• Fli-1 is a proto-oncogene implicated in Ewing's sarcoma and erythroleukemia. (umbc.edu)
  • Targeted inhibition of histone deacetylase leads to suppression of Ewing sarcoma tumor growth through an unappreciated EWS-FLI1/HDAC3/HSP90 signaling axis. (childrensmercy.org)
  • Zhang P, Samuel G, Crow J, Godwin AK, Zeng Y. Molecular assessment of circulating exosomes toward liquid biopsy diagnosis of Ewing sarcoma family of tumors. (childrensmercy.org)
  • In pediatric Ewing's sarcoma a chromosomal translocation generates a fusion of the 5' transactivation domain of EWSR1 (also known as EWS) with the 3' Ets domain of Fli-1. (wikipedia.org)
  • Signature-based small molecule screening identifies cytosine arabinoside as an EWS/FLI modulator in Ewing sarcoma. (broadinstitute.org)
  • One illustrative example is the treatment of Ewing sarcoma. (broadinstitute.org)
  • A gene expression signature for the EWS/FLI off state was determined with microarray expression profiling of Ewing sarcoma cell lines with EWS/FLI-directed RNA interference. (broadinstitute.org)
  • Given the poor outcomes of many patients with Ewing sarcoma and the well-established ARA-C safety profile, clinical trials testing ARA-C are warranted. (broadinstitute.org)
  • Cyclophosphamide and topotecan as first-line salvage therapy in patients with relapsed ewing sarcoma at a single institution. (musc.edu)
  • citation needed] In addition to Friend erythroleukemia, proviral integration at the fli-1 locus also occurs in leukemias induced by the 10A1, Graffi, and Cas-Br-E viruses. (wikipedia.org)
  • Among the ETS family members, Friend leukemia virus integration 1 ( FLI1 ) was first identified as a proto-oncogene activated by proviral integration in F-MuLV-induced erythroleukemias [ 4 ]. (biomedcentral.com)
  • Although the EWS/FLI oncoprotein, present in the vast majority of Ewing tumors, was characterized over ten years ago, it has never been exploited as a target of therapy. (broadinstitute.org)
  • Friend leukemia virus integration 1 (FLI1), an ETS transcription factor family member, acts as an oncogenic driver in hematological malignancies and promotes tumor growth in solid tumors. (biomedcentral.com)
  • However, little is known about the mechanisms underlying the activation of this proto-oncogene in tumors. (biomedcentral.com)
  • The resulting fusion oncoprotein, EWS/Fli-1, acts as an aberrant transcriptional activator. (wikipedia.org)
  • Sterile alpha motif (SAM)/Pointed domain of friend leukemia integration 1 transcription activator. (umbc.edu)
  • SAM Pointed domain of FLI-1 (Friend Leukemia Integration) subfamily of ETS transcriptional regulators is a putative protein-protein interaction domain. (umbc.edu)
  • Friend leukemia integration 1 transcription factor (FLI1), also known as transcription factor ERGB, is a protein that in humans is encoded by the FLI1 gene, which is a proto-oncogene. (wikipedia.org)
  • Despite growing knowledge of the functions of individual human transcriptional effector domains, much less is understood about how multiple effector domains within the same protein combine to regulate gene expression. (bvsalud.org)
  • The Fli-1 gene was initially described in Friend virus-induced erythroleukemias as a site for virus integration. (umbc.edu)
  • Fli-1 is a member of the ETS transcription factor family that was first identified in erythroleukemias induced by Friend Murine Leukemia Virus (F-MuLV). (wikipedia.org)
  • Fli-1 is activated through retroviral insertional mutagenesis in 90% of F-MuLV-induced erythroleukemias. (wikipedia.org)
  • ARA-C reduced EWS/FLI protein abundance and accordingly diminished cell viability and transformation and abrogated tumor growth in a xenograft model. (broadinstitute.org)
  • The FLI-1 protein participates in regulation of cellular differentiation, proliferation, and survival. (umbc.edu)
  • The constitutive activation of fli-1 in erythroblasts leads to a dramatic shift in the Epo/Epo-R signal transduction pathway, blocking erythroid differentiation, activating the Ras pathway, and resulting in massive Epo-independent proliferation of erythroblasts. (wikipedia.org)
  • These results suggest that Fli-1 overexpression in erythroblasts alters their responsiveness to Epo and triggers abnormal proliferation by switching the signaling event(s) associated with terminal differentiation to proliferation. (wikipedia.org)
  • It was originally identified as a protein that provides a retroviral integration site for integration of FRIEND MURINE LEUKEMIA VIRUS. (childrensmercy.org)
  • Here we describe a gene expression-based approach to identify compounds that induce a signature of EWS/FLI attenuation. (broadinstitute.org)
  • The mutant proteins often retain some of their capabilities but are no longer sensitive to the controls that regulate the normal form of the protein. (cancerquest.org)
