Childhood Rhabdomyosarcoma
|
0.100 |
Biomarker
|
disease |
BEFREE |
The histological diagnosis was alveolar (RMA) (n = 38, 23/25 examined patients PAX7/3:FOXO1-positive), embryonal (RME) (n = 100), botryoid (n = 10), anaplastic (n = 1), and spindle-cell RMS (n = 6).
|
30762282 |
2019 |
Childhood Rhabdomyosarcoma
|
0.100 |
Biomarker
|
disease |
BEFREE |
Our findings identify a novel role of both PAX3-FOXO1 and its downstream effector miR-486-5p in exosome-mediated oncogenic paracrine effects of RMS, and suggest its possible use as a biomarker.
|
31578374 |
2019 |
Childhood Rhabdomyosarcoma
|
0.100 |
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 Rhabdomyosarcoma
|
0.100 |
Biomarker
|
disease |
BEFREE |
While RMS has been traditionally classified on the basis of its histological appearance (with embryonal and alveolar being most common), it is now clear that the PAX-FOXO1 fusion product drives prognosis.
|
30806137 |
2019 |
Childhood Rhabdomyosarcoma
|
0.100 |
Biomarker
|
disease |
BEFREE |
We re-examined risk stratification by adding FOXO1 status to traditional clinical prognostic factors in children with localized or metastatic RMS.
|
31456361 |
2019 |
Childhood Rhabdomyosarcoma
|
0.100 |
Biomarker
|
disease |
BEFREE |
This is supported in rhabdomyosarcoma models by characterization of molecular and phenotypic effects of reducing and inhibiting PLK1, including changes to the PAX3-FOXO1 fusion protein.
|
31824851 |
2019 |
Childhood Rhabdomyosarcoma
|
0.100 |
GeneticVariation
|
disease |
BEFREE |
Thus, we examined gene co-expression networks for rhabdomyosarcoma that were <i>FOXO1-PAX3</i> positive, <i>FOXO1-PAX7</i> positive, or fusion negative.
|
31480361 |
2019 |
Childhood Rhabdomyosarcoma
|
0.100 |
Biomarker
|
disease |
BEFREE |
Together with the observed effects on the PAX3-FOXO1 fusion protein, these data suggest SAHA as a possible therapeutic agent for clinical testing in patients with fusion protein-positive RMS.
|
30307360 |
2019 |
Childhood Rhabdomyosarcoma
|
0.100 |
Biomarker
|
disease |
BEFREE |
This study describes the in vitro and in vivo activity of PXD-101 (Belinostat), a novel hydroxamic acid-type pan-HDACs inhibitor characterized by a larger safety and efficacy, on myogenic-derived PAX3/FOXO1 fusion protein positive (RH30) or negative (RD) expressing rhabdomyosarcoma (RMS) cell lines.
|
31325529 |
2019 |
Childhood Rhabdomyosarcoma
|
0.100 |
Biomarker
|
disease |
BEFREE |
Interleukin-24 (IL24) Is Suppressed by PAX3-FOXO1 and Is a Novel Therapy for Rhabdomyosarcoma.
|
30190424 |
2018 |
Childhood Rhabdomyosarcoma
|
0.100 |
AlteredExpression
|
disease |
BEFREE |
In this article, we review the recent understanding of PAX3-FOXO1 as a transcription factor in the pathogenesis of this cancer and discuss recent developments to target this oncoprotein for treatment of RMS.
|
30373318 |
2018 |
Childhood Rhabdomyosarcoma
|
0.100 |
Biomarker
|
disease |
BEFREE |
The rapid onset and increased penetrance of tumorigenesis in this model provide a powerful tool for interrogating aRMS biology and screening novel therapeutics.<b>Significance:</b> A novel mouse model sheds light on the critical role of Hippo/MST downregulation in PAX3-FOXO1-positive rhabdomyosarcoma tumorigenesis.<i></i>.
|
30093562 |
2018 |
Childhood Rhabdomyosarcoma
|
0.100 |
Biomarker
|
disease |
BEFREE |
We compared the event-free survival (EFS) and overall survival by histology, PAX-FOXO1 fusion, and clinical risk factors (Oberlin score) for patients with metastatic RMS using the log-rank test.
