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Gong, H.* ; Xue, B.* ; Ru, J. ; Pei, G.* ; Li, Y.*

Targeted therapy for EWS-FLI1 in ewing sarcoma.

Cancers 15:21 (2023)
Verlagsversion DOI PMC
Open Access Gold
Creative Commons Lizenzvertrag
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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Publikationstyp Artikel: Journalartikel
Dokumenttyp Review
Schlagwörter 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
Sprache englisch
Veröffentlichungsjahr 2023
HGF-Berichtsjahr 2023
ISSN (print) / ISBN 2072-6694
Zeitschrift Cancers
Quellenangaben Band: 15, Heft: 16, Seiten: , Artikelnummer: 21 Supplement: ,
Verlag MDPI
Verlagsort St Alban-anlage 66, Ch-4052 Basel, Switzerland
Begutachtungsstatus Peer reviewed
POF Topic(s) 30203 - Molecular Targets and Therapies
Forschungsfeld(er) Immune Response and Infection
PSP-Element(e) G-554300-001
Förderungen China Scholarship Council
Scopus ID 85169033789
PubMed ID 37627063
Erfassungsdatum 2023-10-06