FLI1: Difference between revisions
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== Clinical significance == | == Clinical significance == | ||
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. Fli-1 aberrant expression is also associated with chromosomal abnormalities in humans. 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. The resulting fusion oncoprotein, EWS/Fli-1, acts as an aberrant transcriptional activator.<ref name="pmid7503813">{{cite journal |vauthors=Ohno T, Rao VN, Reddy ES | title = EWS/Fli-1 chimeric protein is a transcriptional activator | journal = Cancer Res. | volume = 53 | issue = 24 | pages = 5859–63 |date=December 1993 | pmid = 7503813 | doi = }}</ref> with strong transforming capabilities. The importance of Fli-1 in the development of human leukemia, such as acute myelogenous leukemia ([[acute myelogenous leukemia|AML]]), has been demonstrated in studies of translocation involving the [[ETV6|Tel]] transcription factor, which interacts with Fli-1 through protein-protein interactions. A recent study has demonstrated high levels of Fli-1 expression in several benign and malignant neoplasms using immunohistochemistry.{{citation needed|date=December 2010}} | 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. Fli-1 aberrant expression is also associated with chromosomal abnormalities in humans. 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. The resulting fusion oncoprotein, EWS/Fli-1, acts as an aberrant transcriptional activator.<ref name="pmid7503813">{{cite journal |vauthors=Ohno T, Rao VN, Reddy ES | title = EWS/Fli-1 chimeric protein is a transcriptional activator | journal = Cancer Res. | volume = 53 | issue = 24 | pages = 5859–63 |date=December 1993 | pmid = 7503813 | doi = }}</ref> with strong transforming capabilities. EWS/Fli-1 may steer clinically important genes via interaction with enhnacer-like GGAA-microsatellites.<ref>{{Cite journal|last=Musa|first=Julian|last2=Cidre-Aranaz|first2=Florencia|last3=Aynaud|first3=Marie-Ming|last4=Orth|first4=Martin|last5=Mirabeau|first5=Olivier|last6=Varon|first6=Mor|last7=Grossetete|first7=Sandrine|last8=Surdez|first8=Didier|last9=et al.|date=2018-12-27|title=Cooperation of dominant oncogenes with regulatory germline variants shapes clinical outcomes in childhood cancer|url=https://www.biorxiv.org/content/early/2018/12/27/506659|journal=bioRxiv|language=en|volume=|pages=506659|doi=10.1101/506659|via=}}</ref> The importance of Fli-1 in the development of human leukemia, such as acute myelogenous leukemia ([[acute myelogenous leukemia|AML]]), has been demonstrated in studies of translocation involving the [[ETV6|Tel]] transcription factor, which interacts with Fli-1 through protein-protein interactions. A recent study has demonstrated high levels of Fli-1 expression in several benign and malignant neoplasms using immunohistochemistry.{{citation needed|date=December 2010}} | ||
A possible association with [[Paris-Trousseau syndrome]] has been suggested.<ref name="pmid15232614">{{cite journal |vauthors=Raslova H, Komura E, Le Couédic JP, Larbret F, Debili N, Feunteun J, Danos O, Albagli O, Vainchenker W, Favier R | title = FLI1 monoallelic expression combined with its hemizygous loss underlies Paris-Trousseau/Jacobsen thrombopenia | journal = J. Clin. Invest. | volume = 114 | issue = 1 | pages = 77–84 |date=July 2004 | pmid = 15232614 | pmc = 437972 | doi = 10.1172/JCI21197 }}</ref> | A possible association with [[Paris-Trousseau syndrome]] has been suggested.<ref name="pmid15232614">{{cite journal |vauthors=Raslova H, Komura E, Le Couédic JP, Larbret F, Debili N, Feunteun J, Danos O, Albagli O, Vainchenker W, Favier R | title = FLI1 monoallelic expression combined with its hemizygous loss underlies Paris-Trousseau/Jacobsen thrombopenia | journal = J. Clin. Invest. | volume = 114 | issue = 1 | pages = 77–84 |date=July 2004 | pmid = 15232614 | pmc = 437972 | doi = 10.1172/JCI21197 }}</ref> |
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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.[1][2][3]
Function
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). Fli-1 is activated through retroviral insertional mutagenesis in 90% of F-MuLV-induced erythroleukemias. 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. 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. [citation needed]
Clinical significance
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. Fli-1 aberrant expression is also associated with chromosomal abnormalities in humans. 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. The resulting fusion oncoprotein, EWS/Fli-1, acts as an aberrant transcriptional activator.[4] with strong transforming capabilities. EWS/Fli-1 may steer clinically important genes via interaction with enhnacer-like GGAA-microsatellites.[5] 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. A recent study has demonstrated high levels of Fli-1 expression in several benign and malignant neoplasms using immunohistochemistry.[citation needed]
A possible association with Paris-Trousseau syndrome has been suggested.[6]
References
- ↑ Baud V, Lipinski M, Rassart E, Poliquin L, Bergeron D (September 1991). "The human homolog of the mouse common viral integration region, FLI1, maps to 11q23-q24". Genomics. 11 (1): 223–4. doi:10.1016/0888-7543(91)90124-W. PMID 1765382.
