scholarly journals Functional Interactions in Transcription and Splicing of Ewing’s Sarcoma

ISRN Genetics ◽  
2013 ◽  
Vol 2013 ◽  
pp. 1-6 ◽  
Author(s):  
Roumiana Todorova
Keyword(s):  
Gene Expression ◽  
Experimental Data ◽  
Fusion Proteins ◽  
Ewing’S Sarcoma ◽  
Ewing's Sarcoma ◽  
Dna Binding Proteins ◽  
Chromosomal Translocations ◽  
Functional Interactions ◽  
Binding Partners ◽  
Rna And Dna

Ewing’s sarcoma (EWS) protein is a member of the TET (TLS/EWS/TAF15) family of RNA and DNA-binding proteins with unknown cellular role. EWS protein is encoded by the EWS oncogene on chromosome 22q12, a target of chromosomal translocations in Ewing’s sarcoma tumors. The exact mechanism of EWS participation in gene expression and pathogenesis of the resulting cancers is not defined. The binding partners of native EWS and EWS fusion proteins (EFPs) are described schematically in a model, an attempt to link the transcription with the splicing. The experimental data about the partnerships of EWS and EFPs are summarized, which may lead to better understanding of their function.

scholarly journals Non-coding RNAs in cancers with chromosomal rearrangements: the signatures, causes, functions and implications

2019 ◽  
Vol 11 (10) ◽  
pp. 886-898 ◽  
Author(s):  
Cai Han ◽  
Lin-Yu Sun ◽  
Wen-Tao Wang ◽  
Yu-Meng Sun ◽  
Yue-Qin Chen
Keyword(s):  
Gene Expression ◽  
Signaling Pathways ◽  
Fusion Proteins ◽  
Gene Fusion ◽  
Chromosomal Rearrangements ◽  
Chromosomal Translocation ◽  
Chromosomal Translocations ◽  
Rna Molecules ◽  
Non Coding Rnas ◽  
The Relationship

Abstract Chromosomal translocation leads to the juxtaposition of two otherwise separate DNA loci, which could result in gene fusion. These rearrangements at the DNA level are catastrophic events and often have causal roles in tumorigenesis. The oncogenic DNA messages are transferred to RNA molecules, which are in most cases translated into cancerous fusion proteins. Gene expression programs and signaling pathways are altered in these cytogenetically abnormal contexts. Notably, non-coding RNAs have attracted increasing attention and are believed to be tightly associated with chromosome-rearranged cancers. These RNAs not only function as modulators in downstream pathways but also directly affect chromosomal translocation or the associated products. This review summarizes recent research advances on the relationship between non-coding RNAs and chromosomal translocations and on diverse functions of non-coding RNAs in cancers with chromosomal rearrangements.

scholarly journals Profiling and functional annotation of mRNA gene expression in pediatric rhabdomyosarcoma and Ewing's sarcoma

2004 ◽  
Vol 110 (5) ◽  
pp. 687-694 ◽  
Author(s):  
Claudia Baer ◽  
Mattias Nees ◽  
Stephen Breit ◽  
Barbara Selle ◽  
Andreas E. Kulozik ◽  
...  
Keyword(s):  
Gene Expression ◽  
Functional Annotation ◽  
Ewing’S Sarcoma ◽  
Ewing's Sarcoma ◽  
Mrna Gene ◽  
Mrna Gene Expression

scholarly journals Cooperative DNA Binding with AP-1 Proteins Is Required for Transformation by EWS-Ets Fusion Proteins

2006 ◽  
Vol 26 (7) ◽  
pp. 2467-2478 ◽  
Author(s):  
Sungeun Kim ◽  
Christopher T. Denny ◽  
Ron Wisdom
Keyword(s):  
Dna Binding ◽  
Binding Sites ◽  
Fusion Proteins ◽  
Ewing’S Sarcoma ◽  
Ewing's Sarcoma ◽  
Mutant Form ◽  
Control Of Gene Expression ◽  
3T3 Fibroblasts ◽  
Ets Family

ABSTRACT A key molecular event in the genesis of Ewing's sarcoma is the consistent presence of chromosomal translocations that result in the formation of proteins in which the amino terminus of EWS is fused to the carboxyl terminus, including the DNA binding domain, of one of five different Ets family proteins. These fusion proteins function as deregulated transcription factors, resulting in aberrant control of gene expression. Recent data indicate that some EWS-Ets target promoters, including the uridine phosphorylase (UPP) promoter, harbor tandem binding sites for Ets and AP-1 proteins. Here we show that those Ets family proteins that participate in Ewing's sarcoma, including Fli1, ERG, and ETV1, cooperatively bind these tandem elements with Fos-Jun while other Ets family members do not. Analysis of this cooperativity in vitro shows that (i) many different spatial arrangements of the Ets and AP-1 sites support cooperative binding, (ii) the bZIP motifs of Fos and Jun are sufficient to support this cooperativity, and (iii) both the Ets domain and carboxy-terminal sequences of Fli1 are important for cooperative DNA binding. EWS-Fli1 activates the expression of UPP mRNA, is directly bound to the UPP promoter, and transforms 3T3 fibroblasts; in contrast, a C-terminally truncated mutant form of EWS-Fli1 that cannot cooperatively bind DNA with Fos-Jun is defective in all of these properties. The results show that the ability of EWS-Ets proteins to cooperatively bind DNA with Fos-Jun is critical to the biologic activities of these proteins. The results have implications for understanding the pathogenesis of Ewing's sarcoma. In addition, they may be relevant to the mechanisms of Ras-dependent activation of genes that harbor tandem Ets and AP-1 binding sites.

