biostudies-literatureGarcia-Garcia LCentre for Biomedical Network Research on Rare DiseasesInstituto de Salud Carlos IIIAsociación Candela Riera, Asociación Todos Somos Iván & Fundación Sonrisa de AlexAsociación Pablo UgarteASIONAsociación Candela Riera, Asociación Todos Somos Iván &amp;amp; Fundación Sonrisa de Alex5668https://www.ebi.ac.uk/biostudies/studies/S-EPMC8616448biostudies-literatureUnknown13(22)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, CancersThe Transcription Factor <i>FEZF1</i>, a Direct Target of <i>EWSR1-FLI1</i> in Ewing Sarcoma Cells, Regulates the Expression of Neural-Specific Genes.PMC8616448TRPV 205/18U758-PostdocPI16CIII/00026TVP141/17TVP333-19PI20CIII/00020TPI-M 1149/13DTS18CIII/00005TVP-1324/15Melero-Fernandez de Mera RMGonzalez-Gonzalez LFernandez-Tabanera ECervera STGrunewald TGPAlonso JGarcia-Garcia LRodriguez-Martin CJosa SfalseThe Transcription Factor <i>FEZF1</i>, a Direct Target of <i>EWSR1-FLI1</i> in Ewing Sarcoma Cells, Regulates the Expression of Neural-Specific Genes.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, 2021-01-01T00:00:00Z2021 Nov2026-04-08T08:20:47.542Z2025-05-29T19:23:32.303ZS-EPMC86164483483082010.3390/cancers13225668