GEO DataSets

(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) NF1 deficiency drives metabolic reprogramming in ER+ breast cancer. This reprogramming is characterized by oxidative ATP constraints, glutamine TCA influx, and lipid pool expansion, and these metabolic changes introduce novel metabolic-to-targeted inhibitor synergies.

Organism:

Homo sapiens

Type:

Expression profiling by high throughput sequencing

Platform:

GPL24676

6 Samples

Download data: TSV

(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) Malignant peripheral nerve sheath tumor (MPNST) is a type of soft tissue sarcoma that occurs in carriers of mutations in the neurofibromatosis type I gene (Nf1) as well as sporadically.　Plexiform neurofibromas in NF1 patients have a significant risk of developing into MPNSTs leading to increased morbidity and mortality from this syndrome.　Surgery is the primary intervention but it is not always effective due to the tendency of MPNSTs to infiltrate the surrounding tissue or grow in an inoperable location. more...

Organism:

Homo sapiens

Type:

Expression profiling by array

Platform:

GPL4133

24 Samples

Download data: TXT

(Submitter supplied) Analysis of gene expression in pathologically confirmed glioblastoma (GBM) samples. These data were used to test a classifier that was generated to distinguish GBM tumor samples with loss of neurofibromin 1 (NF1) function

Organism:

Homo sapiens

Type:

Expression profiling by array

Platform:

GPL17586

12 Samples

Download data: CEL, TXT

(Submitter supplied) The SWI/SNF-family chromatin remodeling protein ATRX is a tumor suppressor in sarcomas, gliomas and other malignancies. Its loss of function facilitates the alternative lengthening of telomeres (ALT) pathway in tumor cells, while it also affects Polycomb repressive complex 2 (PRC2) silencing of its target genes. To further define the role of inactivating ATRX mutations in carcinogenesis, we knocked out atrx in our previously published p53/nf1-deficient zebrafish line that develops malignant peripheral nerve sheath tumors and gliomas. more...

Organism:

Danio rerio

Type:

Expression profiling by high throughput sequencing

Platform:

GPL20828

10 Samples

Download data: FPKM_TRACKING, TXT