Childhood Alveolar Rhabdomyosarcoma
|
0.300 |
GeneticVariation
|
disease |
BEFREE |
Alveolar rhabdomyosarcoma (RMS) is associated with an underlying pathogenic translocation involving either PAX3 or PAX7 and FOXO1.
|
31299267 |
2019 |
Childhood Alveolar Rhabdomyosarcoma
|
0.300 |
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 |
Childhood Alveolar Rhabdomyosarcoma
|
0.300 |
Biomarker
|
disease |
BEFREE |
<i>PPP2R1A</i> regulated by PAX3/FOXO1 fusion contributes to the acquisition of aggressive behavior in PAX3/FOXO1-positive alveolar rhabdomyosarcoma.
|
29861864 |
2018 |
Childhood Alveolar Rhabdomyosarcoma
|
0.300 |
AlteredExpression
|
disease |
BEFREE |
In this review, we will discuss the current knowledge regarding potential therapeutic targets that might contribute to indirect interference with PAX3-FOXO1 activity in alveolar rhabdomyosarcoma at the different molecular levels and extrapolate these findings to fusion transcription factors in general.
|
29146205 |
2018 |
Childhood Alveolar Rhabdomyosarcoma
|
0.300 |
Biomarker
|
disease |
BEFREE |
Somatic genetic rearrangements that juxtapose the PAX3 DNA binding domain to the transcriptional activation domain of other transcription factors deregulate PAX3 function and contribute to the pathogenesis of the soft tissue cancers alveolar rhabdomyosarcoma and biphenotypic sinonasal sarcoma.
|
29730428 |
2018 |
Childhood Alveolar Rhabdomyosarcoma
|
0.300 |
Biomarker
|
disease |
BEFREE |
PAX3-FOXO1 drives miR-486-5p and represses miR-221 contributing to pathogenesis of alveolar rhabdomyosarcoma.
|
29367756 |
2018 |
Childhood Alveolar Rhabdomyosarcoma
|
0.300 |
GeneticVariation
|
disease |
BEFREE |
Alveolar rhabdomyosarcoma (aRMS) is a pediatric soft tissue cancer commonly associated with a chromosomal translocation that leads to the expression of a Pax3:Foxo1 or Pax7:Foxo1 fusion protein, the developmental underpinnings of which may give clues to its therapeutic approaches.
|
28883017 |
2017 |
Childhood Alveolar Rhabdomyosarcoma
|
0.300 |
Biomarker
|
disease |
BEFREE |
The chromosomal translocation that leads to alveolar rhabdomyosarcoma development generates a novel TAD that is likely to favour ectopic PAX3:FOXO1 oncogene activation in non-PAX3 territories.
|
28615069 |
2017 |
Childhood Alveolar Rhabdomyosarcoma
|
0.300 |
Biomarker
|
disease |
BEFREE |
The presence of PAX3-FOXO1 in SNS and alveolar rhabdomyosarcoma suggests that these two entities are genetically similar lesions arising from distinct progenitor cell pools.
|
26355893 |
2016 |
Childhood Alveolar Rhabdomyosarcoma
|
0.300 |
Biomarker
|
disease |
BEFREE |
RGD liposome-protamine-siRNA (LPR) nanoparticles targeting PAX3-FOXO1 for alveolar rhabdomyosarcoma therapy.
|
27261335 |
2016 |
Childhood Alveolar Rhabdomyosarcoma
|
0.300 |
AlteredExpression
|
disease |
BEFREE |
Therefore, this work identifies CHD4 as an epigenetic coregulator of PAX3-FOXO1 activity, providing rational evidence for CHD4 as a potential therapeutic target in aRMS.
|
27760049 |
2016 |
Childhood Alveolar Rhabdomyosarcoma
|
0.300 |
Biomarker
|
disease |
BEFREE |
In conclusion, we report a novel PAX3-NCOA1 fusion in BSNS, which appears to be associated with focal rhabdomyoblastic differentiation and should be distinguished from PAX3-NCOA1-positive alveolar rhabdomyosarcoma or malignant Triton tumor.