- ↑ Prasad DD, Rao VN, Reddy ES (October 1992). "Structure and expression of human Fli-1 gene". Cancer Research. 52 (20): 5833–7. PMID 1394211.
- ↑ Rao VN, Ohno T, Prasad DD, Bhattacharya G, Reddy ES (August 1993). "Analysis of the DNA-binding and transcriptional activation functions of human Fli-1 protein". Oncogene. 8 (8): 2167–73. PMID 8336942.
- ↑ Ohno T, Rao VN, Reddy ES (December 1993). "EWS/Fli-1 chimeric protein is a transcriptional activator". Cancer Res. 53 (24): 5859–63. PMID 7503813.
- ↑ Musa, Julian; Cidre-Aranaz, Florencia; Aynaud, Marie-Ming; Orth, Martin; Mirabeau, Olivier; Varon, Mor; Grossetete, Sandrine; Surdez, Didier; et al. (2018-12-27). "Cooperation of dominant oncogenes with regulatory germline variants shapes clinical outcomes in childhood cancer". bioRxiv: 506659. doi:10.1101/506659.
- ↑ Raslova H, Komura E, Le Couédic JP, Larbret F, Debili N, Feunteun J, Danos O, Albagli O, Vainchenker W, Favier R (July 2004). "FLI1 monoallelic expression combined with its hemizygous loss underlies Paris-Trousseau/Jacobsen thrombopenia". J. Clin. Invest. 114 (1): 77–84. doi:10.1172/JCI21197. PMC 437972. PMID 15232614.
Further reading
- Ben-David Y, Giddens EB, Bernstein A (February 1990). "Identification and mapping of a common proviral integration site Fli-1 in erythroleukemia cells induced by Friend murine leukemia virus". Proc. Natl. Acad. Sci. U.S.A. 87 (4): 1332–6. doi:10.1073/pnas.87.4.1332. PMC 53469. PMID 2304901.
- Ben-David Y, Giddens EB, Letwin K, Bernstein A (June 1991). "Erythroleukemia induction by Friend murine leukemia virus: insertional activation of a new member of the ets gene family, Fli-1, closely linked to c-ets-1". Genes Dev. 5 (6): 908–18. doi:10.1101/gad.5.6.908. PMID 2044959.
- Delattre O, Zucman J, Plougastel B, Desmaze C, Melot T, Peter M, Kovar H, Joubert I, de Jong P, Rouleau G (September 1992). "Gene fusion with an ETS DNA-binding domain caused by chromosome translocation in human tumours". Nature. 359 (6391): 162–5. doi:10.1038/359162a0. PMID 1522903.
- Pereira R, Quang CT, Lesault I, Dolznig H, Beug H, Ghysdael J (February 1999). "FLI-1 inhibits differentiation and induces proliferation of primary erythroblasts". Oncogene. 18 (8): 1597–608. doi:10.1038/sj.onc.1202534. PMID 10102630.