scholarly journals CCT6A, a Novel Prognostic Biomarker for Ewing's Sarcoma

2020 ◽  
Author(s):  
Jie Jiang ◽  
Chong Liu ◽  
Guoyong Xu ◽  
Tuo Liang ◽  
Chaojie Yu ◽  
...  
Keyword(s):  
Gene Expression ◽  
Overall Survival ◽  
Ewing Sarcoma ◽  
Ewing’S Sarcoma ◽  
Ewing's Sarcoma ◽  
High Expression ◽  
P Value ◽  
Event Free Survival ◽  
Free Survival ◽  
Cox Regression Analysis

Abstract Background: Ewing's sarcoma (ES) is the second most prevalent malignancy among bone tissue tumors, and there is no adequate prognosis biomarker. The protein encoded by CCT6A is a molecular chaperone. Early studies have suggested that CCT6A is involved in the development of many cancers, however, there is no clear evidence of a role for CCT6A in ES.Methods: In this study, we performed a bioinformatics analysis of 32 Ewing sarcoma specimens from the GSE17618 dataset for differences in gene expression and overall survival, event-free survival, and gene expression in different subgroups. Results: After three screenings, we identified CCT6A as highly correlated with Ewing's sarcoma prognosis. Survival analysis showed low overall survival (OS) for CCT6A high expression (P=0.024). On the other hand, Cox regression analysis showed that CCT6A expression, event-free survival (EFS), and age were strongly associated with the prognosis of Ewing sarcoma, identified as independent poor prognostic biomarkers. (CCT6A: P=0.015; Age: P-value=0.026; EFS: P-value=0.001). Conclusion: The expression level of CCT6A is strongly associated with the prognosis of Ewing's sarcoma. High expression of the CCT6A gene may serve as a biomarker for poor prognosis in patients with Ewing's sarcoma.

Abstract 3410: Discovery of prognostic factors in Ewing's sarcoma family of tumors by use of Intercohort co-analysis of public gene expression datasets

2010 ◽  
Author(s):  
Idriss M. Bennani-Baiti ◽  
Elizabeth R. Lawlor ◽  
Aaron Cooper ◽  
Maximilian Kauer ◽  
Jozef Ban ◽  
...  
Keyword(s):  
Gene Expression ◽  
Prognostic Factors ◽  
Ewing’S Sarcoma ◽  
Ewing's Sarcoma

scholarly journals Factors Affecting EWS-FLI1 Activity in Ewing's Sarcoma

Sarcoma ◽  
2011 ◽  
Vol 2011 ◽  
pp. 1-11 ◽  
Author(s):  
David Herrero-Martin ◽  
Argyro Fourtouna ◽  
Stephan Niedan ◽  
Lucia T. Riedmann ◽  
Raphaela Schwentner ◽  
...  
Keyword(s):  
Transcription Factors ◽  
Ewing’S Sarcoma ◽  
Ewing's Sarcoma ◽  
Therapeutic Agents ◽  
Chromosomal Translocations ◽  
Chimeric Proteins ◽  
Fatal Disease ◽  
Factors Affecting ◽  
Factors Influencing ◽  
Novel Therapeutic

Ewing's sarcoma family tumors (ESFT) are characterized by specific chromosomal translocations, which give rise to EWS-ETS chimeric proteins. These aberrant transcription factors are the main pathogenic drivers of ESFT. Elucidation of the factors influencing EWS-ETS expression and/or activity will guide the development of novel therapeutic agents against this fatal disease.

scholarly journals Small-molecule screen identifies modulators of EWS/FLI1 target gene expression and cell survival in Ewing's sarcoma

2012 ◽  
Vol 131 (9) ◽  
pp. 2153-2164 ◽  
Author(s):  
Aleksandar Boro ◽  
Kathya Prêtre ◽  
Florian Rechfeld ◽  
Verena Thalhammer ◽  
Susanne Oesch ◽  
...  
Keyword(s):  
Gene Expression ◽  
Cell Survival ◽  
Small Molecule ◽  
Target Gene ◽  
Ewing’S Sarcoma ◽  
Ewing's Sarcoma ◽  
Target Gene Expression ◽  
Small Molecule Screen

scholarly journals IGF1 Is a Common Target Gene of Ewing's Sarcoma Fusion Proteins in Mesenchymal Progenitor Cells

PLoS ONE ◽  
2008 ◽  
Vol 3 (7) ◽  
pp. e2634 ◽  
Author(s):  
Luisa Cironi ◽  
Nicolò Riggi ◽  
Paolo Provero ◽  
Natalie Wolf ◽  
Mario-Luca Suvà ◽  
...  
Keyword(s):  
Progenitor Cells ◽  
Fusion Proteins ◽  
Target Gene ◽  
Ewing’S Sarcoma ◽  
Ewing's Sarcoma ◽  
Mesenchymal Progenitor Cells ◽  
Common Target
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