t(1;13)(p36;q14) PAX3/FOXO1

2018-01-01   Philippe Dessen  , Jean-Loup Huret  

Clinics and Pathology

Disease

-

Genes Involved and Proteins

Gene name
PAX3
Location
2q36.1
Gene name
FOXO1
Location
13q14.11

Highly cited references

Pubmed IDYearTitleCitations
373451592023Genomic and Epigenetic Changes Drive Aberrant Skeletal Muscle Differentiation in Rhabdomyosarcoma.214
234327282013A call to ARMS: targeting the PAX3-FOXO1 gene in alveolar rhabdomyosarcoma.196
303733182018Therapeutic Approaches Targeting PAX3-FOXO1 and Its Regulatory and Transcriptional Pathways in Rhabdomyosarcoma.175
262092352016Uncovering metabolism in rhabdomyosarcoma.145
358576432022Targeting KDM4 for treating PAX3-FOXO1-driven alveolar rhabdomyosarcoma.110
379682772023PAX3-FOXO1 dictates myogenic reprogramming and rhabdomyosarcoma identity in endothelial progenitors.104
391072802024HDAC3 genetic and pharmacologic inhibition radiosensitizes fusion positive rhabdomyosarcoma by promoting DNA double-strand breaks.98
331825562020FAK Signaling in Rhabdomyosarcoma.97
348369712021BAF complexes drive proliferation and block myogenic differentiation in fusion-positive rhabdomyosarcoma.93
358793662022Therapeutic targeting of ATR in alveolar rhabdomyosarcoma.86
346390122021MS-275 (Entinostat) Promotes Radio-Sensitivity in PAX3-FOXO1 Rhabdomyosarcoma Cells.85
286150692017Regulatory landscape fusion in rhabdomyosarcoma through interactions between the PAX3 promoter and FOXO1 regulatory elements.85
376395932023Myo-differentiation reporter screen reveals NF-Y as an activator of PAX3-FOXO1 in rhabdomyosarcoma.83
389920402024The transcriptional co-repressor Runx1t1 is essential for MYCN-driven neuroblastoma tumorigenesis.82
33490904202117-DMAG dually inhibits Hsp90 and histone lysine demethylases in alveolar rhabdomyosarcoma.81
336277852021FOXF1 is required for the oncogenic properties of PAX3-FOXO1 in rhabdomyosarcoma.80
347205292021Transcription factors specificity protein and nuclear receptor 4A1 in pancreatic cancer.80
293677562018PAX3-FOXO1 drives miR-486-5p and represses miR-221 contributing to pathogenesis of alveolar rhabdomyosarcoma.78
243344542014Alveolar rhabdomyosarcoma-associated PAX3-FOXO1 promotes tumorigenesis via Hippo pathway suppression.73
277600492016Helicase CHD4 is an epigenetic coregulator of PAX3-FOXO1 in alveolar rhabdomyosarcoma.73
331176712020Effects of the Oncoprotein PAX3-FOXO1 on Modulation of Exosomes Function and Protein Content: Implications on Oxidative Stress Protection and Enhanced Plasticity.70
384740362024Transforming Growth Factor Beta and Alveolar Rhabdomyosarcoma: A Challenge of Tumor Differentiation and Chemotherapy Response.68
345733552021High Frequency of Tumor Propagating Cells in Fusion-Positive Rhabdomyosarcoma.61
384022122024KDM3B inhibitors disrupt the oncogenic activity of PAX3-FOXO1 in fusion-positive rhabdomyosarcoma.60
284464392017PAX3-FOXO1 Establishes Myogenic Super Enhancers and Confers BET Bromodomain Vulnerability.60
250306972014Lineage of origin in rhabdomyosarcoma informs pharmacological response.60
363957712022PAX3-FOXO1 coordinates enhancer architecture, eRNA transcription, and RNA polymerase pause release at select gene targets.59
184579142008PAX3-FOXO1 fusion gene in rhabdomyosarcoma.59
398120072025DNA methylation patterns are influenced by Pax3::Foxo1 expression and developmental lineage in rhabdomyosarcoma tumours forming in genetically engineered mouse models.58
260714852015Secreted Frizzled-Related Protein 3 (SFRP3) Is Required for Tumorigenesis of PAX3-FOXO1-Positive Alveolar Rhabdomyosarcoma.57
298696122018PAX3-FOXO1 transgenic zebrafish models identify HES3 as a mediator of rhabdomyosarcoma tumorigenesis.56
318248512019A Perspective on Polo-Like Kinase-1 Inhibition for the Treatment of Rhabdomyosarcomas.55
179866082007PAX3-FOXO1 controls expression of the p57Kip2 cell-cycle regulator through degradation of EGR1.54
315783742019The PAX3-FOXO1 oncogene alters exosome miRNA content and leads to paracrine effects mediated by exosomal miR-486.54
