EWS/FLI1-induced manic fringe renders NIH 3T3 cells tumorigenic

Nat Genet. 1997 Dec;17(4):495-7. doi: 10.1038/ng1297-495.


EWS/FLI1, a fusion gene found in Ewing's sarcoma, encodes a transcriptional regulator and promotes cellular transformation by modulating the transcription of specific target genes. We have found that EWS/FLI1 and structurally related fusion proteins upregulate manic fringe (MFNG), a recently described member of the Fringe gene family instrumental in somatic development. MFNG is also expressed in human tumour-derived cell lines expressing EWS/FLI1. Overexpression of MFNG in NIH 3T3 cells renders them tumorigenic in mice with severe combined immunodeficiency disease (SCID). These data demonstrate that part of the oncogenic effect of EWS/FLI1 is to transcriptionally deregulate a member of a family of morphogenic genes.

Publication types

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

MeSH terms

  • 3T3 Cells
  • Animals
  • Cell Transformation, Neoplastic / genetics*
  • Gene Expression Regulation, Neoplastic
  • Glucosyltransferases
  • Mice
  • Mice, SCID
  • Neoplasm Transplantation
  • Oncogene Proteins, Fusion / genetics
  • Oncogene Proteins, Fusion / physiology*
  • Protein Biosynthesis
  • Proteins / genetics*
  • Proto-Oncogene Protein c-fli-1
  • RNA-Binding Protein EWS
  • Sarcoma, Ewing / etiology
  • Sarcoma, Ewing / genetics*
  • Transcription Factors / genetics
  • Transcription Factors / physiology*
  • Tumor Cells, Cultured


  • EWS-FLI fusion protein
  • Oncogene Proteins, Fusion
  • Proteins
  • Proto-Oncogene Protein c-fli-1
  • RNA-Binding Protein EWS
  • Transcription Factors
  • Glucosyltransferases
  • Mfng protein, mouse