entrectinib and Desmoplastic-Small-Round-Cell-Tumor
entrectinib has been researched along with Desmoplastic-Small-Round-Cell-Tumor* in 1 studies
Other Studies
1 other study(ies) available for entrectinib and Desmoplastic-Small-Round-Cell-Tumor
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Therapeutic Potential of NTRK3 Inhibition in Desmoplastic Small Round Cell Tumor.
Desmoplastic small round cell tumor (DSRCT) is a highly lethal intra-abdominal sarcoma of adolescents and young adults. DSRCT harbors a t(11;22)(p13:q12) that generates the EWSR1-WT1 chimeric transcription factor, the key oncogenic driver of DSRCT. EWSR1-WT1 rewires global gene expression networks and activates aberrant expression of targets that together mediate oncogenesis. EWSR1-WT1 also activates a neural gene expression program.. Among these neural markers, we found prominent expression of neurotrophic tyrosine kinase receptor 3 (NTRK3), a druggable receptor tyrosine kinase. We investigated the regulation of NTRK3 by EWSR1-WT1 and its potential as a therapeutic target. We found that EWSR1-WT1 binds upstream of. Our results indicate that EWSR1-WT1 directly activates NTRK3 expression in DSRCT cells, which are dependent on its expression and activity for growth. Pharmacologic inhibition of NTRK3 by entrectinib significantly reduces growth of DSRCT cells both Topics: Adolescent; Adult; Animals; Benzamides; Cell Line, Tumor; Child; Desmoplastic Small Round Cell Tumor; Female; Gene Expression Regulation, Neoplastic; Humans; Indazoles; Male; Mice; Oncogene Proteins, Fusion; Receptor, trkC; RNA-Binding Protein EWS; WT1 Proteins; Xenograft Model Antitumor Assays; Young Adult | 2021 |