“Functional Genomic Characterization of a Conditional Mouse Model of Alveolar Rhabdomyosarcoma: Comparison to Human Tumors”
Rhabdomyosarcomas are the most common childhood soft tissue sarcoma. We have developed a conditional mouse model of alveolar rhabdomyosarcomas by expressing the Pax3:Fkhr oncogene in skeletal muscle of juvenile mice. We propose to test that our mouse model mimics the secondary genomic and gene expression changes seen in human alveolar rhabdomyosarcomas. We will (1) compare chromosomal segment gains and losses in 8 mouse and 8 human alveolar rhabdomyosarcomas, and (2) correlate genomic imbalances of mouse and human tumors to gene expression changes of those same tumors. Understanding molecular events underlying tumor invasiveness and metastasis, we simultaneously identify potential therapeutic targets.