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Disease models

Mouse model of Ewing's sarcoma with cell-specific expression of a transgenic Ewing sarcoma breakpoint region 1 (EWSR1; EWS)-Friend leukemia virus integration 1 (FLI1) fusion protein

Mice expressing a human EWS-FLI1 fusion protein in specific bone cells could be useful as models for evaluating therapies to treat Ewing's sarcoma. Mice injected with osteochondroprogenitor cells transfected with a human EWS-FLI1 fusion gene developed sarcoma tumors with cell morphology and gene expression profiles that closely matched those found in human Ewing's sarcoma. In the transgenic models, established interventions such as FLI1 siRNA and inhibitors of poly(ADP-ribose) polymerase-1 (PARP-1) or wingless-type MMTV integration site (WNT) and b-catenin (CTNNB1) signaling decreased tumor growth in a dose-dependent manner compared with no treatment. Next steps could include testing candidate Ewing's sarcoma therapies in the model.

SciBX 7(28); doi:10.1038/scibx.2014.841
Published online July 24, 2014

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Tanaka, M. et al. J. Clin. Invest.; published online June 9, 2014;
Contact: Takuro Nakamura, The Cancer Institute, Japanese Foundation for Cancer Research, Tokyo, Japan