.H., Extramural

MeSH terms

  • Adaptor Proteins, Signal Transducing
  • Adenomatous Polyposis Coli Protein / metabolism
  • Animals
  • Axin Protein
  • Cell Line
  • Cell Line, Tumor
  • Cell Nucleus / metabolism
  • Cytoplasm / metabolism
  • Genes, Wilms Tumor
  • Humans
  • Kidney Neoplasms / genetics
  • Protein Binding
  • Protein Interaction Mapping
  • Proteomics
  • RNA Interference
  • Recombinant Fusion Proteins / metabolism
  • Repressor Proteins / metabolism
  • Signal Transduction*
  • Transduction, Genetic
  • Tumor Suppressor Proteins / chemistry
  • Tumor Suppressor Proteins / genetics
  • Tumor Suppressor Proteins / metabolism*
  • Ubiquitin / metabolism
  • Ubiquitin-Protein Ligases / metabolism
  • Wilms Tumor / genetics
  • Wnt Proteins / metabolism*
  • Xenopus Proteins
  • Zebrafish
  • beta Catenin / metabolism*
  • beta-Transducin Repeat-Containing Proteins / metabolism

Substances

  • AMER1 protein, human
  • AXIN1 protein, human
  • Adaptor Proteins, Signal Transducing
  • Adenomatous Polyposis Coli Protein
  • Axin Protein
  • FBXW11 protein, human
  • Recombinant Fusion Proteins
  • Repressor Proteins
  • Tumor Suppressor Proteins
  • Ubiquitin
  • Wnt Proteins
  • Xenopus Proteins
  • axin1 protein, Xenopus
  • beta Catenin
  • beta-Transducin Repeat-Containing Proteins
  • Ubiquitin-Protein Ligases