Merlin neutralizes the inhibitory effect of Mdm2 on p53

J Biol Chem. 2004 Feb 27;279(9):7812-8. doi: 10.1074/jbc.M305526200. Epub 2003 Dec 16.

Abstract

The stability of p53 tumor suppressor is regulated by Mdm2 via the ubiquitination and proteasome-mediated proteolysis pathway. The c-Abl and PTEN tumor suppressors are known to stabilize p53 by blocking the Mdm2-mediated p53 degradation. This study investigated the correlation between p53 and merlin, a neurofibromatosis 2 (NF2)-related tumor suppressor, in association with the Mdm2 function. The results showed that merlin increased the p53 stability by inhibiting the Mdm2-mediated degradation of p53, which accompanied the increase in the p53-dependent transcriptional activity. The stabilization of p53 by merlin appeared to be accomplished through Mdm2 degradation, and the N-terminal region of merlin was responsible for this novel activity. This study also showed that overexpression of merlin-induced apoptosis of cells depending preferentially on p53 in response to the serum starvation or a chemotherapeutic agent. These results suggest that merlin could be a positive regulator of p53 in terms of tumor suppressor activity, and provide the promising therapeutic means for treating tumors with non-functional merlin or Mdm2 overexpression.

MeSH terms

  • Animals
  • Apoptosis
  • Blotting, Western
  • Cell Division
  • DNA Fragmentation
  • DNA-Binding Proteins
  • Drug Stability
  • Gene Expression
  • Glioma
  • Humans
  • Lung Neoplasms
  • Meningioma
  • Mice
  • NIH 3T3 Cells
  • Neurofibromin 2 / genetics
  • Neurofibromin 2 / pharmacology*
  • Neurofibromin 2 / physiology
  • Nuclear Proteins*
  • Osteosarcoma
  • Proto-Oncogene Proteins / antagonists & inhibitors*
  • Proto-Oncogene Proteins / genetics
  • Proto-Oncogene Proteins / pharmacology*
  • Proto-Oncogene Proteins c-mdm2
  • Recombinant Fusion Proteins
  • Saccharomyces cerevisiae Proteins / genetics
  • Transcription Factors / genetics
  • Transcription, Genetic / drug effects
  • Transfection
  • Tumor Cells, Cultured
  • Tumor Suppressor Protein p53 / genetics
  • Tumor Suppressor Protein p53 / metabolism*

Substances

  • DNA-Binding Proteins
  • GAL4 protein, S cerevisiae
  • Neurofibromin 2
  • Nuclear Proteins
  • Proto-Oncogene Proteins
  • Recombinant Fusion Proteins
  • Saccharomyces cerevisiae Proteins
  • Transcription Factors
  • Tumor Suppressor Protein p53
  • MDM2 protein, human
  • Mdm2 protein, mouse
  • Proto-Oncogene Proteins c-mdm2