Inducible expression of chimeric EWS/ETS proteins confers Ewing's family tumor-like phenotypes to human mesenchymal progenitor cells

Mol Cell Biol. 2008 Apr;28(7):2125-37. doi: 10.1128/MCB.00740-07. Epub 2008 Jan 22.

Abstract

Ewing's family tumor (EFT) is a rare pediatric tumor of unclear origin that occurs in bone and soft tissue. Specific chromosomal translocations found in EFT cause EWS to fuse to a subset of ets transcription factor genes (ETS), generating chimeric EWS/ETS proteins. These proteins are believed to play a crucial role in the onset and progression of EFT. However, the mechanisms responsible for the EWS/ETS-mediated onset remain unclear. Here we report the establishment of a tetracycline-controlled EWS/ETS-inducible system in human bone marrow-derived mesenchymal progenitor cells (MPCs). Ectopic expression of both EWS/FLI1 and EWS/ERG proteins resulted in a dramatic change of morphology, i.e., from a mesenchymal spindle shape to a small round-to-polygonal cell, one of the characteristics of EFT. EWS/ETS also induced immunophenotypic changes in MPCs, including the disappearance of the mesenchyme-positive markers CD10 and CD13 and the up-regulation of the EFT-positive markers CD54, CD99, CD117, and CD271. Furthermore, a prominent shift from the gene expression profile of MPCs to that of EFT was observed in the presence of EWS/ETS. Together with the observation that EWS/ETS enhances the ability of cells to invade Matrigel, these results suggest that EWS/ETS proteins contribute to alterations of cellular features and confer an EFT-like phenotype to human MPCs.

MeSH terms

  • Antigens, CD / biosynthesis
  • Cell Line, Transformed / cytology
  • Cell Line, Transformed / metabolism
  • Cell Movement
  • Cell Shape
  • Collagen
  • Doxycycline / pharmacology
  • Drug Combinations
  • Gene Expression Regulation, Neoplastic
  • Genes, Synthetic
  • Humans
  • Immunophenotyping
  • Laminin
  • Mesenchymal Stem Cells / cytology*
  • Mesenchymal Stem Cells / drug effects
  • Mesenchymal Stem Cells / metabolism
  • Neoplastic Stem Cells / cytology*
  • Neoplastic Stem Cells / metabolism
  • Oncogene Proteins, Fusion / genetics
  • Oncogene Proteins, Fusion / physiology*
  • Proteoglycans
  • Proto-Oncogene Protein c-fli-1 / genetics
  • Proto-Oncogene Protein c-fli-1 / physiology*
  • RNA-Binding Protein EWS
  • Recombinant Fusion Proteins / physiology
  • Sarcoma, Ewing / metabolism
  • Sarcoma, Ewing / pathology*
  • Tetracycline / pharmacology
  • Transcription Factors / genetics
  • Transcription Factors / physiology*

Substances

  • Antigens, CD
  • Drug Combinations
  • EWS-ERG fusion protein, human
  • EWS-FLI fusion protein
  • Laminin
  • Oncogene Proteins, Fusion
  • Proteoglycans
  • Proto-Oncogene Protein c-fli-1
  • RNA-Binding Protein EWS
  • Recombinant Fusion Proteins
  • Transcription Factors
  • matrigel
  • Collagen
  • Tetracycline
  • Doxycycline