Common musculoskeletal tumors of childhood and adolescence. The New England journal of medicine, vol.341, pp.342-352, 1999. ,
Ewing sarcoma: clinical state-of-the-art. Pediatric hematology and oncology, vol.29, pp.1-11, 2012. ,
Ewing sarcoma/peripheral primitive neuroectodermal tumor and related tumors. Pediatric and developmental pathology, vol.15, pp.108-126, 2012. ,
Gene fusion with an ETS DNA-binding domain caused by chromosome translocation in human tumours, Nature, vol.359, pp.162-165, 1992. ,
The Ewing's sarcoma EWS/FLI-1 fusion gene encodes a more potent transcriptional activator and is a more powerful transforming gene than FLI-1, Molecular and Cellular Biology, vol.13, pp.7393-7398, 1993. ,
EWS-Fli1 antisense oligodeoxynucleotide inhibits proliferation of human Ewing's sarcoma and primitive neuroectodermal tumor cells, The Journal of clinical investigation, vol.99, pp.239-247, 1997. ,
Inhibition of EWS-FLI-1 fusion protein with antisense oligodeoxynucleotides, J Neurooncol, vol.31, pp.9-16, 1997. ,
EWS/FLI-1 antagonists induce growth inhibition of Ewing tumor cells in vitro, Cell Growth Differ, vol.7, pp.429-437, 1996. ,
Therapeutic potentialities of EWS-Fli-1 mRNA-targeted vectorized antisense oligonucleotides, Ann N Y Acad Sci, vol.1002, pp.72-77, 2003. ,
Loss of tumorigenicity of Ewing's sarcoma cells expressing antisense RNA to EWS-fusion transcripts, Oncogene, vol.11, pp.1049-1054, 1995. ,
Targeting of EWS/FLI-1 by RNA interference attenuates the tumor phenotype of Ewing's sarcoma cells in vitro, J Orthop Res, vol.22, pp.910-917, 2004. ,
Small interfering RNAs expressed from a Pol III promoter suppress the EWS/Fli-1 transcript in an Ewing sarcoma cell line, Mol Ther, vol.7, pp.811-816, 2003. ,
Potentials for RNAi in sarcoma research and therapy: Ewing's sarcoma as a model, Semin Cancer Biol, vol.13, pp.275-281, 2003. ,
Loss of p16 pathways stabilizes EWS/FLI1 expression and complements EWS/FLI1 mediated transformation, Oncogene, vol.20, pp.6731-6741, 2001. ,
The Ewing's sarcoma oncoprotein EWS/FLI induces a p53-dependent www.impactjournals.com/oncotarget growth arrest in primary human fibroblasts, Cancer cell, vol.1, pp.393-401, 2002. ,
EWS/FLI1 oncogene activates caspase 3 transcription and triggers apoptosis in vivo, Cancer research, vol.70, pp.1154-1163, 2010. ,
Development of Ewing's sarcoma from primary bone marrow-derived mesenchymal progenitor cells, Cancer research, vol.65, pp.11459-11468, 2005. ,
EWS-FLI-1 expression triggers a Ewing's sarcoma initiation program in primary human mesenchymal stem cells, Cancer research, vol.68, pp.2176-2185, 2008. ,
Expression of the EWS/FLI-1 oncogene in murine primary bone-derived cells Results in EWS/FLI-1-dependent, ewing sarcoma-like tumors, Cancer research, vol.65, pp.8698-8705, 2005. ,
Modeling initiation of Ewing sarcoma in human neural crest cells, Plos One, vol.6, p.19305, 2011. ,
Ewing's sarcoma precursors are highly enriched in embryonic osteochondrogenic progenitors, The Journal of clinical investigation, vol.124, pp.3061-3074, 2014. ,
Molecular and cellular biology of rhabdomyosarcoma, Future Oncol, vol.5, pp.1449-1475, 2009. ,
Synovial sarcoma: from genetics to genetic-based animal modeling, Clinical orthopaedics and related research, vol.466, pp.2156-2167, 2008. ,
Alveolar rhabdomyosarcomas in conditional Pax3:Fkhr mice: cooperativity of Ink4a/ARF and Trp53 loss of function, Genes Dev, vol.18, pp.2614-2626, 2004. ,
PPARgamma agonists enhance ET-743-induced adipogenic differentiation in a transgenic mouse model of myxoid round cell liposarcoma, The Journal of clinical investigation, vol.122, pp.886-898, 2012. ,
EWS/ ATF1 expression induces sarcomas from neural crestderived cells in mice, The Journal of clinical investigation, vol.123, pp.600-610, 2013. ,
A conditional mouse model of synovial sarcoma: insights into a myogenic origin, Cancer cell, vol.11, pp.375-388, 2007. ,
