|
Rhabdomyosarcoma
|
0.100 |
GeneticVariation
|
disease |
BEFREE |
Standard cytogenetic analyses and RT-PCR testing for the classic gene rearrangements seen in RMS [t(2;13)-PAX3/FKHR] and EWS ([t(11;22) & t(21;22)-EWS/FLI-1 & EWS/ERG), were used for characterization of the MEM, with gene expression microarray analyses on all tumor types.
|
17431644 |
2007 |
|
Rhabdomyosarcoma
|
0.100 |
Biomarker
|
disease |
BEFREE |
The rapid onset and increased penetrance of tumorigenesis in this model provide a powerful tool for interrogating aRMS biology and screening novel therapeutics.<b>Significance:</b> A novel mouse model sheds light on the critical role of Hippo/MST downregulation in PAX3-FOXO1-positive rhabdomyosarcoma tumorigenesis.<i></i>.
|
30093562 |
2018 |
|
Rhabdomyosarcoma
|
0.100 |
GeneticVariation
|
disease |
BEFREE |
The authors applied reverse transcriptase-polymerase chain reaction analysis (RT-PCR) to analyze tumor samples from 14 children with rhabdomyosarcoma for the presence of the chimeric PAX3-FKHR transcript resulting from the translocation t(2;13)(q35,q14).
|
10718207 |
2000 |
|
Rhabdomyosarcoma
|
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 |
|
Rhabdomyosarcoma
|
0.100 |
Biomarker
|
disease |
BEFREE |
PAX3/7-FOXO1 fusion status in older rhabdomyosarcoma patient population by fluorescent in situ hybridization.
|
22089931 |
2012 |
|
Rhabdomyosarcoma
|
0.100 |
GeneticVariation
|
disease |
BEFREE |
Rhabdomyosarcomas (RMSs) are the most frequent soft tissue sarcoma in children and adolescents, defined by skeletal muscle differentiation and the status of FOXO1 fusions.
|
29898687 |
2018 |
|
Rhabdomyosarcoma
|
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 |
|
Rhabdomyosarcoma
|
0.100 |
Biomarker
|
disease |
BEFREE |
A clinicopathologic study of head and neck rhabdomyosarcomas showing FOXO1 fusion-positive alveolar and MYOD1-mutant sclerosing are associated with unfavorable outcome.
|
27688110 |
2016 |
|
Rhabdomyosarcoma
|
0.100 |
GeneticVariation
|
disease |
BEFREE |
In addition, one of the cases classified as PRMS showed evidence for the presence of a PAX3-FOXO1A fusion gene, which is characteristic of the alveolar subtype of RMS.
|
12550764 |
2003 |
|
Rhabdomyosarcoma
|
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 |
|
Rhabdomyosarcoma
|
0.100 |
Biomarker
|
disease |
BEFREE |
CDK4 is preclinically validated as a therapeutic target in PAX3-FOXO1 fusion gene-positive rhabdomyosarcomas.
|
26199390 |
2015 |
|
Rhabdomyosarcoma
|
0.100 |
Biomarker
|
disease |
BEFREE |
These results indicate that the involvement of PAX3 and PAX3-FKHR in rhabdomyosarcoma tumourigenesis may be through downregulation of PTEN tumour suppressor gene, affecting the PTEN/AKT survival pathway.
|
17350164 |
2007 |
|
Rhabdomyosarcoma
|
0.100 |
Biomarker
|
disease |
BEFREE |
A combined clinical and molecular risk-stratification scheme that incorporated the PAX3/FOXO1 fusion gene status was derived from 287 patients with RMS and evaluated.
|
22454413 |
2012 |
|
Rhabdomyosarcoma
|
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 |
|
Rhabdomyosarcoma
|
0.100 |
Biomarker
|
disease |
BEFREE |
We have determined the presence of the PAX3/7-FKHR fusion genes in 91 primary rhabdomyosarcoma tumours using a combination of classical cytogenetics, FISH and RT-PCR, with a view to determining the clinical characteristics of tumours with and without the characteristic translocations.
|
11556833 |
2001 |
|
Rhabdomyosarcoma
|
0.100 |
GeneticVariation
|
disease |
BEFREE |
Our FISH study highlighted the excellent performance of the presently reported commercial break-apart probe for the detection of FKHR gene rearrangements in RMS.
|
18094995 |
2008 |
|
Rhabdomyosarcoma
|
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 |
|
Rhabdomyosarcoma
|
0.100 |
Biomarker
|
disease |
BEFREE |
FOXO1 gene is fused to PAX3 or PAX7 genes in rhabdomyosarcoma.
|
15492844 |
2004 |
|
Rhabdomyosarcoma
|
0.100 |
Biomarker
|
disease |
BEFREE |
Moreover, Notch3 depletion reduces PAX3-FOXO1 alveolar RMS tumor growth in vivo.
|
24797362 |
2014 |
|
Rhabdomyosarcoma
|
0.100 |
Biomarker
|
disease |
BEFREE |
In this investigation, we selected PAX3/FKHR and PAX7/FKHR fusion transcript-positive and -negative alveolar rhabdomyosarcomas (ARMSs) and embryonal rhabdomyosarcomas (ERMSs) with and without anaplastic features, to ascertain genomic imbalance differences and/or similarities within these histopathologic and genetic rhabdomyosarcoma (RMS) variants.
|
11807989 |
2002 |
|
Rhabdomyosarcoma
|
0.100 |
AlteredExpression
|
disease |
LHGDN |
PAX--FKHR fusion genes and AChR-gamma in Chinese patients with rhabdomyosarcoma: diagnosis using formalin-fixed archival tissues.
|
18988640 |
2009 |
|
Rhabdomyosarcoma
|
0.100 |
AlteredExpression
|
disease |
BEFREE |
Furthermore, transfer of a PAX3-FKHR gene into embryonal RMS cell activates CXCR4 expression.
|
12239174 |
2002 |
|
Rhabdomyosarcoma
|
0.100 |
AlteredExpression
|
disease |
BEFREE |
Furthermore, the finding of PAX3 expression with or without PAX3-FKHR transcript in the great majority of the cases raises the question of whether PAX3 expression could play a role in the pathogenesis of RMS.
|
9546061 |
1998 |
|
Rhabdomyosarcoma
|
0.100 |
GeneticVariation
|
disease |
BEFREE |
The presence or absence of the FOXO1 translocation is one of the most important prognostic factors in RMS.
|
24326270 |
2014 |
|
Rhabdomyosarcoma
|
0.100 |
AlteredExpression
|
disease |
BEFREE |
JARID2 levels were altered by silencing or overexpressing PAX3-FOXO1 in RMS cell lines with and without the fusion gene, respectively.
|
23435416 |
2014 |