Regulation of p53 tetramerization and nuclear export by ARC

Proc Natl Acad Sci U S A. 2007 Dec 26;104(52):20826-31. doi: 10.1073/pnas.0710017104. Epub 2007 Dec 17.

Abstract

Inactivation of the transcription factor p53 is central to carcinogenesis. Yet only approximately one-half of cancers have p53 loss-of-function mutations. Here, we demonstrate a mechanism for p53 inactivation by apoptosis repressor with caspase recruitment domain (ARC), a protein induced in multiple cancer cells. The direct binding in the nucleus of ARC to the p53 tetramerization domain inhibits p53 tetramerization. This exposes a nuclear export signal in p53, triggering Crm1-dependent relocation of p53 to the cytoplasm. Knockdown of endogenous ARC in breast cancer cells results in spontaneous tetramerization of endogenous p53, accumulation of p53 in the nucleus, and activation of endogenous p53 target genes. In primary human breast cancers with nuclear ARC, p53 is almost always WT. Conversely, nearly all breast cancers with mutant p53 lack nuclear ARC. We conclude that nuclear ARC is induced in cancer cells and negatively regulates p53.

Publication types

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

MeSH terms

  • Active Transport, Cell Nucleus
  • Breast Neoplasms / embryology*
  • Caspases / metabolism*
  • Cell Line, Tumor
  • Cytoskeletal Proteins / metabolism
  • Cytoskeletal Proteins / physiology*
  • Dimerization
  • Exportin 1 Protein
  • Gene Expression Regulation, Neoplastic*
  • Humans
  • Karyopherins / metabolism
  • Models, Biological
  • Mutation
  • Nerve Tissue Proteins / metabolism
  • Nerve Tissue Proteins / physiology*
  • Protein Structure, Tertiary
  • RNA, Small Interfering / metabolism
  • Receptors, Cytoplasmic and Nuclear / metabolism
  • Transcription, Genetic
  • Tumor Suppressor Protein p53 / chemistry
  • Tumor Suppressor Protein p53 / physiology*

Substances

  • Cytoskeletal Proteins
  • Karyopherins
  • Nerve Tissue Proteins
  • RNA, Small Interfering
  • Receptors, Cytoplasmic and Nuclear
  • TP53 protein, human
  • Tumor Suppressor Protein p53
  • activity regulated cytoskeletal-associated protein
  • Caspases