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Table 1 Notch signaling in STS.

From: Notch signaling in pediatric soft tissue sarcomas

Tumor Results Notch componentinvolved Role of Notch signaling Reference
Synovial sarcoma   Notch1, JAG1 and TLEs over-expression Pro-tumor [33]
   TLE1 over-expression Pro-tumor [3639]
  Prevents EGR1 expression
Reduces apoptosis
Promotes cell growth
Favors epigenetic gene repression
TLE1 over-expression Pro-tumor [41]
Ewing's sarcoma Prevents differentiation
Increases cell proliferation
Decreases apoptosis
Supports tumor growth in vivo
Manic Fringe expression
Notch1 ICD over-expression
Regulates differentiation
  In p53 wild-type tumor cells: Induces p53
Blocks cell proliferation
Inhibits soft agar colony formation
JAG1 and HEY1 down-regulated by EWS-FLI1 and Notch3 Onco-suppressor [52]
  Prevents cell proliferation Notch1 and Notch3 Onco-suppressor [54]
Rhabdomyosarcoma Supports cell proliferation
Inhibits apoptosis
Prevents differentiation
HES1 over-expression in RMS primary samples and in cell lines Pro-tumor [68]
  Increases cell migration
Increases cell invasion
Notch2 and HEY1 over-expressed in patients with alveolar RMS and embryonal RMS Pro-tumor [69]
  Increases cell proliferation
Supports tumor growth in vivo
Notch1 ICD and HEY1 over-expressed in primary embryonal RMS and cell lines Pro-tumor [70]
  Prevents differentiation
Supports cell proliferation
Supports tumor growth in vivo
Notch3 ICD and HES1 over-expressed in alveolar RMS and embryonal RMS cell lines Pro-tumor [71]
  Supports cell proliferation and anchorage-independence in vitro and tumor growth in vivo RBP-Jκ over-expressed in embryonal RMS primary tissues and cell lines Pro-tumor [76]