|
Malignant Peripheral Nerve Sheath Tumor
|
0.400 |
Biomarker
|
disease |
BEFREE |
We demonstrated that several AAV serotypes achieved favorable transduction efficacies in those cells and a membrane-targeting GRD fused with an H-Ras C-terminal motif (C10) dramatically inhibited the Ras pathway and MPNST cells in a NF1-specific manner.
|
31127187 |
2019 |
|
Malignant Peripheral Nerve Sheath Tumor
|
0.400 |
Biomarker
|
disease |
BEFREE |
Functional experiments revealed that HMGA2 knockdown inhibits NF1 MPNST cell growth in vitro and in vivo.
|
31053152 |
2019 |
|
Malignant Peripheral Nerve Sheath Tumor
|
0.400 |
AlteredExpression
|
disease |
BEFREE |
The hallmark of NF1 and NF1-related MPNST is the loss of neurofibromin expression.
|
28859862 |
2018 |
|
Malignant Peripheral Nerve Sheath Tumor
|
0.400 |
Biomarker
|
disease |
BEFREE |
Thus, two-drug combinations that target MEK and mTORC1/2 are most effective in halting the RAS signaling cascade, and the relative success of this and related small molecule interventions in MPNSTs may be predicated upon the molecular status of neurofibromin.
|
29854299 |
2018 |
|
Malignant Peripheral Nerve Sheath Tumor
|
0.400 |
GeneticVariation
|
disease |
BEFREE |
Although loss of the NF1 gene predisposes to MPNST induction, relatively long tumor latency in NF1 patients suggests that additional genetic or epigenetic abnormalities are needed for the development of these nerve sheath malignancies.
|
27477693 |
2017 |
|
Malignant Peripheral Nerve Sheath Tumor
|
0.400 |
AlteredExpression
|
disease |
BEFREE |
Biallelic inactivation of the NF1 gene was observed in the plexiform neurofibroma and the MPNSTs, underlining that somatic biallelic NF1 inactivation is likely to be the initiating event for plexiform neurofibroma genesis, although it is unlikely to be sufficient for the subsequent MPNST development.
|
28124441 |
2017 |
|
Malignant Peripheral Nerve Sheath Tumor
|
0.400 |
GeneticVariation
|
disease |
BEFREE |
Patients with malignant peripheral nerve sheath tumor (MPNST), a rare soft tissue cancer associated with loss of the tumor suppressor neurofibromin (NF1), have poor prognosis and typically respond poorly to adjuvant therapy.
|
28556483 |
2017 |
|
Malignant Peripheral Nerve Sheath Tumor
|
0.400 |
GeneticVariation
|
disease |
BEFREE |
There was an increasing proportion of cells with a somatic NF1 gene mutation as the tumors progressed from benign to malignant, suggesting a clonal process in MPNST development.
|
25925892 |
2015 |
|
Malignant Peripheral Nerve Sheath Tumor
|
0.400 |
AlteredExpression
|
disease |
BEFREE |
Development of malignant peripheral nerve sheath tumors (MPNSTs) is a stepwise process that involves the alteration of many cell cycle regulators and the double inactivation of the NF1 gene.
|
26191206 |
2015 |
|
Malignant Peripheral Nerve Sheath Tumor
|
0.400 |
GeneticVariation
|
disease |
BEFREE |
Here we show that H-Ras, N-Ras, and K-Ras are coexpressed with their activators (guanine nucleotide exchange factors) in neurofibromin-null malignant peripheral nerve sheath tumor (MPNST) cells, and that all 3 Ras proteins are activated.
|
25946318 |
2015 |
|
Malignant Peripheral Nerve Sheath Tumor
|
0.400 |
GeneticVariation
|
disease |
BEFREE |
Malignant peripheral nerve sheath tumors (MPNSTs) are Schwann cell-derived malignancies that arise from plexiform neurofibromas in patients with mutation of the neurofibromin 1 (NF1) gene.
|
24232507 |
2014 |
|
Malignant Peripheral Nerve Sheath Tumor
|
0.400 |
Biomarker
|
disease |
BEFREE |
NFC immunohistochemistry revealed loss of neurofibromin in 22/25 (88 %) of NF1-associated and 26/61 (43 %) of sporadic MPNST.
|
24464231 |
2014 |
|
Malignant Peripheral Nerve Sheath Tumor
|
0.400 |
Biomarker
|
disease |
BEFREE |
Starting with the mTOR inhibitors rapamycin and everolimus, we screened for synergy in 542 FDA approved compounds using MPNST cells with a native NF1 loss in both alleles.
