GLI1 Is a Central Mediator of EWS/FLI1 Signaling in Ewing Tumors
GLI1 Is a Central Mediator of EWS/FLI1 Signaling in Ewing Tumors
The Ewing Sarcoma Family Tumors (ESFT) consist of the classical pathologic entities of Ewing Sarcoma and peripheral Primitive Neuroectodermal Tumor. Occurring largely in the childhood through young adult years, these tumors have an unsurpassed propensity for metastasis and have no defined cell of origin. The biology of these aggressive malignancies centers around EWS/FLI1 and related EWS/ETS chimeric transcription factors, which are largely limited to this tumor class. Much progress has been made in the identification of a network of loci whose expression is modulated by EWS/FLI1 and its congeners. To date, little progress has been made in reconstructing the sequence of direct and indirect events that produce this network of modulated loci. The recent identification of GLI1 as an upregulated target of EWS/ETS transcription factors suggests a target which may be a more central mediator in the ESFT signaling network. In this paper, we further define the relationship of EWS/FLI1 expression and GLI1 upregulation in ESFT. This relationship is supported with data from primary tumor specimens. It is consistently observed across multiple ESFT cell lines and with multiple means of EWS/FLI1 inhibition. GLI1 inhibition affects tumor cell line phenotype whether shRNA or endogenous or pharmacologic inhibitors are employed. As is seen in model transformation systems, GLI1 upregulation by EWS/FLI1 appears to be independent of Hedgehog stimulation. Consistent with a more central role in ESFT pathogenesis, several known EWS/FLI1 targets appear to be targeted through GLI1. These findings further establish a central role for GLI1 in the pathogenesis of Ewing Tumors.
- University of California System United States
- University of Southern California United States
- Children's Hospital of Los Angeles United States
Oncogene Proteins, Fusion, Transcription, Genetic, Proto-Oncogene Protein c-fli-1, Science, Q, R, Sarcoma, Ewing, Models, Biological, Polymerase Chain Reaction, Zinc Finger Protein GLI1, Gene Expression Regulation, Neoplastic, Phenotype, Retroviridae, Cell Line, Tumor, Medicine, Humans, RNA Interference, RNA-Binding Protein EWS, Research Article, Signal Transduction, Transcription Factors
Oncogene Proteins, Fusion, Transcription, Genetic, Proto-Oncogene Protein c-fli-1, Science, Q, R, Sarcoma, Ewing, Models, Biological, Polymerase Chain Reaction, Zinc Finger Protein GLI1, Gene Expression Regulation, Neoplastic, Phenotype, Retroviridae, Cell Line, Tumor, Medicine, Humans, RNA Interference, RNA-Binding Protein EWS, Research Article, Signal Transduction, Transcription Factors
14 Research products, page 1 of 2
- 2017IsRelatedTo
- 2017IsRelatedTo
- 2017IsRelatedTo
- 2017IsRelatedTo
- 2010IsAmongTopNSimilarDocuments
- 2017IsRelatedTo
- 2010IsAmongTopNSimilarDocuments
- 2017IsRelatedTo
chevron_left - 1
- 2
chevron_right
citations This is an alternative to the "Influence" indicator, which also reflects the overall/total impact of an article in the research community at large, based on the underlying citation network (diachronically).72 popularity This indicator reflects the "current" impact/attention (the "hype") of an article in the research community at large, based on the underlying citation network.Top 10% influence This indicator reflects the overall/total impact of an article in the research community at large, based on the underlying citation network (diachronically).Top 10% impulse This indicator reflects the initial momentum of an article directly after its publication, based on the underlying citation network.Top 10%