EWS-FLI1 induces developmental abnormalities and accelerates sarcoma formation in a transgenic mouse model, Cancer research, vol.68, pp.8968-8975, 2008. ,
EWS/ FLI-1 induces rapid onset of myeloid/erythroid leukemia in mice, Molecular and cellular biology, vol.27, pp.7918-7934, 2007. ,
Somatic chromosomal translocation between Ewsr1 and Fli1 loci leads to dilated cardiomyopathy in a mouse model, Sci Rep, vol.5, p.7826, 2015. ,
A small molecule blocking oncogenic protein EWS-FLI1 interaction with RNA helicase A inhibits growth of Ewing's sarcoma, Nature medicine, vol.15, pp.750-756, 2009. ,
Arsenic trioxide inhibits human cancer cell growth and tumor development in mice by blocking Hedgehog/GLI pathway, The Journal of clinical investigation, vol.121, pp.148-160, 2011. ,
A zebrafish transgenic model of Ewing's sarcoma reveals conserved mediators of EWS-FLI1 tumorigenesis, Disease models & mechanisms, vol.5, pp.95-106, 2012. ,
Expression of Runx2 transcription factor in non-skeletal tissues, sperm and brain, J Cell Physiol, vol.217, pp.511-517, 2008. ,
Glucocorticoids suppress bone formation by attenuating osteoblast differentiation via the monomeric glucocorticoid receptor, Cell Metab, vol.11, pp.517-531, 2010. ,
Genomic landscape of Ewing sarcoma defines an aggressive subtype with co-association of STAG2 and TP53 mutations, Cancer discovery, vol.4, pp.1342-1353, 2014. ,
Role of the INK4a locus in tumor suppression and cell mortality, Cell, vol.85, pp.27-37, 1996. ,
The novel zinc finger-containing transcription factor osterix is required for osteoblast differentiation and bone formation, Cell, vol.108, pp.17-29, 2002. ,
Distinct roles for Hedgehog and canonical Wnt signaling in specification, differentiation and maintenance of osteoblast progenitors, Development, vol.133, pp.3231-3244, 2006. ,
Conditional mouse osteosarcoma, dependent on p53 loss and potentiated by loss of Rb, mimics the human disease, Genes Dev, vol.22, pp.1662-1676, 2008. ,
Osx-Cre targets multiple cell types besides osteoblast lineage in postnatal mice, Plos One, vol.9, p.85161, 2014. ,
,
, In Vivo Imaging Reveals Extracellular Vesicle-Mediated Phenocopying of Metastatic Behavior. Cell, vol.161, pp.1046-1057, 2015.
Expression and activity of osteoblast-targeted Cre recombinase transgenes in murine skeletal tissues, Int J Dev Biol, vol.48, pp.645-653, 2004. ,
Ewing's sarcoma family of tumors: Ewing's sarcoma of bone and soft tissue and the peripheral primitive neuroectodermal tumors, Lippincott), 1993. ,
Intragenic regulatory elements contribute to transcriptional control of the neurofilament light gene, Gene, vol.116, pp.205-214, 1992. ,
Site-specific integration of DNA into wild-type and mutant lox sites placed in the plant genome, Plant J, vol.7, pp.649-659, 1995. ,
Expression of transgenes targeted to the Gt(ROSA)26Sor locus is orientation dependent, PLoS One, vol.1, p.4, 2006. ,
Generalized lacZ expression with the ROSA26 Cre reporter strain, Nat Genet, vol.21, pp.70-71, 1999. ,
Bidirectional transcriptional activity of PGK-neomycin and unexpected embryonic lethality in heterozygote chimeric knockout mice, Genesis, vol.30, pp.259-263, 2001. ,
Expression of Cre Recombinase in the developing mouse limb bud driven by a Prxl enhancer, Genesis, vol.33, pp.77-80, 2002. ,
A cre-transgenic mouse strain for the ubiquitous deletion of loxP-flanked gene segments including deletion in germ cells, Nucleic Acids Res, vol.23, pp.5080-5081, 1995. ,
Conditional inactivation of FGF receptor 2 reveals an essential role for FGF signaling in the regulation of osteoblast function and bone growth, Development, vol.130, pp.3063-3074, 2003. ,
Cre recombinase-mediated gene targeting of mesenchymal cells, Genesis, vol.38, pp.139-144, 2004. ,
Osteo-chondroprogenitor cells are derived from Sox9 expressing precursors, P Natl Acad Sci USA, vol.102, pp.14665-14670, 2005. ,
