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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

[36–39]

 

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

Onco-suppressor

Regulates differentiation

[51]

 

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]