UNASSIGNED: @entity980 ( @entity980 ) typically arises from skeletal muscle. Currently, @entity980 in @entity1 with recurrent and @entity3 have no successful treatment. The molecular pathogenesis of @entity980 varies based on @entity5 sub-types. Some embryonal @entity980 but not other sub-types are driven by sonic hedgehog ( @entity4851 ) signaling pathway. However, @entity4851 pathway inhibitors particularly smoothened inhibitors are not highly effective in animals. Here, we show that @entity4851 pathway effectors @entity8889 and/or @entity1357 are over-expressed in the majority of @entity980 cells and that @entity35165 , a specific @entity63854 inhibitor dampens the proliferation of both embryonal and alveolar @entity980 cells-derived xenograft @entity5 thereby blocking their growth. As compared to vehicle-treated control, about 50% @entity5 growth inhibition occurs in @entity19 receiving @entity35165 treatment. The proliferation inhibition was associated with slowing of cell cycle progression which was mediated by the reduced expression of cyclins @entity27173 and the concomitant induction of @entity3544 . @entity35165 not only reduced expression of @entity63854 in these @entity980 but also significantly diminished @entity584 / @entity3470 signaling. The therapeutic action of @entity35165 was significantly augmented when combined with chemotherapeutic agents employed for @entity980 therapy such as @entity13850 or @entity1891 . Finally, reduced expression of proteins driving epithelial mesenchymal transition (EMT) characterized the @entity41 .

GLI inhibitor XXXX diminishes embryonal and alveolar rhabdomyosarcoma growth by inhibiting @entity4851 / @entity584 - @entity3470 axis.

@entity1 :: ('9606', 'Species') :: ['patients'] @entity13850 :: ('MESH:C401859', 'Chemical') :: ['temsirolimus'] @entity1357 :: ('2736', 'Gene') :: ['GLI2'] @entity41 :: ('MESH:D018365', 'Disease') :: ['residual tumors'] @entity5 :: ('MESH:D009369', 'Disease') :: ['tumor', 'cancer', 'tumors'] @entity63854 :: ('2735;2736', 'Gene') :: ['GLI1/2'] @entity19 :: ('10090', 'Species') :: ['mice'] @entity3544 :: ('644914', 'Gene') :: ['p21'] @entity584 :: ('207', 'Gene') :: ['AKT'] @entity8889 :: ('2735', 'Gene') :: ['GLI1'] @entity35165 :: ('MESH:C551027', 'Chemical') :: ['GANT-61'] @entity27173 :: ('55850', 'Gene') :: ['D1/2/3 _ E'] @entity3470 :: ('21977(Tax:10090)', 'Gene') :: ['mTOR'] @entity4851 :: ('6469', 'Gene') :: ['Shh'] @entity3 :: ('MESH:D009362', 'Disease') :: ['metastatic disease'] @entity1891 :: ('MESH:D014750', 'Chemical') :: ['vincristine'] @entity980 :: ('MESH:D012208', 'Disease') :: ['RMS', 'Rhabdomyosarcoma']
@entity35165 :: (MESH:C551027,Chemical) :: ['GANT-61']
