Gene Score gda Association Type Type Original DB Sentence supporting the association PMID PMID Year
Entrez Id: 5077
Gene Symbol: PAX3
PAX3
0.100 Biomarker disease BEFREE We previously identified the class I-specific histone deacetylase inhibitor, entinostat (ENT), as a pharmacological agent that transcriptionally suppresses the PAX3:FOXO1 tumor-initiating fusion gene found in alveolar rhabdomyosarcoma (aRMS), and we further investigated the mechanism by which ENT suppresses PAX3:FOXO1 oncogene and demonstrated the preclinical efficacy of ENT in RMS orthotopic allograft and patient-derived xenograft (PDX) models. 31113472 2019
Entrez Id: 5077
Gene Symbol: PAX3
PAX3
0.100 GeneticVariation disease BEFREE OLIG2 is a novel immunohistochemical marker associated with the presence of PAX3/7-FOXO1 translocation in rhabdomyosarcomas. 31493794 2019
Entrez Id: 5077
Gene Symbol: PAX3
PAX3
0.100 Biomarker disease BEFREE Together with the observed effects on the PAX3-FOXO1 fusion protein, these data suggest SAHA as a possible therapeutic agent for clinical testing in patients with fusion protein-positive RMS. 30307360 2019
Entrez Id: 5077
Gene Symbol: PAX3
PAX3
0.100 Biomarker disease BEFREE This study describes the in vitro and in vivo activity of PXD-101 (Belinostat), a novel hydroxamic acid-type pan-HDACs inhibitor characterized by a larger safety and efficacy, on myogenic-derived PAX3/FOXO1 fusion protein positive (RH30) or negative (RD) expressing rhabdomyosarcoma (RMS) cell lines. 31325529 2019
Entrez Id: 5077
Gene Symbol: PAX3
PAX3
0.100 Biomarker disease BEFREE The fusion protein (FOXO1-PAX3/7) retains both binding domains and becomes a novel and potent transcriptional regulator in rhabdomyosarcoma subtypes. 31480361 2019
Entrez Id: 5077
Gene Symbol: PAX3
PAX3
0.100 Biomarker disease BEFREE This is supported in rhabdomyosarcoma models by characterization of molecular and phenotypic effects of reducing and inhibiting PLK1, including changes to the PAX3-FOXO1 fusion protein. 31824851 2019
Entrez Id: 5077
Gene Symbol: PAX3
PAX3
0.100 Biomarker disease BEFREE The HDAC3-SMARCA4-miR-27a axis promotes expression of the <i>PAX3:FOXO1</i> fusion oncogene in rhabdomyosarcoma. 30459282 2018
Entrez Id: 5077
Gene Symbol: PAX3
PAX3
0.100 AlteredExpression disease BEFREE In this article, we review the recent understanding of PAX3-FOXO1 as a transcription factor in the pathogenesis of this cancer and discuss recent developments to target this oncoprotein for treatment of RMS. 30373318 2018
Entrez Id: 5077
Gene Symbol: PAX3
PAX3
0.100 Biomarker disease BEFREE Our novel zebrafish rhabdomyosarcoma model identifies a new <i>PAX3-FOXO1</i> target, <i>her3</i>/<i>HES3</i>, that contributes to impaired myogenic differentiation and has prognostic significance in human disease. 29869612 2018
Entrez Id: 5077
Gene Symbol: PAX3
PAX3
0.100 Biomarker disease BEFREE Pediatric paired box 3:forkhead box protein O1 fusion-negative (PF-) rhabdomyosarcoma (RMS) represents a diverse spectrum of tumors with marked differences in histology, myogenic differentiation, and clinical behavior. 29461635 2018
Entrez Id: 5077
Gene Symbol: PAX3
PAX3
0.100 Biomarker disease BEFREE Interleukin-24 (IL24) Is Suppressed by PAX3-FOXO1 and Is a Novel Therapy for Rhabdomyosarcoma. 30190424 2018
Entrez Id: 5077
Gene Symbol: PAX3
PAX3
0.100 Biomarker disease BEFREE We identified novel ALK fusions in a neuroblastoma (BEND5-ALK) and an astrocytoma (PPP1CB-ALK), novel BRAF fusions in an astrocytoma (BCAS1-BRAF) and a ganglioglioma (TMEM106B-BRAF), and a novel PAX3-GLI2 fusion in a rhabdomyosarcoma. 28069802 2017