|
28521080 |
2017 |
Childhood Rhabdomyosarcoma
|
0.100 |
Biomarker
|
disease |
BEFREE |
Many findings have demonstrated that PAX/FOXO1-positive ARMS have a worse prognosis than PAX/FOXO1-negative ones and that distinct molecular features characterize RMS with different gene fusion statuses.
|
28380437 |
2017 |
Childhood Rhabdomyosarcoma
|
0.100 |
Biomarker
|
disease |
BEFREE |
We find that PAX3-FOXO1 establishes a myoblastic super enhancer landscape and creates a profound subtype-unique dependence on BET bromodomains, the inhibition of which ablates PAX3-FOXO1 function, providing a mechanistic rationale for exploring BET inhibitors for patients bearing PAX-fusion rhabdomyosarcoma.<i>Cancer Discov; 7(8); 884-99.
|
28446439 |
2017 |
Childhood Rhabdomyosarcoma
|
0.100 |
Biomarker
|
disease |
BEFREE |
The identification of PAX-FOXO1 fusion status in RMS is one of the most important updates in the risk stratification of RMS.
|
28256213 |
2017 |
Childhood Rhabdomyosarcoma
|
0.100 |
Biomarker
|
disease |
BEFREE |
PAX3-FOXO1 is essential for tumour initiation and maintenance but not recurrence in a human myoblast model of rhabdomyosarcoma.
|
28138962 |
2017 |
Childhood Rhabdomyosarcoma
|
0.100 |
AlteredExpression
|
disease |
BEFREE |
PAX3-FOXO1A Expression in Rhabdomyosarcoma Is Driven by the Targetable Nuclear Receptor NR4A1.
|
27864345 |
2017 |
Childhood Rhabdomyosarcoma
|
0.100 |
Biomarker
|
disease |
BEFREE |
Previous studies, including our own, have shown prognostic value for the presence of PAX3/7-FOXO1 fusion genes in rhabdomyosarcoma (RMS).
|
28035744 |
2017 |
Childhood Rhabdomyosarcoma
|
0.100 |
Biomarker
|
disease |
BEFREE |
In the present study, we applied fluorescent in situ hybridization (FISH) and found that the Forkhead box O1 (FOXO1) gene broke apart, amplified, and displayed an aneuploid signal that was related to the RMS pathological subtype.
|
28646473 |
2017 |
Childhood Rhabdomyosarcoma
|
0.100 |
Biomarker
|
disease |
BEFREE |
Regulatory landscape fusion in rhabdomyosarcoma through interactions between the PAX3 promoter and FOXO1 regulatory elements.
|
28615069 |
2017 |
Childhood Rhabdomyosarcoma
|
0.100 |
GeneticVariation
|
disease |
BEFREE |
The current molecular classification identifies 2 major subsets, those harboring the fused Pax3-Foxo1 transcription factor generating from a recurrent specific translocation (fusion-positive RMS), and those lacking this signature but harboring mutations in the RAS/PI3K/AKT signaling axis (fusion-negative RMS).
|
26209235 |
2016 |
Childhood Rhabdomyosarcoma
|
0.100 |
AlteredExpression
|
disease |
BEFREE |
In addition, the seven chimeric RNAs that follow the exact transient expression pattern as PAX3-FOXO1 are specific to rhabdomyosarcoma cells.
|
27799565 |
2016 |
Childhood Rhabdomyosarcoma
|
0.100 |
Biomarker
|
disease |
BEFREE |
Inhibition of HO-1 by tin protoporphyrin (SnPP) or siRNA downregulated Pax3/7-FoxO1 target genes and induced a myogenic program in RMS.
|
27488535 |
2016 |
Childhood Rhabdomyosarcoma
|
0.100 |
GeneticVariation
|
disease |
BEFREE |
Rhabdomyosarcoma (RMS) is the most common pediatric soft tissue sarcoma and includes a PAX3- or PAX7-FOXO1 fusion-positive subtype.
|
25810375 |
2015 |