|
24668609 |
2014 |
|
Malignant Peripheral Nerve Sheath Tumor
|
0.400 |
Biomarker
|
disease |
BEFREE |
Preclinical studies on neurofibromin-deficient malignancies have mainly been on malignant peripheral nerve sheath tumour cell lines or xenografts derived from NF1 patients.
|
25026295 |
2014 |
|
Malignant Peripheral Nerve Sheath Tumor
|
0.400 |
Biomarker
|
disease |
BEFREE |
MPNST cell lines with complete neurofibromin deficiency were sensitive to apoptotic cell death induced by TRAIL whereas MPNST cells with retained neurofibromin expression or normal human Schwann cells were resistant.
|
23437333 |
2013 |
|
Malignant Peripheral Nerve Sheath Tumor
|
0.400 |
Biomarker
|
disease |
BEFREE |
Collectively, our results suggest that upregulation of Bcl-xL in MPNST-derived SCs may be caused by the NF1 deficiency-mediated elevation in Ras/MAPK signaling and may provide a new potential chemotherapeutic target in patients with NF1 and MPNSTs.
|
23292448 |
2013 |
|
Malignant Peripheral Nerve Sheath Tumor
|
0.400 |
AlteredExpression
|
disease |
BEFREE |
Although neurofibromin expression was maintained, P(0)-GGFβ3 MPNSTs exhibited Ras hyperactivation, as in human NF1-associated MPNSTs.
|
23321323 |
2013 |
|
Malignant Peripheral Nerve Sheath Tumor
|
0.400 |
Biomarker
|
disease |
BEFREE |
The pathway-specific gene changes provide a greater understanding of the complex role of neurofibromin in MPNST pathology and novel targets for drug discovery.
|
23423222 |
2013 |
|
Malignant Peripheral Nerve Sheath Tumor
|
0.400 |
Biomarker
|
disease |
BEFREE |
We show here that in cells derived from neurofibromin-deficient (Nf1(-/-)) malignant peripheral nerve sheath tumors (MPNSTs), Ras inhibition by S-trans,trans-farnesylthiosalicylic-acid (FTS; Salirasib) shifts the pattern of galectin expression.
|
23530091 |
2013 |
|
Malignant Peripheral Nerve Sheath Tumor
|
0.400 |
Biomarker
|
disease |
BEFREE |
Nerve Schwann cells and/or their precursors are the tumorigenic cell types in MPNST because of the loss of the NF1 gene, which encodes the RasGAP protein neurofibromin.
|
22811580 |
2012 |
|
Malignant Peripheral Nerve Sheath Tumor
|
0.400 |
GeneticVariation
|
disease |
BEFREE |
Neurofibromatosis type-1 (NF1), resulting from NF1 gene loss of function, is characterized by an increased risk of developing benign and malignant peripheral nerve sheath tumors (MPNSTs).
|
23244685 |
2012 |
|
Malignant Peripheral Nerve Sheath Tumor
|
0.400 |
Biomarker
|
disease |
BEFREE |
These models have been designed to address key questions including: (1) whether NF1 loss in the Schwann cell lineage is essential for tumorigenesis; (2) what cell type(s) in the Schwann cell lineage gives rise to dermal neurofibromas, plexiform neurofibromas and MPNSTs; (3) how the tumor microenvironment contributes to neoplasia; (4) what additional mutations contribute to neurofibroma-MPNST progression; (5) what role different neurofibromin-regulated Ras proteins play in this process and (6) how dysregulated growth factor signaling facilitates PNS tumorigenesis.
|
21855613 |
2012 |
|
Malignant Peripheral Nerve Sheath Tumor
|
0.400 |
GeneticVariation
|
disease |
BEFREE |
Malignant peripheral nerve sheath tumor of the vagus nerve in a teenager with the neurofibromatosis 1 gene mutation: a case report.
|
21843710 |
2011 |
|
Malignant Peripheral Nerve Sheath Tumor
|
0.400 |
Biomarker
|
disease |
BEFREE |
Discovery of a small molecule targeting IRA2 deletion in budding yeast and neurofibromin loss in malignant peripheral nerve sheath tumor cells.
|
21697395 |
2011 |
|
Malignant Peripheral Nerve Sheath Tumor
|
0.400 |
Biomarker
|
disease |
BEFREE |
Neurofibromin-deficient (Nf1(-/-)) malignant peripheral nerve sheath tumors (MPNST) are highly invasive, refractory to chemotherapy, and characterized by overactivated Ras.
|
21632464 |
2011 |