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Study Description

Desmoplastic Small Round Cell Tumor (DSRCT) is an aggressive mesenchymal tumor driven by fusions between the disordered domain of the Ewing sarcoma RNA binding protein 1 (EWSR1) and the developmental transcription factor Wilms tumor 1 (WT1). We used genome-wide chromatin profiling to identify EWSR1-WT1-dependent gene regulatory networks and target genes. Our studies show that EWS-WT1 is a powerful activator of distal regulatory elements and controls an oncogenic gene expression program that characterizes primary DSRCTs. ChIP-seq profiles for histone marks in primary DSRCT samples are available through dbGaP.

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Study Attribution
  • Principal Investigator
    • Miguel Rivera. Massachusetts General Hospital, Boston, MA, USA.
  • Co-Investigators
    • Nicolo Riggi. University of Lausanne, Lausanne, Switzerland.
    • Gaylor Boulay. Massachusetts General Hospital, Boston, MA, USA.
    • Rui Dong. Massachusetts General Hospital, Boston, MA, USA.
    • Liliane Broye. University of Lausanne, Lausanne, Switzerland.