Thursday, July 24, 2014
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Mouse model of Ewing's
sarcoma with cell-specific expression of a transgenic Ewing
sarcoma breakpoint region 1 (EWSR1;
leukemia virus integration 1 (FLI1)
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)
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.
Published online July 24, 2014
Patent and licensing status
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
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