A mouse model of rhabdomyosarcoma originating from the adipocyte lineage

Activation of Sonic Hedgehog signaling through expression of a constitutively active Smoothened allele under control of an aP2 adipocyte-restricted transgene in mice gives rise to aggressive skeletal muscle tumors that display the histologic and molecular characteristics of human embryonal rhabdomyosarcoma with high penetrance. Conditional mouse models of embryonal rhabdomyosarcoma were created by activation of the oncogenic SmoM2 allele by both the non-muscle aP2-Cre or myotube Myogenin-Cre.