|
Alveolar rhabdomyosarcoma
|
1.000 |
Biomarker
|
disease |
BEFREE |
Here, we show that upregulation of the cannabinoid receptor 1 (Cnr1/Cb1) by PAX3-FOXO1 in mouse primary myoblasts and ARMS cell lines, contributes to PAX3-FOXO1 phenotypes, both in vivo and in vitro.
|
22037868 |
2011 |
|
Alveolar rhabdomyosarcoma
|
1.000 |
Biomarker
|
disease |
BEFREE |
Identification of target genes regulated by PAX3 and PAX3-FKHR in embryogenesis and alveolar rhabdomyosarcoma.
|
11863357 |
2002 |
|
Alveolar rhabdomyosarcoma
|
1.000 |
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 |
|
Alveolar rhabdomyosarcoma
|
1.000 |
Biomarker
|
disease |
BEFREE |
The engineered repressor approach that directs a transcriptional repression domain to target genes deregulated by the PAX3-FKHR oncogene may be a useful strategy to identify the target genes critical for ARMS tumorigenesis.
|
10866659 |
2000 |
|
Alveolar rhabdomyosarcoma
|
1.000 |
GeneticVariation
|
disease |
BEFREE |
In this study, cytogenetic and/or molecular characterization to include FISH, reverse transcription polymerase chain reaction (RT-PCR), and sequencing analyses of five rhabdomyosarcomas [four ARMS and one embryonal rhabdomyosarcoma (ERMS)] with novel, recurrent t(2;2)(p23;q35) or t(2;8)(q35;q13) revealed that these noncanonical translocations fuse PAX3 to NCOA1 or NCOA2, respectively.
|
19953635 |
2010 |
|
Alveolar rhabdomyosarcoma
|
1.000 |
Biomarker
|
disease |
BEFREE |
Prognostic value of PAX3/7-FOXO1 fusion status in alveolar rhabdomyosarcoma: Systematic review and meta-analysis.
|
26008753 |
2015 |
|
Alveolar rhabdomyosarcoma
|
1.000 |
GeneticVariation
|
disease |
BEFREE |
Recent studies of ARMS have shown two specific recurrent translocations: PAX3-FKHR [t(2;13)(q35;q14)] or PAX7-FKHR [t(1;13)(p36;q14)].
|
24427371 |
2014 |
|
Alveolar rhabdomyosarcoma
|
1.000 |
GeneticVariation
|
disease |
BEFREE |
Alveolar rhabdomyosarcoma (ARMS), a paediatric soft tissue malignant tumour, is characterized by the recurrent translocation t(2;13)(q35;q14) that fuses the 5' DNA binding domain-encoding sequences of the Pax3 gene with the 3' sequences of the FKHR gene.
|
11735247 |
2001 |
|
Alveolar rhabdomyosarcoma
|
1.000 |
GeneticVariation
|
disease |
BEFREE |
In alveolar rhabdomyosarcoma, a (2;13)(q35;qt4) translocation is associated with a chimeric gene between PAX3 and FKHR.
|
8875708 |
1996 |
|
Alveolar rhabdomyosarcoma
|
1.000 |
Biomarker
|
disease |
BEFREE |
The alveolar rhabdomyosarcoma PAX3/FKHR fusion protein is a transcriptional activator.
|
7630639 |
1995 |
|
Alveolar rhabdomyosarcoma
|
1.000 |
GeneticVariation
|
disease |
BEFREE |
We have isolated two isoforms of cDNA clones from the human PAX3 gene, a candidate gene responsible for Waardenburg syndrome type I (WSI) as well as a gene associated with development of alveolar rhabdomyosarcoma.
|
7545913 |
1994 |
|
Alveolar rhabdomyosarcoma
|
1.000 |
GeneticVariation
|
disease |
BEFREE |
Alveolar Rhabdomyosarcomas (RMA) are characterized by chromosomal translocations, fusing the PAX3 or PAX7 gene with FKHR in about 85%.
