title: EWS/FLI1-induced manic fringe renders NIH 3T3 cells tumorigenic.
abstract: 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.
authors.affiliation: Department of Pediatrics, University of Alabama at Birmingham School of Medicine, USA.
authors.forename: W A
authors.initials: WA
authors.lastname: May
authors.forename: A
authors.initials: A
authors.lastname: Arvand
authors.forename: A D
authors.initials: AD
authors.lastname: Thompson
authors.forename: B S
authors.initials: BS
authors.lastname: Braun
authors.forename: M
authors.initials: M
authors.lastname: Wright
authors.forename: C T
authors.initials: CT
authors.lastname: Denny
mesh_terms: D016475:3T3 Cells; D000818:Animals; D002471:Cell Transformation, Neoplastic / Q000235:genetics*; D015972:Gene Expression Regulation, Neoplastic; D005964:Glucosyltransferases; D051379:Mice; D016513:Mice, SCID; D009368:Neoplasm Transplantation; D015514:Oncogene Proteins, Fusion / Q000235:genetics / Q000502:physiology*; D014176:Protein Biosynthesis; D011506:Proteins / Q000235:genetics*; D051789:Proto-Oncogene Protein c-fli-1; D034802:RNA-Binding Protein EWS; D012512:Sarcoma, Ewing / Q000209:etiology / Q000235:genetics*; D014157:Transcription Factors / Q000235:genetics / Q000502:physiology*; D014407:Tumor Cells, Cultured
chemical_list: C106031:EWS-FLI fusion protein; D015514:Oncogene Proteins, Fusion; D011506:Proteins; D051789:Proto-Oncogene Protein c-fli-1; D034802:RNA-Binding Protein EWS; D014157:Transcription Factors; D005964:Glucosyltransferases; C486343:Mfng protein, mouse