|
Adult Rhabdomyosarcoma
|
0.100 |
Biomarker
|
disease |
BEFREE |
Translocation of the 5' region of the FKHR gene to the derivative chromosome 2, and retention of the 3' region of FKHR on the derivative chromosome 13 [(der(13)], were demonstrated in metaphase cells from a rhabdomyosarcoma cell line with a previously identified t(2;13) translocation.
|
7536457 |
1995 |
|
Adult Rhabdomyosarcoma
|
0.100 |
Biomarker
|
disease |
BEFREE |
Sequential immunoprecipitations with anti-PAX3 and anti-FKHR sera demonstrated expression of a 97-kDa PAX3-FKHR fusion protein in the t(2;13)-positive rhabdomyosarcoma Rh30 cell line and verified that a single polypeptide contains epitopes derived from each protein.
|
7862145 |
1995 |
|
Adult Rhabdomyosarcoma
|
0.100 |
GeneticVariation
|
disease |
BEFREE |
We evaluated the clinical features of the common PAX3-FKHR and variant PAX7-FKHR gene fusions observed in rhabdomyosarcoma.
|
9164192 |
1997 |
|
Adult Rhabdomyosarcoma
|
0.100 |
AlteredExpression
|
disease |
BEFREE |
We used a myogenic tumor cell culture system and rhabdomyosarcoma tumor specimens to assess candidate gene expression in relationship to various PAX3-FKHR expression levels.
|
9721857 |
1998 |
|
Adult Rhabdomyosarcoma
|
0.100 |
Biomarker
|
disease |
BEFREE |
These results support the hypothesis that PAX3-FKHR interacts with IGF-II to play a critical role in the oncogenesis of rhabdomyosarcoma.
|
9766674 |
1998 |
|
Adult Rhabdomyosarcoma
|
0.100 |
Biomarker
|
disease |
BEFREE |
The Pax3-FKHR fusion is unresponsive to this repressive effect despite an observed endogenous interaction with hDaxx in a rhabdomyosarcoma tumor cell line.
|
10393185 |
1999 |
|
Adult Rhabdomyosarcoma
|
0.100 |
GeneticVariation
|
disease |
BEFREE |
The authors applied reverse transcriptase-polymerase chain reaction analysis (RT-PCR) to analyze tumor samples from 14 children with rhabdomyosarcoma for the presence of the chimeric PAX3-FKHR transcript resulting from the translocation t(2;13)(q35,q14).
|
10718207 |
2000 |
|
Adult Rhabdomyosarcoma
|
0.100 |
GeneticVariation
|
disease |
BEFREE |
The availability of an ARMS cell line that harbors the t(1;13)(p36;q14) constitutes a useful tool for further understanding the role of the PAX7-FKHR fusion gene in RMS oncogenesis and may improve knowledge of the possible relation between PAX7-FKHR and MYCN amplification.
|
11063797 |
2000 |
|
Adult Rhabdomyosarcoma
|
0.100 |
Biomarker
|
disease |
BEFREE |
We could previously demonstrate that one of the oncogenic functions of PAX3 and PAX3/FKHR in RMS is protection from apoptosis.
|
10871843 |
2000 |
|
Adult Rhabdomyosarcoma
|
0.100 |
Biomarker
|
disease |
BEFREE |
We have determined the presence of the PAX3/7-FKHR fusion genes in 91 primary rhabdomyosarcoma tumours using a combination of classical cytogenetics, FISH and RT-PCR, with a view to determining the clinical characteristics of tumours with and without the characteristic translocations.
|
11556833 |
2001 |
|
Adult Rhabdomyosarcoma
|
0.100 |
Biomarker
|
disease |
BEFREE |
In this investigation, we selected PAX3/FKHR and PAX7/FKHR fusion transcript-positive and -negative alveolar rhabdomyosarcomas (ARMSs) and embryonal rhabdomyosarcomas (ERMSs) with and without anaplastic features, to ascertain genomic imbalance differences and/or similarities within these histopathologic and genetic rhabdomyosarcoma (RMS) variants.
|
11807989 |
2002 |
|
Adult Rhabdomyosarcoma
|
0.100 |
Biomarker
|
disease |
BEFREE |
We determined PAX3-FKHR or PAX7-FKHR fusion status in 171 childhood rhabdomyosarcoma (RMS) patients entered onto the Intergroup Rhabdomyosarcoma Study IV, including 78 ARMS patients, using established reverse transcriptase polymerase chain reaction assays.
