Notably, the RREB1-MKL2 chimeric transcription factor encoded by this fusion gene produced an increase in MKL2 expression, which regulates both neural and myogenic differentiation, mimicking the crucial role of PAX3 reported in SNS oncogenesis.
We applied this <i>PAX3-FOXO1</i> transgenic zebrafish model to study how <i>PAX3-FOXO1</i> leverages early developmental pathways for oncogenesis and found that <i>her3</i> is a unique target.
Recently, PAX3 overexpression has been implicated in glioma tumorigenesis by enhancing proliferation, increasing invasiveness and inducing resistance to apoptosis of glioma cells, while maintaining brain glioma stem cells (BGSCs) stemness.