Human Gene Set: STAEGE_EWING_FAMILY_TUMOR


Standard name STAEGE_EWING_FAMILY_TUMOR
Systematic name M10582
Brief description Genes up-regulated in Ewing family tumors (EFT) compared with normal bone marrow samples.
Full description or abstract Ewing family tumors (EFTs) are small round blue cell tumors that show features of neuroectodermal differentiation. However, the histogenetic origin of EFTs is still a matter of debate. We used high-density DNA microarrays for the identification of EFT-specific gene expression profiles in comparison with normal tissues of diverse origin. We identified 37 genes that are up-regulated in EFTs compared with normal tissues and validated expression of these genes in EFTs by both conventional and quantitative reverse transcription-polymerase chain reaction. The expression pattern of EFT-associated genes in normal tissues indicated a high similarity between EFTs and fetal and neuronal as well as endothelial tissues and supports the concept that a primitive neural crest-derived progenitor at the transition to mesenchymal and endothelial differentiation is transformed in EFTs. EFT-associated genes could be used for molecular discrimination between EFTs and other small round blue cell tumors and clearly identified a cell line (SK-N-MC) that was initially established as neuroblastoma as being an EFT. Ectopic expression of the EFT-specific EWS-FLI1 fusion protein in human embryonic kidney (HEK293) cells was not sufficient to induce the complete EFT-specific gene expression signature, suggesting that the EFT-specific gene expression profile is not just a consequence of EWS-FLI1 expression but depends on the histogenetic background of the EFT stem cell.
Collection C2: Curated
      CGP: Chemical and Genetic Perturbations
Source publication Pubmed 15548687   Authors: Staege MS,Hutter C,Neumann I,Foja S,Hattenhorst UE,Hansen G,Afar D,Burdach SE
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Source species Homo sapiens
Contributed by Kevin Vogelsang (MSigDB Team)
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identifier namespace		 HUMAN_SEQ_ACCESSION
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