The EWS protein is dispensable for Ewing tumor growth.

Abstract	EWS encodes a ubiquitously expressed RNA binding protein with largely unknown function. In Ewing sarcoma family tumors (EFT), one allele is rearranged with an ETS gene. This is the first description of an EFT with a complete EWS deficiency in the presence of two copies of a rearranged chromosome 22 carrying an interstitial EWS-FLI1 translocation. Absence of EWS protein suggested that it is dispensable for EFT growth. By sequencing of EWS cDNA from unrelated EFTs, we excluded inactivation of EWS as a general mechanism in EFT pathogenesis. Rather, EWS was found to be uniformly expressed in two splicing variants of similar abundancy, EWSalpha and EWSbeta, which differ in a single amino acid. Three EWS negative cell lines were established, which will serve as valuable models to study normal and aberrant EWS function upon reintroduction into the tumor cells.
Authors	H Kovar, G Jug, C Hattinger, L Spahn, D N Aryee, P F Ambros, A Zoubek, H Gadner
Journal	Cancer research (Cancer Res) Vol. 61 Issue 16 Pg. 5992-7 (Aug 15 2001) ISSN: 0008-5472 [Print] United States
PMID	11507040 (Publication Type: Journal Article, Research Support, Non-U.S. Gov't)
Chemical References	EWS-FLI fusion protein Heterogeneous-Nuclear Ribonucleoproteins Oncogene Proteins, Fusion Proto-Oncogene Protein c-fli-1 RNA-Binding Protein EWS Ribonucleoproteins Transcription Factors
Topics	Alleles Alternative Splicing Bone Neoplasms (genetics, pathology) Cell Division (genetics, physiology) Child, Preschool Chromosomes, Human, Pair 22 (genetics) Female Gene Expression Gene Expression Regulation, Neoplastic Gene Rearrangement Gene Silencing Heterogeneous-Nuclear Ribonucleoproteins Humans Oncogene Proteins, Fusion (genetics) Proto-Oncogene Protein c-fli-1 RNA-Binding Protein EWS Ribonucleoproteins (genetics, physiology) Sarcoma, Ewing (genetics, pathology) Transcription Factors (genetics) Translocation, Genetic Tumor Cells, Cultured

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