Entrez Id: 5077
Gene Symbol: PAX3
PAX3
0.100 Biomarker disease BEFREE Rhabdomyosarcomas (RMS) in children and adolescents are heterogeneous sarcomas broadly defined by skeletal muscle features and the presence/absence of PAX3/7-FOXO1 fusion genes. 27984116 2017
Entrez Id: 5077
Gene Symbol: PAX3
PAX3
0.100 Biomarker disease BEFREE We find that PAX3-FOXO1 establishes a myoblastic super enhancer landscape and creates a profound subtype-unique dependence on BET bromodomains, the inhibition of which ablates PAX3-FOXO1 function, providing a mechanistic rationale for exploring BET inhibitors for patients bearing PAX-fusion rhabdomyosarcoma.<i>Cancer Discov; 7(8); 884-99. 28446439 2017
Entrez Id: 5077
Gene Symbol: PAX3
PAX3
0.100 Biomarker disease BEFREE Regulatory landscape fusion in rhabdomyosarcoma through interactions between the PAX3 promoter and FOXO1 regulatory elements. 28615069 2017
Entrez Id: 5077
Gene Symbol: PAX3
PAX3
0.100 Biomarker disease BEFREE Building on previous work, an inducible model in human myoblast cells was used to show that PAX3-FOXO1 and MYCN can initiate rhabdomyosarcoma development but, contrary to current thinking, tumour recurrences occasionally arose independent of the fusion protein. 28378394 2017
Entrez Id: 5077
Gene Symbol: PAX3
PAX3
0.100 Biomarker disease BEFREE PAX3-FOXO1 is essential for tumour initiation and maintenance but not recurrence in a human myoblast model of rhabdomyosarcoma. 28138962 2017
Entrez Id: 5077
Gene Symbol: PAX3
PAX3
0.100 AlteredExpression disease BEFREE In this study, we report overexpression of the nuclear receptor NR4A1 in rhabdomyosarcomas that is sufficient to drive high expression of PAX3-FOXO1A there. 27864345 2017
Entrez Id: 5077
Gene Symbol: PAX3
PAX3
0.100 Biomarker disease BEFREE Previous studies, including our own, have shown prognostic value for the presence of PAX3/7-FOXO1 fusion genes in rhabdomyosarcoma (RMS). 28035744 2017
Entrez Id: 5077
Gene Symbol: PAX3
PAX3
0.100 Biomarker disease BEFREE For treatment stratification, RMS is further subclassified as fusion-positive (FP-RMS) or fusion-negative (FN-RMS), depending on whether a gene fusion involving PAX3 or PAX7 is present or not. 26482321 2016
Entrez Id: 5077
Gene Symbol: PAX3
PAX3
0.100 GeneticVariation disease BEFREE The current molecular classification identifies 2 major subsets, those harboring the fused Pax3-Foxo1 transcription factor generating from a recurrent specific translocation (fusion-positive RMS), and those lacking this signature but harboring mutations in the RAS/PI3K/AKT signaling axis (fusion-negative RMS). 26209235 2016
Entrez Id: 5077
Gene Symbol: PAX3
PAX3
0.100 Biomarker disease BEFREE Inhibition of HO-1 by tin protoporphyrin (SnPP) or siRNA downregulated Pax3/7-FoxO1 target genes and induced a myogenic program in RMS. 27488535 2016
Entrez Id: 5077
Gene Symbol: PAX3
PAX3
0.100 AlteredExpression disease BEFREE In addition, the seven chimeric RNAs that follow the exact transient expression pattern as PAX3-FOXO1 are specific to rhabdomyosarcoma cells. 27799565 2016
Entrez Id: 5077
Gene Symbol: PAX3
PAX3
0.100 Biomarker disease BEFREE The PAX3-FOXO1 fusion protein present in rhabdomyosarcoma interferes with normal FOXO activity and the TGF-β pathway. 25806826 2015
Entrez Id: 5077
Gene Symbol: PAX3
PAX3
0.100 Biomarker disease BEFREE CDK4 is preclinically validated as a therapeutic target in PAX3-FOXO1 fusion gene-positive rhabdomyosarcomas. 26199390 2015