- Tamir A, Howard J, Higgins RR, Li YJ, Berger L, Zacksenhaus E, Reis M, Ben-David Y (June 1999). "Fli-1, an Ets-related transcription factor, regulates erythropoietin-induced erythroid proliferation and differentiation: evidence for direct transcriptional repression of the Rb gene during differentiation". Mol. Cell. Biol. 19 (6): 4452–64. PMC 104404. PMID 10330185.
- Zochodne B, Truong AH, Stetler K, Higgins RR, Howard J, Dumont D, Berger SA, Ben-David Y (May 2000). "Epo regulates erythroid proliferation and differentiation through distinct signaling pathways: implication for erythropoiesis and Friend virus-induced erythroleukemia". Oncogene. 19 (19): 2296–304. doi:10.1038/sj.onc.1203590. PMID 10822380.
- Starck J, Doubeikovski A, Sarrazin S, Gonnet C, Rao G, Skoultchi A, Godet J, Dusanter-Fourt I, Morle F (January 1999). "Spi-1/PU.1 is a positive regulator of the Fli-1 gene involved in inhibition of erythroid differentiation in friend erythroleukemic cell lines". Mol. Cell. Biol. 19 (1): 121–35. PMC 83871. PMID 9858537.
- Truong AH, Ben-David Y (December 2000). "The role of Fli-1 in normal cell function and malignant transformation". Oncogene. 19 (55): 6482–9. doi:10.1038/sj.onc.1204042. PMID 11175364.
- Ohno T, Rao VN, Reddy ES (December 1993). "EWS/Fli-1 chimeric protein is a transcriptional activator". Cancer Res. 53 (24): 5859–63. PMID 7503813.
- Dhulipala PD, Lee L, Rao VN, Reddy ES (September 1998). "Fli-1b is generated by usage of differential splicing and alternative promoter". Oncogene. 17 (9): 1149–57. doi:10.1038/sj.onc.1202030. PMID 9764825.
- Rao VN, Ohno T, Prasad DD, Bhattacharya G, Reddy ES (August 1993). "Analysis of the DNA-binding and transcriptional activation functions of human Fli-1 protein". Oncogene. 8 (8): 2167–73. PMID 8336942.
- Ban J, Siligan C, Kreppel M, et al. (2007). "EWS-FLI1 in Ewing's sarcoma: real targets and collateral damage". Adv. Exp. Med. Biol. 587: 41–52. doi:10.1007/978-1-4020-5133-3_4. PMID 17163154.
- Prasad DD, Rao VN, Reddy ES (1992). "Structure and expression of human Fli-1 gene". Cancer Res. 52 (20): 5833–7. PMID 1394211.
- Watson DK, Smyth FE, Thompson DM, et al. (1992). "The ERGB/Fli-1 gene: isolation and characterization of a new member of the family of human ETS transcription factors". Cell Growth Differ. 3 (10): 705–13. PMID 1445800.
- Delattre O, Zucman J, Plougastel B, et al. (1992). "Gene fusion with an ETS DNA-binding domain caused by chromosome translocation in human tumours". Nature. 359 (6391): 162–5. doi:10.1038/359162a0. PMID 1522903.
- Baud V, Lipinski M, Rassart E, et al. (1992). "The human homolog of the mouse common viral integration region, FLI1, maps to 11q23-q24". Genomics. 11 (1): 223–4. doi:10.1016/0888-7543(91)90124-W. PMID 1765382.
- Bergeron D, Poliquin L, Kozak CA, Rassart E (1991). "Identification of a common viral integration region in Cas-Br-E murine leukemia virus-induced non-T-, non-B-cell lymphomas". J. Virol. 65 (1): 7–15. PMC 240483. PMID 1845910.
- Bhagirath T, Abe S, Nojima T, Yoshida MC (1995). "Molecular analysis of a t(11;22) translocation junction in a case of Ewing's sarcoma". Genes Chromosomes Cancer. 13 (2): 126–32. doi:10.1002/gcc.2870130209. PMID 7542907.