274540802016Comparative transcriptomic analysis reveals the oncogenic fusion protein PAX3-FOXO1 globally alters mRNA and miRNA to enhance myoblast invasion.54
331825482020Synthetic MIR143-3p Suppresses Cell Growth in Rhabdomyosarcoma Cells by Interrupting RAS Pathways Including PAX3-FOXO1.54
258068262015The PAX3-FOXO1 fusion protein present in rhabdomyosarcoma interferes with normal FOXO activity and the TGF-β pathway.53
326970142020KDM3A/Ets1 epigenetic axis contributes to PAX3/FOXO1-driven and independent disease-promoting gene expression in fusion-positive Rhabdomyosarcoma.53
381021362023PAX3-FOXO1 uses its activation domain to recruit CBP/P300 and shape RNA Pol2 cluster distribution.52
377329052023Piperacetazine Directly Binds to the PAX3::FOXO1 Fusion Protein and Inhibits Its Transcriptional Activity.52
341879332021Negative correlation of single-cell PAX3:FOXO1 expression with tumorigenicity in rhabdomyosarcoma.51
280588502017Molecular diagnostics in the management of rhabdomyosarcoma.51
333388752021Mutant myogenin promoter-controlled oncolytic adenovirus selectively kills PAX3-FOXO1-positive rhabdomyosarcoma cells.51
335895192021A Fusion Transcription Factor-Driven Cancer Progresses to a Fusion-Independent Relapse via Constitutive Activation of a Downstream Transcriptional Target.51
256591242015Modeling of the human alveolar rhabdomyosarcoma Pax3-Foxo1 chromosome translocation in mouse myoblasts using CRISPR-Cas9 nuclease.50
360971782022Strategies to inhibit FGFR4 V550L-driven rhabdomyosarcoma.50
199049782009Phosphorylation of serine 205 by the protein kinase CK2 persists on Pax3-FOXO1, but not Pax3, throughout early myogenic differentiation.50
356049322022Divergent transcriptional and transforming properties of PAX3-FOXO1 and PAX7-FOXO1 paralogs.49
301904242018Interleukin-24 (IL24) Is Suppressed by PAX3-FOXO1 and Is a Novel Therapy for Rhabdomyosarcoma.48
258219472015Inhibiting phosphorylation of the oncogenic PAX3-FOXO1 reduces alveolar rhabdomyosarcoma phenotypes identifying novel therapy options.47
335092642021ACTA1 is inhibited by PAX3-FOXO1 through RhoA-MKL1-SRF signaling pathway and impairs cell proliferation, migration and tumor growth in Alveolar Rhabdomyosarcoma.44
394488672025Dependence of PAX3-FOXO1 chromatin occupancy on ETS1 at important disease-promoting genes exposes new targetable vulnerability in Fusion-Positive Rhabdomyosarcoma.44
241074482013Small molecule inhibition of PAX3-FOXO1 through AKT activation suppresses malignant phenotypes of alveolar rhabdomyosarcoma.44
288830172017NFκB signaling in alveolar rhabdomyosarcoma.43
237330152013Identification of target genes of PAX3-FOXO1 in alveolar rhabdomyosarcoma.40
238144862013PAX3-FOXO1 induces up-regulation of Noxa sensitizing alveolar rhabdomyosarcoma cells to apoptosis.39
259852102015The oncogenic role of the cochaperone Sgt1.39
305932632019PAX3-FOXO1 escapes miR-495 regulation during muscle differentiation.38
345521552021Reduced B7-H3 expression by PAX3-FOXO1 knockdown inhibits cellular motility and promotes myogenic differentiation in alveolar rhabdomyosarcoma.38
298618642018PPP2R1A regulated by PAX3/FOXO1 fusion contributes to the acquisition of aggressive behavior in PAX3/FOXO1-positive alveolar rhabdomyosarcoma.35
376270612023Preclinical Evaluation of the FGFR-Family Inhibitor Futibatinib for Pediatric Rhabdomyosarcoma.35
281389622017PAX3-FOXO1 is essential for tumour initiation and maintenance but not recurrence in a human myoblast model of rhabdomyosarcoma.33
304592822018The HDAC3-SMARCA4-miR-27a axis promotes expression of the PAX3:FOXO1 fusion oncogene in rhabdomyosarcoma.33
324165892020Miswired Enhancer Logic Drives a Cancer of the Muscle Lineage.33
321009352020Pediatric rhabdomyosarcoma with bone marrow metastasis.32
220899312012PAX3/7-FOXO1 fusion status in older rhabdomyosarcoma patient population by fluorescent in situ hybridization.28
214400832011Identification of serines 201 and 209 as sites of Pax3 phosphorylation and the altered phosphorylation status of Pax3-FOXO1 during early myogenic differentiation.28
