{"database":"biostudies-literature","file_versions":[],"scores":null,"additional":{"omics_type":["Unknown"],"volume":["6(4)"],"submitter":["Komura S"],"funding":["Ministry of Health, Labour and Welfare","Naito Foundation","Takeda Science Foundation","Ministry of Education, Culture, Sports, Science and Technology"],"pubmed_abstract":["EWS-FLI1, a multi-functional fusion oncogene, is exclusively detected in Ewing sarcomas. However, previous studies reported that rare varieties of osteosarcomas also harbor EWS-ETS family fusion. Here, using the doxycycline-inducible EWS-FLI1 system, we established an EWS-FLI1-dependent osteosarcoma model from murine bone marrow stromal cells. We revealed that the withdrawal of EWS-FLI1 expression enhances the osteogenic differentiation of sarcoma cells, leading to mature bone formation. Taking advantage of induced pluripotent stem cell (iPSC) technology, we also show that sarcoma-derived iPSCs with cancer-related genetic abnormalities exhibited an impaired differentiation program of osteogenic lineage irrespective of the EWS-FLI1 expression. Finally, we demonstrate that EWS-FLI1 contribut"],"journal":["Stem cell reports"],"pagination":["592-606"],"full_dataset_link":["https://www.ebi.ac.uk/biostudies/studies/S-EPMC4834047"],"repository":["biostudies-literature"],"pubmed_title":["An EWS-FLI1-Induced Osteosarcoma Model Unveiled a Crucial Role of Impaired Osteogenic Differentiation on Osteosarcoma Development."],"pmcid":["PMC4834047"],"pubmed_authors":["Itakura F","Ohno T","Woltjen K","Akiyama H","Shibata H","Hotta A","Yamamoto T","Yamada Y","Komura S","Semi K"],"additional_accession":[]},"is_claimable":false,"name":"An EWS-FLI1-Induced Osteosarcoma Model Unveiled a Crucial Role of Impaired Osteogenic Differentiation on Osteosarcoma Development.","description":"EWS-FLI1, a multi-functional fusion oncogene, is exclusively detected in Ewing sarcomas. However, previous studies reported that rare varieties of osteosarcomas also harbor EWS-ETS family fusion. Here, using the doxycycline-inducible EWS-FLI1 system, we established an EWS-FLI1-dependent osteosarcoma model from murine bone marrow stromal cells. We revealed that the withdrawal of EWS-FLI1 expression enhances the osteogenic differentiation of sarcoma cells, leading to mature bone formation. Taking advantage of induced pluripotent stem cell (iPSC) technology, we also show that sarcoma-derived iPSCs with cancer-related genetic abnormalities exhibited an impaired differentiation program of osteogenic lineage irrespective of the EWS-FLI1 expression. Finally, we demonstrate that EWS-FLI1 contribut","dates":{"release":"2016-01-01T00:00:00Z","publication":"2016 Apr","modification":"2026-05-05T09:25:40.183Z","creation":"2019-03-27T02:11:43Z"},"accession":"S-EPMC4834047","cross_references":{"pubmed":["26997645"],"doi":["10.1016/j.stemcr.2016.02.009"]}}