ASPSCR1:TFE3 Drives Alveolar Soft Part Sarcoma by Inducing Targetable Transcriptional Programs
This study involves a new possible therapeutic strategy for alveolar soft part sarcoma (ASPS), a sarcoma known to be driven by the ASPSCR1::TFE3 fusion. In ASPSCR1:TFE3 Drives Alveolar Soft Part Sarcoma by Inducing Targetable Transcriptional Programs, the investigators strive to gain a better understanding of the mechanisms by which the fusion drives cancer growth and help identify potential therapeutic targets. They determined that the ASPSCR1:TFE3 fusion regulated essential cell programs involved in cell proliferation, angiogenesis, and mitochondrial biology. They also found that the fusion increased cell proliferation by increasing levels of a protein called cyclin D1. They found that inhibition of cyclin D1 signaling decreased cell growth and when combined with inhibition of another protein called CDK4/6, it led to tumor growth in a model of ASPS. These results indicate a possible therapeutic strategy for ASPS.