Journal
CANCER CELL
Volume 22, Issue 4, Pages 536-546Publisher
CELL PRESS
DOI: 10.1016/j.ccr.2012.09.004
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Funding
- National Institutes of Health
- Leducq Foundation
- Robert A. Welch Foundation
- Cancer Prevention and Research Institute of Texas
- Alex's Lemonade Stand Foundation
- Eunice Kennedy Shriver National Institute of Child Health and Human Development [K12-HD000850]
- American Lebanese Syrian Associated Charities (ALSAC)
- Burroughs Wellcome Fund Career Award for Medical Scientists
- American Cancer Society/Simmons Cancer Center Institutional Research Grant [ACS-IRG-02-196]
- CureSearch for Children's Cancer Young Investigator Program
- NIDDK [R01-DK066566, R01-DK064261, R01-DK088220]
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Rhabdomyosarcoma (RMS) is an aggressive skeletal muscle-lineage tumor composed of malignant myoblasts that fail to exit the cell cycle and are blocked from fusing into syncytial muscle. Rhabdomyosarcoma includes two histolopathologic subtypes: alveolar rhabdomyosarcoma, driven by the fusion protein PAX3-FOXO1 or PAX7-FOXO1, and embryonal rhabdomyosarcoma (ERMS), which is genetically heterogeneous. Here, we show that adipocyte-restricted activation of Sonic hedgehog signaling through expression of a constitutively active Smoothened allele in mice gives rise to aggressive skeletal muscle tumors that display the histologic and molecular characteristics of human ERMS with high penetrance. Our findings suggest that adipocyte progenitors can be a cell of origin for Sonic hedgehog-driven ERMS, showing that RMS can originate from nonskeletal muscle precursors.
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