Authors: | Lessnick, S. L.; Ladanyi, M. |
Article Title: | Molecular pathogenesis of Ewing sarcoma: New therapeutic and transcriptional targets |
Abstract: | Approximately one-third of sarcomas contain specific translocations. Ewing sarcoma is the prototypical member of this group of sarcomas; it was the first to be recognized pathologically as a singular entity and to have its signature translocation defined cytogenetically, which led to the identification of its key driver alteration, the EWS-FLI1 gene fusion that encodes this aberrant, chimeric transcription factor. We review recent progress in selected areas of Ewing sarcoma research, including the application of genome-wide chromatin immunoprecipitation analyses, to provide a comprehensive view of the EWS-FLI1 target gene repertoire, the identification of EWS-FLI1 target genes that may also point to therapeutically targetable pathways, and data from model systems as they relate to the elusive cell of origin of Ewing sarcoma and its possible similarities to mesenchymal stem cells. Copyright ©2012 by Annual Reviews. All rights reserved. |
Keywords: | signal transduction; unclassified drug; review; nonhuman; cell proliferation; cell survival; gene targeting; gene expression; sonic hedgehog protein; genetic association; transcription factor; rna interference; somatomedin c receptor; ewing sarcoma; chromatin immunoprecipitation; gene identification; gene fusion; transcription; telomerase reverse transcriptase; stem cells; mesenchymal stem cell; dna binding; rna binding protein ews; octamer transcription factor 4; transcription factor sox2; cell strain 3t3; transcription factor nanog; somatomedin binding protein 3; molecular pathology; transcription factor ezh2; receptor upregulation; chromosomal translocation; transcription factor runx2; oncogenesis; ews-fli1; nuclear receptor dax 1; protein fl11; transcription factor nkx2.2 |
Journal Title: | Annual Review of Pathology: Mechanisms of Disease |
Volume: | 7 |
ISSN: | 1553-4006 |
Publisher: | Annual Reviews |
Date Published: | 2012-01-01 |
Start Page: | 145 |
End Page: | 159 |
Language: | English |
DOI: | 10.1146/annurev-pathol-011110-130237 |
PROVIDER: | scopus |
PUBMED: | 21942527 |
PMCID: | PMC3555146 |
DOI/URL: | |
Notes: | --- - "Export Date: 2 April 2012" - "Source: Scopus" |