Childhood Alveolar Rhabdomyosarcoma
|
0.300 |
Biomarker
|
disease |
BEFREE |
In vivo amplification of the PAX3-FKHR and PAX7-FKHR fusion genes in alveolar rhabdomyosarcoma.
|
8789435 |
1996 |
Childhood Alveolar Rhabdomyosarcoma
|
0.300 |
Biomarker
|
disease |
BEFREE |
Effects of PAX3-FKHR on malignant phenotypes in alveolar rhabdomyosarcoma.
|
18022385 |
2008 |
Childhood Alveolar Rhabdomyosarcoma
|
0.300 |
Biomarker
|
disease |
BEFREE |
These assays detect PAX3-FKHR and PAX7-FKHR chimeric transcripts in alveolar rhabdomyosarcoma, EWS-FLI1 and EWS-ERG chimeric transcripts in Ewing's sarcoma, and EWS-WT1 chimeric transcripts in desmoplastic small round cell tumor.
|
7530783 |
1995 |
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 |
GeneticVariation
|
disease |
BEFREE |
PAX3-FKHR results from a t(2,13) chromosomal translocation, a unique genetic marker of alveolar rhabdomyosarcoma.
|
12401804 |
2003 |
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 |
Biomarker
|
disease |
BEFREE |
RGD liposome-protamine-siRNA (LPR) nanoparticles targeting PAX3-FOXO1 for alveolar rhabdomyosarcoma therapy.
|
27261335 |
2016 |
Childhood Alveolar Rhabdomyosarcoma
|
0.300 |
GeneticVariation
|
disease |
BEFREE |
In alveolar rhabdomyosarcoma, a (2;13)(q35;qt4) translocation is associated with a chimeric gene between PAX3 and FKHR.
|
8875708 |
1996 |
Childhood Alveolar Rhabdomyosarcoma
|
0.300 |
Biomarker
|
disease |
BEFREE |
These results suggest that Pax3 may suppress the terminal differentiation of migrating limb myoblasts and that the PAX3-forkhead fusion may contribute to the phenotype of alveolar rhabdomyosarcoma by preventing terminal differentiation.
|
7744814 |
1995 |
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 |
GeneticVariation
|
disease |
BEFREE |
We report the first mouse model of alveolar rhabdomyosarcoma using a conditional Pax3:Fkhr knock-in allele whose activation in late embryogenesis and postnatally is targeted to terminally differentiating Myf6-expressing skeletal muscle.
|
15489287 |
2004 |
Childhood Alveolar Rhabdomyosarcoma
|
0.300 |
GeneticVariation
|
disease |
BEFREE |
Alveolar rhabdomyosarcoma is characterized by a t(2;13)(q35;q14) chromosome translocation, which leads to the fusion of the PAX3 and the FKHR genes.
|
9294613 |
1997 |
Childhood Alveolar Rhabdomyosarcoma
|
0.300 |
AlteredExpression
|
disease |
BEFREE |
Thus, gene-specific mechanisms were selected to overexpress PAX3-FKHR and PAX7-FKHR in alveolar rhabdomyosarcoma, presumably due to differences in regulation between the wild-type loci.
|
9223312 |
1997 |
Childhood Alveolar Rhabdomyosarcoma
|
0.300 |
Biomarker
|
disease |
BEFREE |
PAX-3-FORKHEAD chimeric mRNAs were amplified by PCR in 8 of 15 cases of alveolar rhabdomyosarcoma.
|
8943815 |
1996 |
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 |
Fusion of a fork head domain gene to PAX3 in the solid tumour alveolar rhabdomyosarcoma.
|
8275086 |
1993 |
Childhood Alveolar Rhabdomyosarcoma
|
0.300 |
AlteredExpression
|
disease |
BEFREE |
The t(2;13) translocation of alveolar rhabdomyosarcoma results in tumor-specific expression of a chimeric transcription factor containing the N-terminal DNA-binding domain of PAX3 and the C-terminal transactivation domain of FKHR.
|
8643596 |
1996 |
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 |
Childhood Alveolar Rhabdomyosarcoma
|
0.300 |
Biomarker
|
disease |
BEFREE |
Gene expression profiling identifies potential relevant genes in alveolar rhabdomyosarcoma pathogenesis and discriminates PAX3-FKHR positive and negative tumors.
|
16381018 |
2006 |
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 |
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 |
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 |
MGD |
|
|
|
Childhood Alveolar Rhabdomyosarcoma
|
0.300 |
GeneticVariation
|
disease |
BEFREE |
Structural analysis of PAX3 genomic rearrangements in alveolar rhabdomyosarcoma.
|
9530337 |
1998 |
Childhood Alveolar Rhabdomyosarcoma
|
0.300 |
Biomarker
|
disease |
BEFREE |
Rearrangement of the PAX3 paired box gene in the paediatric solid tumour alveolar rhabdomyosarcoma.
|
8098985 |
1993 |