  • The importance of Fli-1 in the development of human leukemia, such as acute myelogenous leukemia (AML), has been demonstrated in studies of translocation involving the Tel transcription factor, which interacts with Fli-1 through protein-protein interactions. (wikipedia.org)
  • A key feature of oncogene activity is that a single altered copy leads to unregulated growth. (cancerquest.org)
  • These results suggested that the Ews/Fli-1 fusion gene, resulting from a translocation unique to ES/PNETs, might account for the loss of NB-specific markers. (nih.gov)
  • The importance of Fli-1 in the development of human leukemia, such as acute myelogenous leukemia (AML), has been demonstrated in studies of translocation involving the Tel transcription factor, which interacts with Fli-1 through protein-protein interactions. (wikipedia.org)
  • Friend leukemia integration 1 transcription factor (FLI1), also known as transcription factor ERGB, is a protein that in humans is encoded by the FLI1 gene, which is a proto-oncogene. (wikipedia.org)
  • 11. Transactivation of GATA-1 promoter with ETS1, ETS2 and ERGB/Hu-FLI-1 proteins: stabilization of the ETS1 protein binding on GATA-1 promoter sequences by monoclonal antibody. (nih.gov)
  • Therefore, we propose that Ews/Fli-1 contributes to the etiology of ES/PNET by subverting the differentiation program of its neural crest precursor cell to a less differentiated and more proliferative state. (nih.gov)
  • The proteins produced from these genes control many important cellular processes, such as cell growth and division (proliferation), maturation (differentiation), and survival. (medlineplus.gov)
  • The constitutive activation of fli-1 in erythroblasts leads to a dramatic shift in the Epo/Epo-R signal transduction pathway, blocking erythroid differentiation, activating the Ras pathway, and resulting in massive Epo-independent proliferation of erythroblasts. (wikipedia.org)
  • These results suggest that Fli-1 overexpression in erythroblasts alters their responsiveness to Epo and triggers abnormal proliferation by switching the signaling event(s) associated with terminal differentiation to proliferation. (wikipedia.org)
  • EWS/Fli-1 chimeric protein is a transcriptional activator. (medlineplus.gov)
  • The resulting fusion oncoprotein, EWS/Fli-1, acts as an aberrant transcriptional activator. (wikipedia.org)
  • 15. EWS/FLI-1 antagonists induce growth inhibition of Ewing tumor cells in vitro. (nih.gov)
  • Fli-1 aberrant expression is also associated with chromosomal abnormalities in humans. (wikipedia.org)
  • To test this hypothesis, we generated two different NB cell lines that stably expressed the Ews/Fli-1 gene. (nih.gov)
  • This analysis revealed that the gene expression pattern of the Ews/Fli-1-expressing NB cells resembled that observed in pooled ES/PNET cell lines and differed significantly from the NB parental cells. (nih.gov)
  • The FLI1 gene provides instructions for making the FLI protein, which controls the activity (transcription) of genes. (medlineplus.gov)
  • The protein produced from the EWSR1/FLI1 fusion gene, called EWS/FLI, has functions of the protein products of both genes. (medlineplus.gov)
  • Like the FLI protein, the EWS protein, produced from the EWSR1 gene, can regulate transcription. (medlineplus.gov)
  • The EWSR1/FLI1 fusion gene occurs in approximately 85 percent of Ewing sarcomas. (medlineplus.gov)
  • 9. Regulation of the human stress response gene GADD153 expression: role of ETS1 and FLI-1 gene products. (nih.gov)
  • The FLI protein attaches (binds) to certain regions of DNA and turns on (activates) the transcription of nearby genes. (medlineplus.gov)
  • 10. Exon 4-encoded acidic domain in the epithelium-restricted Ets factor, ESX, confers potent transactivating capacity and binds to TATA-binding protein (TBP). (nih.gov)
  • Fli-1 is a member of the ETS transcription factor family that was first identified in erythroleukemias induced by Friend Murine Leukemia Virus (F-MuLV). (wikipedia.org)
  • 1 Greehey Children's Cancer Research Institute, The University of Texas Health Science Center, San Antonio, TX 78229, USA. (nih.gov)
  • citation needed] In addition to Friend erythroleukemia, proviral integration at the fli-1 locus also occurs in leukemias induced by the 10A1, Graffi, and Cas-Br-E viruses. (wikipedia.org)
  • We observed that heterologous expression of the Ews/Fli-1 protein led to the suppression of NB-specific markers and de novo expression of ES/PNET markers. (nih.gov)
  • Here, we demonstrate that EWS-FLI1 positively regulates the expression of proteins required for serine-glycine biosynthesis and uptake of the alternative nutrient source glutamine. (nih.gov)
  • A recent study has demonstrated high levels of Fli-1 expression in several benign and malignant neoplasms using immunohistochemistry. (wikipedia.org)
  • The FLI protein is found primarily in blood cells and is thought to regulate their development. (medlineplus.gov)