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.

Abstract 3408: RNAi screening identifies STK10 as a novel therapeutic target for Ewing's sarcoma

2010 ◽  
Author(s):  
Shilpi Arora ◽  
Irma M. Gonzales ◽  
Chao Sima ◽  
Javed Khan ◽  
Spyro Mousses ◽  
...  
Keyword(s):  
Therapeutic Target ◽  
Ewing’S Sarcoma ◽  
Ewing's Sarcoma ◽  
Rnai Screening ◽  
Novel Therapeutic

scholarly journals Potential Downstream Target Genes of Aberrant ETS Transcription Factors Are Differentially Affected in Ewing’s Sarcoma and Prostate Carcinoma

PLoS ONE ◽  
2012 ◽  
Vol 7 (11) ◽  
pp. e49819 ◽  
Author(s):  
Maria J. Camões ◽  
Paula Paulo ◽  
Franclim R. Ribeiro ◽  
João D. Barros-Silva ◽  
Mafalda Almeida ◽  
...  
Keyword(s):  
Transcription Factors ◽  
Prostate Carcinoma ◽  
Target Genes ◽  
Ewing’S Sarcoma ◽  
Ewing's Sarcoma ◽  
Ets Transcription Factors ◽  
Downstream Target

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.

Abstract 2961: Reactivation of EWS-FLI1 suppressed FOXO1 expression as a novel therapeutic strategy for Ewing's sarcoma

2012 ◽  
Author(s):  
Stephan Niedan ◽  
Max Kauer ◽  
Gunhild Jug ◽  
Robert L. Walker ◽  
Paul S. Meltzer ◽  
...  
Keyword(s):  
Therapeutic Strategy ◽  
Ewing’S Sarcoma ◽  
Ewing's Sarcoma ◽  
Novel Therapeutic

scholarly journals Dual Transforming Activities of the FUS (TLS)-ERG Leukemia Fusion Protein Conferred by Two N-Terminal Domains of FUS (TLS)

1999 ◽  
Vol 19 (11) ◽  
pp. 7639-7650 ◽  
Author(s):  
Hitoshi Ichikawa ◽  
Kimiko Shimizu ◽  
Rieko Katsu ◽  
Misao Ohki
Keyword(s):  
Amino Acids ◽  
Cell Line ◽  
Ewing’S Sarcoma ◽  
Ewing's Sarcoma ◽  
Ectopic Expression ◽  
Mouse Fibroblast ◽  
Chimeric Proteins ◽  
Dependent Manner ◽  
Transforming Activity ◽  
Nih 3T3

ABSTRACT The FUS (TLS)-ERG chimeric protein associated with t(16;21)(p11;q22) acute myeloid leukemia is structurally similar to the Ewing’s sarcoma chimeric transcription factor EWS-ERG. We found that both FUS-ERG and EWS-ERG could induce anchorage-independent proliferation of the mouse fibroblast cell line NIH 3T3. However, only FUS-ERG was able to inhibit the differentiation into neutrophils of a mouse myeloid precursor cell line L-G and induce its granulocyte colony-stimulating factor-dependent growth. We constructed several deletion mutants of FUS-ERG lacking a part of the N-terminal FUS region. A deletion mutant lacking the region between amino acids 1 and 173 (exons 1 to 5) lost the NIH 3T3-transforming activity but retained the L-G-transforming activity. On the other hand, a mutant lacking the region between amino acids 174 and 265 (exons 6 and 7) lost the L-G-transforming activity but retained the NIH 3T3-transforming activity. These results indicate that the N-terminal region of FUS contains two independent functional domains required for the NIH 3T3 and L-G transformation, which we named TR1 and TR2, respectively. Although EWS intrinsically possessed the TR2 domain, the EWS-ERG construct employed lacked the EWS sequence containing this domain. Since the TR2 domain is always found in chimeric proteins identified from t(16;21) leukemia patients but not in chimeric proteins from Ewing’s sarcoma patients, it seems that the TR2 function is required only for the leukemogenic potential. In addition, we identified three cellular genes whose expression was altered by ectopic expression of FUS-ERG and found that these are regulated in either a TR1-dependent or a TR2-dependent manner. These results suggest that FUS-ERG may activate two independent oncogenic pathways during the leukemogenic process by modulating the expression of two different groups of genes simultaneously.

Local therapy and other factors influencing site of relapse in patients with localised Ewing's sarcoma

1999 ◽  
Vol 35 (12) ◽  
pp. 1698-1704 ◽  
Author(s):  
A.G. Shankar ◽  
C.R. Pinkerton ◽  
A. Atra ◽  
S. Ashley ◽  
I. Lewis ◽  
...  
Keyword(s):  
Ewing’S Sarcoma ◽  
Ewing's Sarcoma ◽  
Local Therapy ◽  
Factors Influencing

scholarly journals The dual inhibitory effect of thiostrepton on FoxM1 and EWS/FLI1 provides a novel therapeutic option for Ewing’s sarcoma

2013 ◽  
Vol 43 (3) ◽  
pp. 803-812 ◽  
Author(s):  
ANIRUDDHA SENGUPTA ◽  
MAHBUBUR RAHMAN ◽  
SILVIA MATEO-LOZANO ◽  
OSCAR M. TIRADO ◽  
VICENTE NOTARIO
Keyword(s):  
Ewing’S Sarcoma ◽  
Ewing's Sarcoma ◽  
Therapeutic Option ◽  
Inhibitory Effect ◽  
Novel Therapeutic

Risk analysis factors for local recurrence in Ewing’s sarcoma

2018 ◽  
Vol 100-B (2) ◽  
pp. 247-255 ◽  
Author(s):  
J. I. Albergo ◽  
C. L. L. Gaston ◽  
M. C. Parry ◽  
M. K. Laitinen ◽  
L. M. Jeys ◽  
...  
Keyword(s):  
Prognostic Factors ◽  
Local Recurrence ◽  
Ewing’S Sarcoma ◽  
Ewing's Sarcoma ◽  
Histological Response ◽  
Wide Margin ◽  
Free Survival ◽  
Factors Affecting ◽  
Response To Chemotherapy

Aims The aim of this study was to analyse a group of patients with non-metastatic Ewing’s sarcoma at presentation and identify prognostic factors affecting the development of local recurrence, in order to assess the role of radiotherapy. Patients and Methods A retrospective review of all patients with a Ewing’s sarcoma treated between 1980 and 2012 was carried out. Only those treated with chemotherapy followed by surgery and/or radiotherapy were included. Patients were grouped according to site (central or limb) for further analysis of the prognostic factors. Results A total of 388 patients were included in the study. Of these, 60 (15%) developed local recurrence at a mean median of 27 months (sd 24, range 7 to 150) and the five-year local recurrence-free survival (5yrLRFS) was 83%. For central tumours, the size of the tumour and histological response to chemotherapy were found to be significant factors for local recurrence. For limb tumours, local recurrence was affected by intralesional and marginal resections, but not by the histological response to chemotherapy. Radiotherapy in those with a marginal resection reduced the risk of local recurrence (5yrLRFS: 96% versus 81%, p = 0.044). Conclusion Local recurrence significantly affects the overall survival in patients with a Ewing’s sarcoma. For those with a tumour in a limb, radiotherapy reduced the risk of local recurrence, especially in those with a marginal margin of excision, but the effect in central tumours was less clear. Radiotherapy for those who have had a wide margin of resection does not reduce the risk of local recurrence, regardless of the histological response to chemotherapy. Cite this article: Bone Joint J 2018;100-B: 247–55.

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