A novel transgenic technique that allows specific marking of the neural crest cell lineage in mice, Dev Biol, vol.212, pp.191-203, 1999. ,
Isolation and characterization of bone marrow-derived mesenchymal progenitor cells with myogenic and neuronal properties, Exp Cell Res, vol.313, pp.1008-1023, 2007. ,
Adult stem cells from bone marrow (MSCs) isolated from different strains of inbred mice vary in surface epitopes, rates of proliferation, and differentiation potential, Blood, vol.103, pp.1662-1668, 2004. ,
Generation of an inducible and optimized piggyBac transposon system, Nucleic Acids Res, vol.35, p.87, 2007. ,
Cooperation of oncogenic K-ras and p53 deficiency in pleomorphic rhabdomyosarcoma development in adult mice, Oncogene, vol.25, pp.7673-7679, 2006. ,
Mouse model for lung tumorigenesis through Cre/ lox controlled sporadic activation of the K-Ras oncogene, Oncogene, vol.20, pp.6551-6558, 2001. ,
Ductal origin of pancreatic adenocarcinomas induced by conditional activation of a human Ha-ras oncogene in rat pancreas, Carcinogenesis, vol.27, pp.2497-2510, 2006. ,
Development of a mouse model www.impactjournals.com/oncotarget for sporadic and metastatic colon tumors and its use in assessing drug treatment, P Natl Acad Sci USA, vol.107, pp.1565-1570, 2010. ,
EWS/FLI function varies in different cellular backgrounds, Experimental cell research, vol.290, pp.414-419, 2003. ,
The EWS/FLI1 oncogenic protein inhibits expression of the Wnt inhibitor DICKKOPF-1 gene and antagonizes beta-catenin/TCF-mediated transcription, Carcinogenesis, vol.31, pp.394-401, 2010. ,
Wnt/ beta-catenin is essential for intestinal homeostasis and maintenance of intestinal stem cells, Mol Cell Biol, vol.27, pp.7551-7559, 2007. ,
Fusion of Chop to a Novel Rna-Binding Protein in Human Myxoid Liposarcoma, Nature, vol.363, pp.640-644, 1993. ,
Further characterization of the human clear cell sarcoma cell line HS-MM demonstrating a specific t(12;22)(q13;q12) translocation and hybrid EWS/ ATF-1 transcript, J Pathol, vol.187, pp.594-597, 1999. ,
The histopathology of a human mesenchymal stem cell experimental tumor model: support for an hMSC origin for Ewing's sarcoma?, Histology and histopathology, vol.23, pp.1229-1240, 2008. ,
Mesenchymal Stem Cells and the Origin of Ewing's Sarcoma, Sarcoma, 2011. ,
Genetically defined EWS/FLI1 model system suggests mesenchymal origin of Ewing's family tumors, Laboratory investigation, vol.88, pp.1291-1302, 2008. ,
Mesenchymal stem cell features of Ewing tumors, Cancer cell, vol.11, pp.421-429, 2007. ,
URL : https://hal.archives-ouvertes.fr/inserm-02438637
The Ewing's sarcoma fusion protein, EWS-FLI, binds Runx2 and blocks osteoblast differentiation, J Cell Biochem, vol.111, pp.933-943, 2010. ,
EWS-FLI1 utilizes divergent chromatin remodeling mechanisms to directly activate or repress enhancer elements in Ewing sarcoma, Cancer cell, vol.26, pp.668-681, 2014. ,
Epigenome mapping reveals distinct modes of gene regulation and widespread enhancer reprogramming by the oncogenic fusion protein EWS-FLI1, Cell Rep, vol.10, pp.1082-1095, 2015. ,
Blocking the road, stopping the engine or killing the driver? Advances in targeting EWS/FLI-1 fusion in Ewing sarcoma as novel therapy, Expert Opin Ther Targets, vol.18, pp.1315-1328, 2014. ,
Alternative splicing in cancer: implications for biology and therapy, Oncogene, vol.34, pp.1-14, 2015. ,
Oncogenic fusion protein EWS-FLI1 is a network hub that regulates alternative splicing, P Natl Acad Sci USA, vol.112, pp.1307-1316, 2015. ,
EWS/FLI alters 5 '-splice site selection, Journal of Biological Chemistry, vol.276, pp.22317-22322, 2001. ,
EWS center dot Fli-1 fusion protein interacts with hyperphosphorylated RNA polymerase II and interferes with serine-arginine protein-mediated RNA splicing, Journal of Biological Chemistry, vol.275, pp.37612-37618, 2000. ,
Oncogenic TLS/ERG and EWS/Fli-1 fusion proteins inhibit RNA splicing mediated by YB-1 protein, Cancer Res, vol.61, pp.3586-3590, 2001. ,
