The transforming mechanism of Small Round Cell sarcomas

Small Round Cell Tumors (SRCTs) are a heterogenous class of sarcomas driven by various genomic translocations. These translocations include EWSR1-ETS, EWSR1-WT1, BCOR-CCNB3 and CIC-DUX alterations. As part of our core program, the establishment of pediatric cancer organoids, we have developed novel patient derived in-vitro models (tumoroids) for the entities mentioned, and are using these to study SRCT pathophysiology and discover novel drug vulnerabilities.

In this project, CRISPR/CAS technologies will be used to tag the different fusion proteins with degron- and/or epitope tags. The generated recombinant lines will be pivotal in subsequent protein and chromatin immunoprecipitation (ChIP). Combined results will give insight in the interactome of the transforming fusion proteins and may direct novel strategies to treat these diseases.