2026-07-16T14:10:36Zhttps://repisalud.isciii.es/rest/oai/request

oai:repisalud.isciii.es:20.500.12105/134992025-04-29T10:36:43Zcom_20.500.12105_14556com_20.500.12105_2337com_20.500.12105_2336com_20.500.12105_2052com_20.500.12105_2051col_20.500.12105_14558col_20.500.12105_19616

00925njm 22002777a 4500 dc Garcia-Garcia, Laura author Fernández-Tabanera, Enrique author Cervera Mayor, Saint Thomas author Melero-Fernández de Mera, Raquel María author Josa, Santiago author Gonzalez-Gonzalez, Laura author Rodriguez-Martin, Carlos author Grünewald, Thomas G P author Alonso, Javier author 2021-11-12 Ewing sarcoma is a rare pediatric tumor characterized by chromosomal translocations that give rise to aberrant chimeric transcription factors (e.g., EWSR1-FLI1). EWSR1-FLI1 promotes a specific cellular transcriptional program. Therefore, the study of EWSR1-FLI1 target genes is important to identify critical pathways involved in Ewing sarcoma tumorigenesis. In this work, we focused on the transcription factors regulated by EWSR1-FLI1 in Ewing sarcoma. Transcriptomic analysis of the Ewing sarcoma cell line A673 indicated that one of the genes more strongly upregulated by EWSR1-FLI1 was FEZF1 (FEZ family zinc finger protein 1), a transcriptional repressor involved in neural cell identity. The functional characterization of FEZF1 was performed in three Ewing sarcoma cell lines (A673, SK-N-MC, SK-ES-1) through an shRNA-directed silencing approach. FEZF1 knockdown inhibited clonogenicity and cell proliferation. Finally, the analysis of the FEZF1-dependent expression profile in A673 cells showed several neural genes regulated by FEZF1 and concomitantly regulated by EWSR1-FLI1. In summary, FEZF1 is transcriptionally regulated by EWSR1-FLI1 in Ewing sarcoma cells and is involved in the regulation of neural-specific genes, which could explain the neural-like phenotype observed in several Ewing sarcoma tumors and cell lines. Cancers (Basel). 2021 Nov 12;13(22):5668. 10.3390/cancers13225668 2072-6694 Cancers 34830820 http://hdl.handle.net/20.500.12105/13499 EWSR1-FLI1 Ewing sarcoma FEZF11 GGAA-microsatellites The Transcription Factor FEZF1, a Direct Target of EWSR1-FLI1 in Ewing Sarcoma Cells, Regulates the Expression of Neural-Specific Genes