Journal article

Transformation induced by Ewing's sarcoma associated EWS/FLI-I is suppressed by KRAB/FLI-I

D Chan, TJ Wilson, D Xu, HE Cowdery, E Sanij, PJ Hertzog, I Kola

British Journal of Cancer | NATURE PUBLISHING GROUP | Published : 2003

Abstract

Ewing's sarcoma is a childhood bone tumour with poor prognosis, most commonly associated with a t(11;22)(q24;q12) reciprocal translocation that fuses the EWS and FLI-1 genes, resulting in the production of an aberrant chimeric transcription factor EWS/FLI-1. To elucidate the mechanisms by which EWS/FLI-1 mediates transformation in mouse models, we have generated a murine Ews/Fli-1 fusion protein. We demonstrate that this protein transforms fibroblast cells in vitro similar to human EWS/FLI-1 as demonstrated by serum and anchorage-independent growth, the formation of tumours in nude mice and elevation of the oncogenic marker c-myc. Furthermore, transformation of these cells was inhibited by a..

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