|
21254373 |
2011 |
|
Alveolar rhabdomyosarcoma
|
1.000 |
Biomarker
|
disease |
HPO |
|
|
|
|
Alveolar rhabdomyosarcoma
|
1.000 |
Biomarker
|
disease |
BEFREE |
Chimeric proteins encoded in most ARMS from the fusion of PAX3 or PAX7 with FOXO1 are expressed, result in a distinct pattern of downstream protein expression, and appear to be the proximate cause of the bad outcome associated with this subtype.
|
24113309 |
2013 |
|
Alveolar rhabdomyosarcoma
|
1.000 |
GeneticVariation
|
disease |
BEFREE |
Classically, the alveolar subtype is characterized by a chromosomal translocation t(2;13)(q35;q14) or t(1;13)(p36;q14) fusing the PAX3 or PAX7 gene, respectively, to the FOXO1 gene, although fusion-negative cases of alveolar rhabdomyosarcoma (ARMS) occur; these share considerably more with the genomic profiles and biological behavior of embryonal rhabdomyosarcoma than with fusion-positive ARMS.
|
24614150 |
2014 |
|
Alveolar rhabdomyosarcoma
|
1.000 |
Biomarker
|
disease |
BEFREE |
Alveolar rhabdomyosarcoma-associated PAX3-FOXO1 promotes tumorigenesis via Hippo pathway suppression.
|
24334454 |
2014 |
|
Alveolar rhabdomyosarcoma
|
1.000 |
Biomarker
|
disease |
BEFREE |
Alveolar rhabdomyosarcoma (ARMS) is a soft tissue cancer in which chromosomal translocations generate PAX3-FKHR and PAX7-FKHR gene fusions.
|
16645206 |
2006 |
|
Alveolar rhabdomyosarcoma
|
1.000 |
Biomarker
|
disease |
BEFREE |
The Pax3-FKHR fusion protein is present in alveolar rhabdomyosarcoma and results from the t(2;13) (q35;q14) chromosomal translocation.
|
10393185 |
1999 |
|
Alveolar rhabdomyosarcoma
|
1.000 |
GeneticVariation
|
disease |
BEFREE |
The FKHR gene, which contains a forkhead DNA-binding motif, is fused to either PAX3 or PAX7 by the t(2;13) or t(1;13) translocation in alveolar rhabdomyosarcoma,respectively.
|
8634710 |
1995 |
|
Alveolar rhabdomyosarcoma
|
1.000 |
Biomarker
|
disease |
BEFREE |
Using phospho-specific antibodies we demonstrate a changing pattern of phosphorylation at serines 201, 205, and 209 throughout early myogenic differentiation and that this pattern of phosphorylation is different for Pax3-FOXO1 in primary myoblasts and in several ARMS cell lines.
|
21440083 |
2011 |
|
Alveolar rhabdomyosarcoma
|
1.000 |
Biomarker
|
disease |
BEFREE |
PAX3-FKHR, the catalytic unit of telomerase hTERT, and MycN, in cooperation with down-regulation of p16INK4A/p14ARF expression, were necessary and sufficient to convert normal human myoblasts into tumorigenic cells that gave rise to aRMS tumors.
|
19047133 |
2008 |
|
Alveolar rhabdomyosarcoma
|
1.000 |
Biomarker
|
disease |
LHGDN |
Solid alveolar rhabdomyosarcoma originating from the urinary bladder in an adult. Diagnostic value of molecular genetics.
|
15140004 |
2004 |
|
Alveolar rhabdomyosarcoma
|
1.000 |
Biomarker
|
disease |
BEFREE |
To identify genes cooperating with PAX3-FOXO1 in ARMS tumorigenesis, we generated a retroviral complementary DNA (cDNA) expression library from the Rh30 ARMS cell line.
|
21177767 |
2011 |
|
Alveolar rhabdomyosarcoma
|
1.000 |
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 |
|
Alveolar rhabdomyosarcoma
|
1.000 |
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 |