Modeling Initiation of Ewing Sarcoma in Human Neural Crest Cells EWS-FLI1 Expression in Human Neural Crest Cells
2011

Modeling Ewing Sarcoma in Human Neural Crest Cells

publication Evidence: moderate

Author Information

Author(s): von Levetzow Cornelia, Jiang Xiaohua, Gwye Ynnez, von Levetzow Gregor, Hung Long, Cooper Aaron, Hsu Jessie Hao-Ru, Lawlor Elizabeth R.

Primary Institution: Children's Hospital Los Angeles

Hypothesis

EWS-FLI1 expression in human neural crest stem cells (hNCSC) leads to altered gene expression and may initiate Ewing sarcoma.

Conclusion

EWS-FLI1 expression in hNCSC induces a state similar to Ewing sarcoma, suggesting that these cells may be a source of the tumor.

Supporting Evidence

  • EWS-FLI1 expression was tolerated in hNCSC and led to altered gene expression.
  • The molecular signature of Ewing sarcoma is more similar to hNCSC than to other normal tissues.
  • EWS-FLI1 induced genes associated with neural crest stem cells.
  • Up-regulation of BMI-1 was linked to avoidance of cellular senescence.

Takeaway

Scientists studied how a gene linked to a type of cancer affects special cells in the body, finding that it can change these cells in ways that might lead to cancer.

Methodology

The study involved transducing human neural crest stem cells with EWS-FLI1 and analyzing gene expression changes.

Limitations

The study does not explore the full range of genetic changes that may occur in Ewing sarcoma.

Participant Demographics

The study focuses on human embryonic stem cell-derived neural crest stem cells.

Digital Object Identifier (DOI)

10.1371/journal.pone.0019305

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