371463022022Rhabdomyosarcomas are oncogene addicted to the activation of AVIL.27
332991092021Immunohistochemical detection of PAX-FOXO1 fusion proteins in alveolar rhabdomyosarcoma using breakpoint specific monoclonal antibodies.25
391478202024Entinostat as a combinatorial therapeutic for rhabdomyosarcoma.24
234691532013Cyclin-dependent kinase 4 phosphorylates and positively regulates PAX3-FOXO1 in human alveolar rhabdomyosarcoma cells.23
258839052015The case for primary salivary rhabdomyosarcoma.16
385810352024TRIB3 silencing promotes the downregulation of Akt pathway and PAX3-FOXO1 in high-risk rhabdomyosarcoma.12
263558932016Alternate PAX3-FOXO1 oncogenic fusion in biphenotypic sinonasal sarcoma.11
291462052018PAX3-FOXO1: Zooming in on an "undruggable" target.0
260087532015Prognostic value of PAX3/7-FOXO1 fusion status in alveolar rhabdomyosarcoma: Systematic review and meta-analysis.0
384770902023Biphenotypic sinonasal sarcoma with PAX3/FOXO1 fusion.0
274533502016P/CAF mediates PAX3-FOXO1-dependent oncogenesis in alveolar rhabdomyosarcoma.0
389967062024Anlotinib destabilizes PAX3-FOXO1 to induce rhabdomyosarcoma cell death via upregulating NEK2.0
398682472025PAX3-FOXO1 drives targetable cell state-dependent metabolic vulnerabilities in rhabdomyosarcoma.0
218822542012PAX3-FOXO1 and FGFR4 in alveolar rhabdomyosarcoma.0
354656632022Dentithecamides A-H, Diacylated Zoanthoxanthin Derivatives with PAX3-FOXO1 Inhibitory Activity from the Hydroid Dentitheca habereri.0
349804432022[Alveolar rhabdomyosarcoma: Two fusion-negative cases lacking PAX3-FOXO1 and PAX7-FOXO1].0
253984392015PLK1 phosphorylates PAX3-FOXO1, the inhibition of which triggers regression of alveolar Rhabdomyosarcoma.0
388851482024Targeted immunotherapy and nanomedicine for rhabdomyosarcoma: The way of the future.0
220378682011PAX3-FOXO1 induces cannabinoid receptor 1 to enhance cell invasion and metastasis.0
328008272021Gene Fusions in Ocular Adnexal Tumors.0
319895082020Signaling pathways in Rhabdomyosarcoma invasion and metastasis.0
236457522013Regulation of target genes of PAX3-FOXO1 in alveolar rhabdomyosarcoma.0
318888892020Aurora A Kinase Inhibition Destabilizes PAX3-FOXO1 and MYCN and Synergizes with Navitoclax to Induce Rhabdomyosarcoma Cell Death.0
272613352016RGD liposome-protamine-siRNA (LPR) nanoparticles targeting PAX3-FOXO1 for alveolar rhabdomyosarcoma therapy.0
224544132012PAX3/FOXO1 fusion gene status is the key prognostic molecular marker in rhabdomyosarcoma and significantly improves current risk stratification.0
390712992024Rhabdomyosarcoma fusion oncoprotein initially pioneers a neural signature in vivo.0
367220032023PAX-FOXO1 fusion status in children and adolescents with alveolar rhabdomyosarcoma: Impact on clinical, pathological, and survival features.0
385267902024CRISPR-Cas9-Mediated Bioluminescent Tagging of Endogenous Proteins by Fluorescent Protein-Assisted Cell Sorting.0
184140342008Guilt by association: PAX3-FOXO1 regulates gene expression through selective destabilization of the EGR1 transcription factor.0
360448552022The RNA helicase DDX5 cooperates with EHMT2 to sustain alveolar rhabdomyosarcoma growth.0
392110842024PAX fusion proteins deregulate gene networks controlling mitochondrial translation in pediatric rhabdomyosarcoma.0
292777582018Sphingosine Induces Apoptosis and Down-regulation of MYCN in PAX3-FOXO1-positive Alveolar Rhabdomyosarcoma Cells Irrespective of TP53 Mutation.0
342182272022A Review of Effusion Cytomorphology of Small Round Cell Tumors.0

Bibliography

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Summary

Fusion gene

PAX3/FOXO1 PAX3 (2q36.1) FOXO1 (13q14.11)

Note

Non-annotated chromosomal abnormality. Preliminary data :
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Citation

Philippe Dessen ; Jean-Loup Huret

t(1;13)(p36;q14) PAX3/FOXO1

Atlas Genet Cytogenet Oncol Haematol. 2018-01-01

Online version: http://atlasgeneticsoncology.org/haematological/3054/t(1;13)(p36;q14)-pax3-foxo1