Review Article

PAX3: A Molecule with Oncogenic or Tumor Suppressor Function Is Involved in Cancer

Table 1

Pax3 transcription factor plays a dual role in various types of cancers.

Cancer TypeEffectsRole of PAX3

RhabdomyosarcomaOncogenic effects PAX3-FKHR can induce cellular transformation and prevent apoptosis by altering the MET, PTEN or AP2 signalling pathways [55, 57, 58, 87, 89, 90, 92].
MelanomaRegulating the expression of a variety of melanocytic genes, followed by a decrease in MITF mRNA and gene promoter activity and progression genes SCF, TGF-β, MUC18, RhoC and TIMP [84, 85]
NeuroblastomaInhibition of PAX3 expression significantly decreased the attachment of S-type SH-EP1 cells to extra-cellular matrix proteins, fibronectin, laminin and collagen IV and therefore downregulation of PAX3 reduces migration and invasion [31].
GlioblastomaPAX3 was upregulated in the glioma SHG-44 cells promoted tumor formation in vivo cell lines, while its knockdown resulted in decreased cell proliferation, invasion and induced apoptosis in these cells. PAX3 expression in glioblastoma U-87MG cells suppressed tumorigenicity [30].
Gastric cancerMiR-206 significantly suppressed gastric cancer cell invasion and metastasis both in vitro and in vivo by down regulating PAX3. In vivo metastasis assay shows that overexpression of PAX3 promotes invasiveness and pulmonary metastasis [35].
Urothelial cancerFOXO3a negatively regulates Twist1 and Y-box–binding protein 1 (YB-1), and positively regulated E-cadherin in With FOXO3a PAX3 acts as a prognostic factor [94].

Thyroid cancerSuppressor PAX3 a tumor suppressor in thyroid cancer, through inhibiting the activities of PI3K/Akt and MAPK signaling pathways and promoting transcription factor FOXO3a.). The ectopic expression of PAX3, up-regulated the expression of ZIC1, a zinc-finger transcription factor, which in turn increases FOXO3a transcriptional activity [36].
RhabdomyosarcomaOverexpression of miR-29 and 206 downregulates the expression of cell cycle genes, induce cell cycle arrest through stabilizing PAX3 [6].
Alveolar rhabdomyosarcomaPAX3-FOXO1 up-regulate Noxa (Promote activation of caspases and apoptosis) [95].