GEO DataSets

(Submitter supplied) Neurofibromatosis Type 1 (NF1) is a common cancer predisposition syndrome, caused by heterozygous loss of function mutations in the tumor suppressor gene NF1. Individuals with NF1 develop benign tumors of the peripheral nervous system (neurofibromas), originating from the Schwann cell linage after somatic loss of the wild type NF1 allele, some of which progress further to malignant peripheral nerve sheath tumors (MPNST). more...

Organism:

Homo sapiens

Type:

Expression profiling by high throughput sequencing

Platform:

GPL24676

51 Samples

Download data: TXT

(Submitter supplied) Neurofibromatosis Type 1 (NF1) patients develop benign neurofibromas and malignant peripheral nerve sheath tumors (MPNST). These incurable peripheral nerve tumors result from loss of NF1 tumor suppressor gene function, causing hyperactive Ras signaling. Activated Ras controls numerous downstream effectors, but specific pathways mediating effects of hyperactive Ras in NF1 tumors are unknown. Cross-species transcriptome analyses of mouse and human neurofibromas and MPNSTs identified global negative feedback of genes that regulate Ras-Raf- MEK- extracellular signal-regulated protein kinase (ERK) signaling in both species. more...

Organism:

Homo sapiens; Mus musculus

Type:

Expression profiling by array

Platforms:

GPL10371 GPL14757

83 Samples

Download data: CEL, TXT

(Submitter supplied) 7 MPNSTs from 7 neurofibromatosis-type 1 patients were screened with a high resolution array-CGH. Each MPNST DNA (somatic tumor DNA) was individually hybridized on Agilent whole human genome 244K microarrays (Platform GPL4091) using the pooled genomic constitutional DNA (lymphocytes DNA) from the normal control patients as reference, in order to detect tumor-specific aberrations.

Organism:

Homo sapiens

Type:

Genome variation profiling by genome tiling array

Platform:

GPL4091

7 Samples

Download data: TXT

(Submitter supplied) We established a new genetically engineered mouse (GEM) model of malignant peripheral nerve sheath tumors (MPNST) based on postnatal deletion of a Nf1;Trp53 cis-conditional allele by the tamoxifen-inducible Plp-CreER (NP-Plp). We also generated two Lats1;2 conditional knockout models by using Nestin-Cre (Lats-Nes) and Plp-CreER (Lats-Plp), both of which also develop tumors similar to MPNST (GEM-PNST). more...

Organism:

Homo sapiens; Mus musculus

Type:

Expression profiling by high throughput sequencing

Platforms:

GPL24247 GPL24676

44 Samples

Download data: TXT

(Submitter supplied) We identified a molecule in a synthetic lethal screen with ira2Δ in yeast called Y100. Y100 targets ira2Δ deficient yeast and inhibits NF1-deficient tumor cells. Y100 disrupts proteostasis, metabolic homeostasis, and induces the formation of mitochondrial superoxide in NF1 deficient cancer cells. Here, we examined the transcriptional response following treatment with Y100 or a vehicle control.

Organism:

Homo sapiens

Type:

Expression profiling by array

Platform:

GPL10558

5 Samples

Download data: TXT

(Submitter supplied) Patients with neurofibromatosis type 1 (NF1) develop benign plexiform neurofibromas that frequently progress to become malignant peripheral nerve sheath tumors (MPNSTs). A genetically engineered mouse model that accurately models plexiform neurofibroma-MPNST progression would facilitate the identification of somatic mutations driving this process. We have previously reported that transgenic mice overexpressing the growth factor neuregulin-1 in Schwann cells (P0-GGFβ3 mice) develop MPNSTs. more...

Organism:

Mus musculus

Type:

Genome variation profiling by array

Platform:

GPL11288

12 Samples

Download data: TXT

(Submitter supplied) Background: Malignant peripheral nerve sheath tumors (MPNST) are soft-tissue sarcomas that can arise either sporadically or in association with neurofibromatosis type 1 (NF1). These aggressive malignancies confer poor survival, with no effective therapy available. Methods: We generated five patient-derived MPNST orthoxenograft models (three NF1-related and two sporadic) and performed an exhaustive histological and molecular characterization of primary MPNSTs and their corresponding orthoxenografts. more...

Organism:

Homo sapiens

Type:

Expression profiling by array

Platform:

GPL6244

12 Samples

Download data: CEL