|
12039929 |
2002 |
|
Adult Rhabdomyosarcoma
|
0.100 |
AlteredExpression
|
disease |
BEFREE |
The different results obtained with these RMS cell lines does not appear to be related to expression of pax3-FKHR, p21, Bax or Bcl-2 but may in part be due to differential regulation of p53 target genes, such as MDM2.
|
12394275 |
2002 |
|
Adult Rhabdomyosarcoma
|
0.100 |
AlteredExpression
|
disease |
BEFREE |
Furthermore, transfer of a PAX3-FKHR gene into embryonal RMS cell activates CXCR4 expression.
|
12239174 |
2002 |
|
Adult Rhabdomyosarcoma
|
0.100 |
AlteredExpression
|
disease |
BEFREE |
The expression levels of these genes correlate with the PAX3 expression levels in mouse embryos or with PAX3-FKHR expression levels in rhabdomyosarcoma cell lines, and indicate they may be part of the PAX3 regulatory circuitry during embryogenesis and tumor formation.
|
11863357 |
2002 |
|
Adult Rhabdomyosarcoma
|
0.100 |
Biomarker
|
disease |
BEFREE |
FOXO1 gene is fused to PAX3 or PAX7 genes in rhabdomyosarcoma.
|
15492844 |
2004 |
|
Adult Rhabdomyosarcoma
|
0.100 |
AlteredExpression
|
disease |
BEFREE |
Furthermore, PI3K-AKT appeared to regulate D-type cyclin transcription in RMS lines through FKHR and FKHRL1.
|
15342383 |
2004 |
|
Adult Rhabdomyosarcoma
|
0.100 |
GeneticVariation
|
disease |
BEFREE |
The most common types of rhabdomyosarcoma (RMS) are alveolar RMS (ARMS), which are characterized by the specific translocation t(2;13)(q35;q14) or its rarer variant, t(1;13)(p36;q14), producing the fusion genes PAX3-FKHR and PAX7-FKHR, respectively, and embryonal RMS (ERMS), which is characterized by multiple numeric chromosome changes.
|
16337856 |
2005 |
|
Adult Rhabdomyosarcoma
|
0.100 |
AlteredExpression
|
disease |
BEFREE |
In conclusion, these studies reveal that PAX3, PAX7 and their fusions with FKHR are each expressed in RMS tumors as a consistent mixture of functionally distinct isoforms.
|
15688409 |
2005 |
|
Adult Rhabdomyosarcoma
|
0.100 |
AlteredExpression
|
disease |
BEFREE |
We found that ARMS tumors expressing either PAX-FKHR gene share a common expression profile distinct from fusion-negative ARMS and from the other rhabdomyosarcoma variants.
|
16849537 |
2006 |
|
Adult Rhabdomyosarcoma
|
0.100 |
Biomarker
|
disease |
BEFREE |
These results indicate that the involvement of PAX3 and PAX3-FKHR in rhabdomyosarcoma tumourigenesis may be through downregulation of PTEN tumour suppressor gene, affecting the PTEN/AKT survival pathway.
|
17350164 |
2007 |
|
Adult Rhabdomyosarcoma
|
0.100 |
GeneticVariation
|
disease |
BEFREE |
Standard cytogenetic analyses and RT-PCR testing for the classic gene rearrangements seen in RMS [t(2;13)-PAX3/FKHR] and EWS ([t(11;22) & t(21;22)-EWS/FLI-1 & EWS/ERG), were used for characterization of the MEM, with gene expression microarray analyses on all tumor types.
|
17431644 |
2007 |
|
Adult Rhabdomyosarcoma
|
0.100 |
AlteredExpression
|
disease |
BEFREE |
Real-time RT-PCR assays specific for Ewing's sarcoma (EWS-FLI1, EWS-ERG, EWS-ETV1, EWS-ETV4, and EWS-FEV), synovial sarcoma (SYT-SSX1 and SYT-SSX2), and rhabdomyosarcoma (PAX3-FKHR and PAX7-FKHR) were tested across the samples.
|
17334332 |
2007 |
|
Adult Rhabdomyosarcoma
|
0.100 |
Biomarker
|
disease |
BEFREE |
A less frequent variant translocation t(1;13) involves another PAX family gene, /PAX7/, located in chromosome 1 and /FOXO1/ and is present in 10-15% of cases of the alveolar subtype in RMS.
|
17652054 |
2007 |
|
Adult Rhabdomyosarcoma
|
0.100 |
Biomarker
|
disease |
BEFREE |
FOXO1 break-apart by FISH was positive in 4 of 6 (66%) ARMS and 2 of 5 (40%) RMS-NOS cases.
|
18303411 |
2008 |