The EWS protein is dispensable for Ewing tumor growth, Cancer Res, vol.61, pp.5992-5997, 2001. ,
Microsatellites as EWIS/ FLI response elements in Ewing's sarcoma, P Natl Acad Sci USA, vol.105, pp.10149-10154, 2008. ,
GSTM4 is a microsatellite-containing EWS/ FLI target involved in Ewing's sarcoma oncogenesis and therapeutic resistance, Oncogene, vol.28, pp.4126-4132, 2009. ,
Emergent Properties of EWS/FLI Regulation via GGAA Microsatellites in Ewing's Sarcoma, Genes Cancer, vol.1, pp.177-187, 2010. ,
Microsatellites with Macro-Influence in Ewing Sarcoma, Genes-Basel, vol.3, pp.444-460, 2012. ,
EWS/FLI-responsive GGAA microsatellites exhibit polymorphic differences between European and African populations, Cancer Genet-Ny, vol.205, pp.304-312, 2012. ,
Clinical and Biochemical Function of Polymorphic NR0B1 www.impactjournals.com/oncotarget GGAA-Microsatellites in Ewing Sarcoma: A Report from the Children's Oncology Group, Plos One, vol.9, p.104378, 2014. ,
Chimeric EWSR1-FLI1 regulates the Ewing sarcoma susceptibility gene EGR2 via a GGAA microsatellite, Nat Genet, vol.47, pp.1073-1078, 2015. ,
MIC2 is a specific marker for Ewing's sarcoma and peripheral primitive neuroectodermal tumors. Evidence for a common histogenesis of Ewing's sarcoma and peripheral primitive neuroectodermal tumors from MIC2 expression and specific chromosome aberration, Cancer, vol.67, pp.1886-1893, 1991. ,
Overexpression of the pseudoautosomal gene MIC2 in Ewing's sarcoma and peripheral primitive neuroectodermal tumor, Oncogene, vol.5, pp.1067-1070, 1990. ,
Immunohistochemical analysis of Ewing's sarcoma cell surface antigen p30/32MIC2. The American journal of pathology, vol.139, pp.317-325, 1991. ,
Ewing's sarcoma-routine diagnostic utilization of MIC2 analysis: a Pediatric Oncology Group/Children's Cancer Group Intergroup Study, Human pathology, vol.25, pp.304-307, 1994. ,
Suppression of KCMF1 by constitutive high CD99 expression is involved in the migratory ability of Ewing's sarcoma cells, Oncogene, vol.25, pp.2795-2800, 2006. ,
CD99 engagement: an effective therapeutic strategy for Ewing tumors, Cancer research, vol.60, pp.5134-5142, 2000. ,
Engagement of CD99 induces apoptosis through a calcineurin-independent pathway in Ewing's sarcoma cells. The American journal of pathology, vol.153, pp.1937-1945, 1998. ,
CD99 inhibits neural differentiation of human Ewing sarcoma cells and thereby contributes to oncogenesis, The Journal of clinical investigation, vol.120, pp.668-680, 2010. ,
Targeting CD99 in association with doxorubicin: an effective combined treatment for Ewing's sarcoma, European journal of cancer, vol.42, pp.91-96, 2006. ,
Mouse CD99 participates in T-cell recruitment into inflamed skin, Blood, vol.104, pp.3205-3213, 2004. ,
Cloning, genomic organization, alternative transcripts and expression analysis of CD99L2, a novel paralog of human CD99, and identification of evolutionary conserved motifs, Gene, vol.307, pp.63-76, 2003. ,
The role of FLI-1-EWS, a fusion gene reciprocal to EWS-FLI-1, in Ewing sarcoma, Genes & cancer, vol.6, pp.452-461, 2015. ,
Control of RUNX2 isoform expression: the role of promoters and enhancers, J Cell Biochem, vol.95, pp.506-517, 2005. ,
Production and characterization of mouse monoclonal antibodies to wild-type and oncogenic FLI-1 proteins, Hybridoma, vol.16, pp.457-464, 1997. ,
Activated Kras and Ink4a/Arf deficiency cooperate to produce metastatic pancreatic ductal adenocarcinoma, Genes & development, vol.17, pp.3112-3126, 2003. ,
Endochondral ossification is required for haematopoietic stem-cell niche formation, Nature, vol.457, pp.490-494, 2009. ,
Hematopoietic stem cell quiescence is maintained by compound contributions of the retinoblastoma gene family, Cell Stem Cell, vol.3, pp.416-428, 2008. ,
Transgenic mice expressing beta-galactosidase in mature neurons under neuron-specific enolase promoter control, Neuron, vol.5, pp.187-197, 1990. ,