ΔNp63 promotes pediatric neuroblastoma and osteosarcoma by regulating tumor angiogenesis

Cancer Res. 2014 Jan 1;74(1):320-9. doi: 10.1158/0008-5472.CAN-13-0894. Epub 2013 Oct 23.

Abstract

The tumor suppressor gene p53 and its family members p63/p73 are critical determinants of tumorigenesis. ΔNp63 is a splice variant of p63, which lacks the N-terminal transactivation domain. It is thought to antagonize p53-, p63-, and p73-dependent translation, thus blocking their tumor suppressor activity. In our studies of the pediatric solid tumors neuroblastoma and osteosarcoma, we find overexpression of ΔNp63; however, there is no correlation of ΔNp63 expression with p53 mutation status. Our data suggest that ΔNp63 itself endows cells with a gain-of-function that leads to malignant transformation, a function independent of any p53 antagonism. Here, we demonstrate that ΔNp63 overexpression, independent of p53, increases secretion of interleukin (IL)-6 and IL-8, leading to elevated phosphorylation of STAT3 (Tyr-705). We show that elevated phosphorylation of STAT3 leads to stabilization of hypoxia-inducible factor 1α (HIF-1α) protein, resulting in VEGF secretion. We also show human clinical data, which suggest a mechanistic role for ΔNp63 in osteosarcoma metastasis. In summary, our studies reveal the mechanism by which ΔNp63, as a master transcription factor, modulates tumor angiogenesis.

Publication types

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

MeSH terms

  • Adolescent
  • Animals
  • Bone Neoplasms / blood supply*
  • Bone Neoplasms / genetics
  • Bone Neoplasms / metabolism
  • Cell Line, Tumor
  • Child
  • Disease Models, Animal
  • Female
  • Heterografts
  • Humans
  • Membrane Proteins / genetics
  • Membrane Proteins / metabolism*
  • Mice
  • Mice, SCID
  • Neovascularization, Pathologic / genetics
  • Neovascularization, Pathologic / metabolism
  • Neuroblastoma / blood supply*
  • Neuroblastoma / genetics
  • Neuroblastoma / metabolism
  • Osteosarcoma / blood supply*
  • Osteosarcoma / genetics
  • Osteosarcoma / metabolism
  • Transcription Factors
  • Tumor Suppressor Protein p53 / genetics
  • Tumor Suppressor Protein p53 / metabolism

Substances

  • CKAP4 protein, human
  • Membrane Proteins
  • TP53 protein, human
  • Transcription Factors
  • Tumor Suppressor Protein p53