PUBLICATION

Cell-to-cell heterogeneity of EWSR1-FLI1 activity determines proliferation/migration choices in Ewing sarcoma cells

Authors
Franzetti, G.A., Laud-Duval, K., van der Ent, W., Brisac, A., Irondelle, M., Aubert, S., Dirksen, U., Bouvier, C., de Pinieux, G., Snaar-Jagalska, E., Chavrier, P., Delattre, O.
ID
ZDB-PUB-170916-12
Date
2017
Source
Oncogene   36: 3505-3514 (Journal)
Registered Authors
Snaar-Jagalska, Ewa B., van der Ent, Wietske
Keywords
none
MeSH Terms
  • Animals
  • Calmodulin-Binding Proteins/biosynthesis*
  • Calmodulin-Binding Proteins/genetics
  • Cell Line, Tumor
  • Cell Movement*
  • Cell Proliferation*
  • Gene Expression Regulation, Neoplastic*
  • Mice
  • Mice, SCID
  • Neoplasm Invasiveness
  • Neoplasm Metastasis
  • Oncogene Proteins, Fusion/biosynthesis*
  • Oncogene Proteins, Fusion/genetics
  • Proto-Oncogene Protein c-fli-1/biosynthesis*
  • Proto-Oncogene Protein c-fli-1/genetics
  • RNA-Binding Proteins/biosynthesis*
  • RNA-Binding Proteins/genetics
  • Sarcoma, Ewing/genetics
  • Sarcoma, Ewing/metabolism*
  • Sarcoma, Ewing/pathology
  • Zebrafish
PubMed
28135250 Full text @ Oncogene
Abstract
Ewing sarcoma is characterized by the expression of the chimeric EWSR1-FLI1 transcription factor. Proteomic analyses indicate that the decrease of EWSR1-FLI1 expression leads to major changes in effectors of the dynamics of the actin cytoskeleton and the adhesion processes with a shift from cell-to-cell to cell-matrix adhesion. These changes are associated with a dramatic increase of in vivo cell migration and invasion potential. Importantly, EWSR1-FLI1 expression, evaluated by single-cell RT-ddPCR/immunofluorescence analyses, and activity, assessed by expression of EWSR1-FLI1 downstream targets, are heterogeneous in cell lines and in tumours and can fluctuate along time in a fully reversible process between EWSR1-FLI1high states, characterized by highly active cell proliferation, and EWSR1-FLI1low states where cells have a strong propensity to migrate, invade and metastasize. This new model of phenotypic plasticity proposes that the dynamic fluctuation of the expression level of a dominant oncogene is an intrinsic characteristic of its oncogenic potential.
Genes / Markers
Figures
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Expression
Phenotype
Mutations / Transgenics
Human Disease / Model
Sequence Targeting Reagents
Fish
Antibodies
Orthology
Engineered Foreign Genes
Mapping