|
26371783 |
2016 |
Childhood Alveolar Rhabdomyosarcoma
|
0.300 |
GeneticVariation
|
disease |
BEFREE |
The reciprocal t(2;13)(q36.1;q14.1) in human alveolar rhabdomyosarcoma (A-RMS) creates a pathognomonic PAX3-FOXO1 fusion gene.
|
25659124 |
2015 |
Childhood Alveolar Rhabdomyosarcoma
|
0.300 |
Biomarker
|
disease |
BEFREE |
Taken together, these preclinical studies validate the PLK1-PAX3-FOXO1 axis as a rational target to treat aRMS.
|
25398439 |
2015 |
Childhood Alveolar Rhabdomyosarcoma
|
0.300 |
Biomarker
|
disease |
BEFREE |
Prognostic value of PAX3/7-FOXO1 fusion status in alveolar rhabdomyosarcoma: Systematic review and meta-analysis.
|
26008753 |
2015 |
Childhood Alveolar Rhabdomyosarcoma
|
0.300 |
Biomarker
|
disease |
BEFREE |
Alveolar rhabdomyosarcoma-associated PAX3-FOXO1 promotes tumorigenesis via Hippo pathway suppression.
|
24334454 |
2014 |
Childhood Alveolar Rhabdomyosarcoma
|
0.300 |
GeneticVariation
|
disease |
BEFREE |
Therapeutic cytodifferentiation in alveolar rhabdomyosarcoma without genetic change of the PAX3-FKHR chimeric fusion gene: a case study.
|
23797277 |
2013 |
Childhood Alveolar Rhabdomyosarcoma
|
0.300 |
Biomarker
|
disease |
BEFREE |
Identification of target genes of PAX3-FOXO1 in alveolar rhabdomyosarcoma.
|
23733015 |
2013 |
Childhood Alveolar Rhabdomyosarcoma
|
0.300 |
Biomarker
|
disease |
BEFREE |
P-cadherin is a direct PAX3-FOXO1A target involved in alveolar rhabdomyosarcoma aggressiveness.
|
22710718 |
2013 |
Childhood Alveolar Rhabdomyosarcoma
|
0.300 |
Biomarker
|
disease |
BEFREE |
A call to ARMS: targeting the PAX3-FOXO1 gene in alveolar rhabdomyosarcoma.
|
23432728 |
2013 |
Childhood Alveolar Rhabdomyosarcoma
|
0.300 |
Biomarker
|
disease |
BEFREE |
Regulation of target genes of PAX3-FOXO1 in alveolar rhabdomyosarcoma.
|
23645752 |
2013 |
Childhood Alveolar Rhabdomyosarcoma
|
0.300 |
Biomarker
|
disease |
BEFREE |
PAX3-FOXO1 and FGFR4 in alveolar rhabdomyosarcoma.
|
21882254 |
2012 |
Childhood Alveolar Rhabdomyosarcoma
|
0.300 |
GeneticVariation
|
disease |
BEFREE |
Alveolar rhabdomyosarcoma (RMS) is an aggressive pediatric cancer of the myogenic lineage with frequent chromosomal translocations involving the PAX3 or PAX7 and FOXO1 genes.
|
22447499 |
2012 |
Childhood Alveolar Rhabdomyosarcoma
|
0.300 |
GeneticVariation
|
disease |
BEFREE |
Alveolar rhabdomyosarcoma (ARMS) is characterized by the t(2;13) or t(1;13) chromosomal translocations, which generate the PAX3-FOXO1 or PAX7-FOXO1 fusion genes, respectively.
|
21177767 |
2011 |
Childhood Alveolar Rhabdomyosarcoma
|
0.300 |
GeneticVariation
|
disease |
BEFREE |
One example for such a tumor type is alveolar rhabdomyosarcoma (aRMS), which is characterized by a specific translocation creating the oncogenic PAX3/FKHR transcription factor, believed to be the molecular basis of the disease.
|
20453878 |
2010 |