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Targeted therapy for EWS-FLI1 in ewing sarcoma.
Cancers 15:21 (2023)
Ewing sarcoma (EwS) is a rare and predominantly pediatric malignancy of bone and soft tissue in children and adolescents. Although international collaborations have greatly improved the prognosis of most EwS, the occurrence of macrometastases or relapse remains challenging. The prototypic oncogene EWS-FLI1 acts as an aberrant transcription factor that drives the cellular transformation of EwS. In addition to its involvement in RNA splicing and the DNA damage response, this chimeric protein directly binds to GGAA repeats, thereby modifying the transcriptional profile of EwS. Direct pharmacological targeting of EWS-FLI1 is difficult because of its intrinsically disordered structure. However, targeting the EWS-FLI1 protein complex or downstream pathways provides additional therapeutic options. This review describes the EWS-FLI1 protein partners and downstream pathways, as well as the related target therapies for the treatment of EwS.
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Publication type
Article: Journal article
Document type
Review
Keywords
Ews-fli1 ; Ewing Sarcoma ; Immunotherapy ; Protein Complex ; Targeted Therapy; 6-transmembrane Epithelial Antigen; Direct Transcriptional Target; Fusion Protein; Tumor-growth; In-vitro; T-cells; Histone Deacetylase; Ewsr1-fli1 Activity; Genomic Landscape; Binding Protein
ISSN (print) / ISBN
2072-6694
Journal
Cancers
Quellenangaben
Volume: 15,
Issue: 16,
Article Number: 21
Publisher
MDPI
Publishing Place
St Alban-anlage 66, Ch-4052 Basel, Switzerland
Non-patent literature
Publications
Reviewing status
Peer reviewed
Institute(s)
Institute of Virology (VIRO)
Grants
China Scholarship Council