Novel patient-derived models of desmoplastic small round cell tumor confirm a targetable dependency on ERBB signaling

• Main Authors: Roger S. Smith (Author), Igor Odintsov (Author), Zebing Liu (Author), Allan Jo-Weng Lui (Author), Takuo Hayashi (Author), Morana Vojnic (Author), Yoshiyuki Suehara (Author), Lukas Delasos (Author), Marissa S. Mattar (Author), Julija Hmeljak (Author), Hillary A. Ramirez (Author), Melissa Shaw (Author), Gabrielle Bui (Author), Alifiani B. Hartono (Author), Eric Gladstone (Author), Siddharth Kunte (Author), Heather Magnan (Author), Inna Khodos (Author), Elisa De Stanchina (Author), Michael P. La Quaglia (Author), Jinjuan Yao (Author), Marick Laé (Author), Sean B. Lee (Author), Lee Spraggon (Author), Christine A. Pratilas (Author), Marc Ladanyi (Author), Romel Somwar (Author)
• Format: Book
• Published: The Company of Biologists, 2022-01-01T00:00:00Z.
• Subjects: article
• Online Access: Connect to this object online.

Desmoplastic small round cell tumor (DSRCT) is characterized by the t(11;22)(p13;q12) translocation, which fuses the transcriptional regulatory domain of EWSR1 with the DNA-binding domain of WT1, resulting in the oncogenic EWSR1-WT1 fusion protein. The paucity of DSRCT disease models has hampered pr...