Ewing's sarcoma: general insights from a rare model

Cancer Cell. 2006 May;9(5):331-2. doi: 10.1016/j.ccr.2006.05.003.

Abstract

Ewing's sarcoma is characterized by the presence of fusion oncoproteins involving EWSR1 and an ETS gene, most commonly FLI1. In this issue of Cancer Cell, Smith et al. have combined RNA interference with expression profiling to study the pattern of gene expression downstream of the most common of these fusions, EWS/FLI. Using this strategy, Smith et al. have identified a homeobox gene, NKX2.2, which is both highly expressed in Ewing's sarcoma and essential for the transforming activity of EWS/FLI.

Publication types

  • Research Support, Non-U.S. Gov't
  • Comment

MeSH terms

  • Animals
  • Cell Transformation, Neoplastic
  • Gene Expression Regulation, Neoplastic
  • Homeobox Protein Nkx-2.2
  • Homeodomain Proteins / genetics
  • Homeodomain Proteins / metabolism
  • Mice
  • Models, Biological
  • Nuclear Proteins
  • Oncogene Proteins, Fusion / metabolism*
  • Proto-Oncogene Protein c-fli-1 / metabolism*
  • RNA-Binding Protein EWS
  • Sarcoma, Ewing / genetics*
  • Sarcoma, Ewing / pathology
  • Transcription Factors / genetics
  • Transcription Factors / metabolism
  • Zebrafish Proteins

Substances

  • EWS-FLI fusion protein
  • Homeobox Protein Nkx-2.2
  • Homeodomain Proteins
  • NKX2-2 protein, human
  • Nkx2-2 protein, mouse
  • Nuclear Proteins
  • Oncogene Proteins, Fusion
  • Proto-Oncogene Protein c-fli-1
  • RNA-Binding Protein EWS
  • Transcription Factors
  • Zebrafish Proteins
  • nkx2.2b protein, zebrafish