Why the study?
Alveolar soft part sarcoma is characterized by a prominent vascular network and high metastatic potential, indicating the importance of its angiogenic activity.
Does ASPSCR1::TFE3 loss or JQ1 treatment reduce in vivo tumor development and angiogenesis in ASPS models?
Does ASPSCR1::TFE3 loss or JQ1 treatment reduce in vivo tumor development and angiogenesis in ASPS models?
ASPSCR1::TFE3 orchestrates angiogenesis in alveolar soft part sarcoma by modulating super-enhancer activity, which can be targeted by BET inhibitors like JQ1 to suppress tumor growth.
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ASPSCR1::TFE3 drives in vivo angiogenesis-dependent growth but not in vitro maintenance; leaves open fusion-targeted therapy development in ASPS.
Tanaka et al. (2023) studied this question.
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