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EWS-FLI1 causes neuroepithelial defects and abrogates emigration of neural crest stem cells.
Stem Cells. 2008 Sep; 26(9):2237-44.SC

Abstract

The most frequently occurring chromosomal translocation that gives rise to the Ewing's sarcoma family of tumors (ESFT) is the chimeric fusion gene EWS-FLI1 that encodes an oncogenic protein composed of the N terminus of EWS and the C terminus of FLI1. Although the genetic basis of ESFT is fairly well understood, its putative cellular origin remains to be determined. Previous work has proposed that neural crest progenitor cells may be the causative cell type responsible for ESFT. However, surprisingly little is known about the expression pattern or role of either wild-type EWS or wild-type FLI1 in this cell population during early embryonic development. Using the developing chick embryo as a model system, we identified EWS expression in emigrating and migratory neural crest stem cells, whereas FLI1 transcripts were found to be absent in these populations and were restricted to developing endothelial cells. By ectopically expressing EWS-FLI1 or wild-type FLI1 in the developing embryo, we have been able to study the cellular transformations that ensue in the context of an in vivo model system. Our results reveal that misexpression of the chimeric EWS-FLI1 fusion gene, or wild-type FLI1, in the developing neural crest stem cell population leads to significant aberrations in neural crest development. An intriguing possibility is that misexpression of the EWS-FLI1 oncogene in neural crest-derived stem cells may be an initiating event in ESFT genesis.

Authors+Show Affiliations

Division of Biology M/C 139-74, California Institute of Technology, Pasadena, California 91125, USA.No affiliation info availableNo affiliation info available

Pub Type(s)

Journal Article
Research Support, N.I.H., Extramural

Language

eng

PubMed ID

18556509

Citation

Coles, Edward G., et al. "EWS-FLI1 Causes Neuroepithelial Defects and Abrogates Emigration of Neural Crest Stem Cells." Stem Cells (Dayton, Ohio), vol. 26, no. 9, 2008, pp. 2237-44.
Coles EG, Lawlor ER, Bronner-Fraser M. EWS-FLI1 causes neuroepithelial defects and abrogates emigration of neural crest stem cells. Stem Cells. 2008;26(9):2237-44.
Coles, E. G., Lawlor, E. R., & Bronner-Fraser, M. (2008). EWS-FLI1 causes neuroepithelial defects and abrogates emigration of neural crest stem cells. Stem Cells (Dayton, Ohio), 26(9), 2237-44. https://doi.org/10.1634/stemcells.2008-0133
Coles EG, Lawlor ER, Bronner-Fraser M. EWS-FLI1 Causes Neuroepithelial Defects and Abrogates Emigration of Neural Crest Stem Cells. Stem Cells. 2008;26(9):2237-44. PubMed PMID: 18556509.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - EWS-FLI1 causes neuroepithelial defects and abrogates emigration of neural crest stem cells. AU - Coles,Edward G, AU - Lawlor,Elizabeth R, AU - Bronner-Fraser,Marianne, Y1 - 2008/06/12/ PY - 2008/6/17/pubmed PY - 2009/2/12/medline PY - 2008/6/17/entrez SP - 2237 EP - 44 JF - Stem cells (Dayton, Ohio) JO - Stem Cells VL - 26 IS - 9 N2 - The most frequently occurring chromosomal translocation that gives rise to the Ewing's sarcoma family of tumors (ESFT) is the chimeric fusion gene EWS-FLI1 that encodes an oncogenic protein composed of the N terminus of EWS and the C terminus of FLI1. Although the genetic basis of ESFT is fairly well understood, its putative cellular origin remains to be determined. Previous work has proposed that neural crest progenitor cells may be the causative cell type responsible for ESFT. However, surprisingly little is known about the expression pattern or role of either wild-type EWS or wild-type FLI1 in this cell population during early embryonic development. Using the developing chick embryo as a model system, we identified EWS expression in emigrating and migratory neural crest stem cells, whereas FLI1 transcripts were found to be absent in these populations and were restricted to developing endothelial cells. By ectopically expressing EWS-FLI1 or wild-type FLI1 in the developing embryo, we have been able to study the cellular transformations that ensue in the context of an in vivo model system. Our results reveal that misexpression of the chimeric EWS-FLI1 fusion gene, or wild-type FLI1, in the developing neural crest stem cell population leads to significant aberrations in neural crest development. An intriguing possibility is that misexpression of the EWS-FLI1 oncogene in neural crest-derived stem cells may be an initiating event in ESFT genesis. SN - 1549-4918 UR - https://www.unboundmedicine.com/medline/citation/18556509/EWS_FLI1_causes_neuroepithelial_defects_and_abrogates_emigration_of_neural_crest_stem_cells_ L2 - https://doi.org/10.1634/stemcells.2008-0133 DB - PRIME DP - Unbound Medicine ER -