Childhood Alveolar Rhabdomyosarcoma
|
0.300 |
Biomarker
|
disease |
MGD |
|
|
|
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 |
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 |
Childhood Alveolar Rhabdomyosarcoma
|
0.300 |
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 |
Childhood Alveolar Rhabdomyosarcoma
|
0.300 |
Biomarker
|
disease |
BEFREE |
The alveolar rhabdomyosarcoma PAX3/FKHR fusion protein is a transcriptional activator.
|
7630639 |
1995 |
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 |
GeneticVariation
|
disease |
BEFREE |
Sequence analysis of the last intron of PAX3, which contains the previously mapped t(2;13)(q35;q14) translocation breakpoints of alveolar rhabdomyosarcoma, revealed the presence of a pair of inverted Alu repeats and a pair of inverted (GT)n-rich microsatellite repeats within a 5-kb region.
|
7782066 |
1995 |
Childhood Alveolar Rhabdomyosarcoma
|
0.300 |
Biomarker
|
disease |
BEFREE |
Rearrangement of the PAX3 paired box gene in the paediatric solid tumour alveolar rhabdomyosarcoma.
|
8098985 |
1993 |
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 |
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 |
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 |
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 |
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 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 |
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 |
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 |
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 |
GeneticVariation
|
disease |
BEFREE |
Structural analysis of PAX3 genomic rearrangements in alveolar rhabdomyosarcoma.
|
9530337 |
1998 |
Childhood Alveolar Rhabdomyosarcoma
|
0.300 |
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 |
Childhood Alveolar Rhabdomyosarcoma
|
0.300 |
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 |
Childhood Alveolar Rhabdomyosarcoma
|
0.300 |
AlteredExpression
|
disease |
BEFREE |
PAX3 and PAX7 exhibit conserved cis-acting transcription repression domains and utilize a common gain of function mechanism in alveolar rhabdomyosarcoma.
|
10439042 |
1999 |
Childhood Alveolar Rhabdomyosarcoma
|
0.300 |
Biomarker
|
disease |
BEFREE |
The PAX3-FKHR fusion protein of human alveolar rhabdomyosarcoma consists of the DNA-binding domains of PAX3 and the transcriptional activation domain of FKHR.
|
10616906 |
1999 |
Childhood Alveolar Rhabdomyosarcoma
|
0.300 |
AlteredExpression
|
disease |
BEFREE |
A sample from a patient with a diagnosis of embryonal rhabdomyosarcoma on presentation and expression of PAX3-FKHR fusion transcript yielded a small focus of alveolar rhabdomyosarcoma and was reclassified as alveolar rhabdomyosarcoma.
|
10718207 |
2000 |
Childhood Alveolar Rhabdomyosarcoma
|
0.300 |
Biomarker
|
disease |
BEFREE |
With the difficulties in morphological diagnosis of alveolar rhabdomyosarcoma on increasingly used small needle biopsy specimens, these data suggest that molecular analysis for PAX3-FKHR will be a clinically useful tool in treatment stratification in the future.
|
11556833 |
2001 |
Childhood Alveolar Rhabdomyosarcoma
|
0.300 |
Biomarker
|
disease |
BEFREE |
Identification of target genes regulated by PAX3 and PAX3-FKHR in embryogenesis and alveolar rhabdomyosarcoma.
|
11863357 |
2002 |