- Liang H, Mao X, Olejniczak ET, et al. (1995). "Solution structure of the ets domain of Fli-1 when bound to DNA". Nat. Struct. Biol. 1 (12): 871–5. doi:10.1038/nsb1294-871. PMID 7773776.
- May WA, Lessnick SL, Braun BS, et al. (1994). "The Ewing's sarcoma EWS/FLI-1 fusion gene encodes a more potent transcriptional activator and is a more powerful transforming gene than FLI-1". Mol. Cell. Biol. 13 (12): 7393–8. PMC 364810. PMID 8246959.
- Hromas R, May W, Denny C, et al. (1993). "Human FLI-1 localizes to chromosome 11Q24 and has an aberrant transcript in neuroepithelioma". Biochim. Biophys. Acta. 1172 (1–2): 155–8. doi:10.1016/0167-4781(93)90283-J. PMID 8439553.
- Magnaghi-Jaulin L, Masutani H, Robin P, et al. (1996). "SRE elements are binding sites for the fusion protein EWS-FLI-1". Nucleic Acids Res. 24 (6): 1052–8. doi:10.1093/nar/24.6.1052. PMC 145748. PMID 8604338.
- Barbeau B, Bergeron D, Beaulieu M, et al. (1996). "Characterization of the human and mouse Fli-1 promoter regions". Biochim. Biophys. Acta. 1307 (2): 220–32. doi:10.1016/0167-4781(96)00060-7. PMID 8679708.
- Watson DK, Robinson L, Hodge DR, et al. (1997). "FLI1 and EWS-FLI1 function as ternary complex factors and ELK1 and SAP1a function as ternary and quaternary complex factors on the Egr1 promoter serum response elements". Oncogene. 14 (2): 213–21. doi:10.1038/sj.onc.1200839. PMID 9010223.
- Kwiatkowski BA, Bastian LS, Bauer TR, et al. (1998). "The ets family member Tel binds to the Fli-1 oncoprotein and inhibits its transcriptional activity". J. Biol. Chem. 273 (28): 17525–30. doi:10.1074/jbc.273.28.17525. PMID 9651344.
- Carrère S, Verger A, Flourens A, et al. (1998). "Erg proteins, transcription factors of the Ets family, form homo, heterodimers and ternary complexes via two distinct domains". Oncogene. 16 (25): 3261–8. doi:10.1038/sj.onc.1201868. PMID 9681824.
- Zucman-Rossi J, Legoix P, Victor JM, et al. (1998). "Chromosome translocation based on illegitimate recombination in human tumors". Proc. Natl. Acad. Sci. U.S.A. 95 (20): 11786–91. doi:10.1073/pnas.95.20.11786. PMC 21718. PMID 9751743.
- Dalgleish P, Sharrocks AD (2000). "The mechanism of complex formation between Fli-1 and SRF transcription factors". Nucleic Acids Res. 28 (2): 560–9. doi:10.1093/nar/28.2.560. PMC 102515. PMID 10606656.
- Knoop LL, Baker SJ (2000). "The splicing factor U1C represses EWS/FLI-mediated transactivation". J. Biol. Chem. 275 (32): 24865–71. doi:10.1074/jbc.M001661200. PMID 10827180.
- Spyropoulos DD, Pharr PN, Lavenburg KR, et al. (2000). "Hemorrhage, impaired hematopoiesis, and lethality in mouse embryos carrying a targeted disruption of the Fli1 transcription factor". Mol. Cell. Biol. 20 (15): 5643–52. doi:10.1128/MCB.20.15.5643-5652.2000. PMC 86032. PMID 10891501.
- Mastrangelo T, Modena P, Tornielli S, et al. (2000). "A novel zinc finger gene is fused to EWS in small round cell tumor". Oncogene. 19 (33): 3799–804. doi:10.1038/sj.onc.1203762. PMID 10949935.
External links
- FLI1+protein,+human at the US National Library of Medicine Medical Subject Headings (MeSH)
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