|
Alveolar rhabdomyosarcoma
|
0.800 |
Biomarker
|
disease |
HPO |
|
|
|
|
Alveolar rhabdomyosarcoma
|
0.800 |
Biomarker
|
disease |
CTD_human |
|
|
|
|
Alveolar rhabdomyosarcoma
|
0.800 |
FusionGene
|
disease |
ORPHANET |
|
|
|
|
Alveolar rhabdomyosarcoma
|
0.800 |
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 |
|
Alveolar rhabdomyosarcoma
|
0.800 |
GeneticVariation
|
disease |
BEFREE |
Alveolar rhabdomyosarcoma (ARMS) is characterized cytogenetically by a t(2;13)(q35;q14) chromosomal translocation involving two transcription factor genes: PAX3 and FKHR.
|
7624119 |
1995 |
|
Alveolar rhabdomyosarcoma
|
0.800 |
GeneticVariation
|
disease |
BEFREE |
The PAX3-FKHR fusion protein created by the t(2;13) translocation in alveolar rhabdomyosarcomas is a more potent transcriptional activator than PAX3.
|
7862145 |
1995 |
|
Alveolar rhabdomyosarcoma
|
0.800 |
GeneticVariation
|
disease |
BEFREE |
Detection of the t(2;13)(q35;q14) and PAX3-FKHR fusion in alveolar rhabdomyosarcoma by fluorescence in situ hybridization.
|
7536457 |
1995 |
|
Alveolar rhabdomyosarcoma
|
0.800 |
GeneticVariation
|
disease |
BEFREE |
Using pulsed-field and fluorescence in situ hybridization analyses, we demonstrate that the 130kb FKHR intron 1 is rearranged in t(2;13)-containing alveolar rhabdomyosarcomas.
|
8634710 |
1995 |
|
Alveolar rhabdomyosarcoma
|
0.800 |
Biomarker
|
disease |
BEFREE |
These data suggest that the PAX3/FKHR fusion gene and wild-type PAX genes play a causative role in the formation of RMS and presumably other tumor types, possibly by suppressing the apoptotic program that would normally eliminate these cells.
|
8917562 |
1996 |
|
Alveolar rhabdomyosarcoma
|
0.800 |
Biomarker
|
disease |
BEFREE |
The hybrid PAX3-FKHR fusion protein of alveolar rhabdomyosarcoma transforms fibroblasts in culture.
|
8790412 |
1996 |
|
Alveolar rhabdomyosarcoma
|
0.800 |
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 |
|
Alveolar rhabdomyosarcoma
|
0.800 |
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
|
0.800 |
GeneticVariation
|
disease |
BEFREE |
Comparative genomic hybridization revealed amplification at 13q14 and 1p36, suggesting amplification of the PAX7-FKHR fusion gene in two cases of alveolar rhabdomyosarcoma.
|
8889501 |
1996 |
|
Alveolar rhabdomyosarcoma
|
0.800 |
Biomarker
|
disease |
BEFREE |
In vivo amplification of the PAX3-FKHR and PAX7-FKHR fusion genes in alveolar rhabdomyosarcoma.
|
8789435 |
1996 |
|
Alveolar rhabdomyosarcoma
|
0.800 |
Biomarker
|
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 |
|
Alveolar rhabdomyosarcoma
|
0.800 |
GeneticVariation
|
disease |
BEFREE |
It is highly homologous to the human FKHR protein, the gene of which is disrupted by the t(2;13)(q35;q14), a chromosome rearrangement characteristic of alveolar rhabdomyosarcomas.
|
9010221 |
1997 |
|
Alveolar rhabdomyosarcoma
|
0.800 |
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 |
|
Alveolar rhabdomyosarcoma
|
0.800 |
GeneticVariation
|
disease |
BEFREE |
Molecular analysis with RT-PCR amplification of RNA isolated from the tumour demonstrated the presence of a PAX3/FKHR fusion transcript, product of a t(2;13) reciprocal translocation, a genetic marker specific for alveolar rhabdomyosarcoma.
|
9543674 |
1998 |
|
Alveolar rhabdomyosarcoma
|
0.800 |
Biomarker
|
disease |
BEFREE |
These findings indicate that MET, which encodes a receptor involved in growth and motility signaling, is a downstream target of PAX3-FKHR in alveolar rhabdomyosarcoma.
|
9721857 |
1998 |
|
Alveolar rhabdomyosarcoma
|
0.800 |
GeneticVariation
|
disease |
BEFREE |
Together with the FKHR gene it is involved in the t(2;13)(q35;q14), a specific translocation associated with alveolar rhabdomyosarcoma (ARMS).
|
9546061 |
1998 |
|
Alveolar rhabdomyosarcoma
|
0.800 |
GeneticVariation
|
disease |
BEFREE |
Among these 37 were genes related to both primary (PAX3-FKHR) and secondary (CDK4) genetic alterations in ARMS.
|
9823299 |
1998 |
|
Alveolar rhabdomyosarcoma
|
0.800 |
GeneticVariation
|
disease |
BEFREE |
The t(2;13) and t(1;13) translocations of alveolar rhabdomyosarcoma (ARMS) result in chimeric PAX3-FKHR or PAX7-FKHR transcription factors, respectively.
|
10439042 |
1999 |
|
Alveolar rhabdomyosarcoma
|
0.800 |
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
|
0.800 |
Biomarker
|
disease |
BEFREE |
A genetic analysis of PAX3-FKHR, the oncogene of alveolar rhabdomyosarcoma.
|
10616906 |
1999 |
|
Alveolar rhabdomyosarcoma
|
0.800 |
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 |