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Runx1 and Runx3 cooperatively repress Pmp22 to drive neurofibromagenesis

ABSTRACT: Runx1 and Runx3 cooperatively repress Pmp22 to drive neurofibromagenesis

PROVIDER: PRJNA506263 | ENA |

REPOSITORIES: ENA

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			Action	DRS
	SRR8218080.fastq.gz	Fastqsanger.gz
	SRR8218061.fastq.gz	Fastqsanger.gz
	SRR8218062.fastq.gz	Fastqsanger.gz
	SRR8218079.fastq.gz	Fastqsanger.gz
	SRR8223311_1.fastq.gz	Fastqsanger.gz

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Runx1 and Runx3 cooperatively repress Pmp22 to drive neurofibromagenesis [microarray]

Project description:Runx1 and Runx3 cooperatively repress Pmp22 to drive neurofibromagenesis [microarray]

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Runx1 and Runx3 cooperatively repress Pmp22 to drive neurofibromagenesis [microarray]

Project description:Neurofibromatosis type 1 (NF1) patients are predisposed to develop neurofibromas but the underlying molecular mechanism(s) of neurofibromagenesis are not fully understood. We showed that dual genetic deletion of Runx1 (Rx1) and Runx3 (Rx3) in Schwann cells (SCs) and Schwann cell precursors (SCPs) significantly delayed neurofibromagenesis and prolonged mouse survival. We identified peripheral myelin protein 22 (Pmp22/Gas3) related to tumor initiation. Knockdown of Pmp22 with shRNAs increased Rx1fl/fl;Rx3fl/fl;Nf1fl/fl;DhhCre sphere numbers and enabled significantly more neurofibroma like micro-lesions on transplantation. Conversely, overexpression of Pmp22 in mouse neurofibroma SCs decreased proliferation. Mechanistically, Rx1/3 regulated alterative Pmp22 promoter usage and reduced post transcriptional expression of Pmp22. Finally, pharmacological inhibition of Runx/core binding factor beta (Cbf-β) activity significantly reduced neurofibroma volume in vivo. Thus, we identified a novel signaling pathway involving Rx1/3 suppression of Pmp22 in neurofibroma initiation and/or maintenance. Targeting disruption of Runx/Cbf-β interaction might provide a novel therapy for neurofibroma patients.

2019-05-01 | GSE122773 | GEO

Runx1 and Runx3 cooperatively repress Pmp22 to drive neurofibromagenesis [ChIP-seq]

Project description:Runx1 and Runx3 cooperatively repress Pmp22 to drive neurofibromagenesis [ChIP-seq]

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Runx1 and Runx3 cooperatively repress Pmp22 to drive neurofibromagenesis [RNA-seq]

Project description:Runx1 and Runx3 cooperatively repress Pmp22 to drive neurofibromagenesis [RNA-seq]

| PRJNA506269 | ENA

Runx1 and Runx3 cooperatively repress Pmp22 to drive neurofibromagenesis [ATAC-seq]

Project description:Runx1 and Runx3 cooperatively repress Pmp22 to drive neurofibromagenesis [ATAC-seq]

| PRJNA506265 | ENA

Runx1 and Runx3 cooperatively repress Pmp22 to drive neurofibromagenesis [ATAC-seq]

2019-05-01 | GSE122776 | GEO

Runx1 and Runx3 cooperatively repress Pmp22 to drive neurofibromagenesis [ChIP-seq]

2019-05-01 | GSE122775 | GEO

Runx1 and Runx3 cooperatively repress Pmp22 to drive neurofibromagenesis [RNA-seq]

2019-05-01 | GSE122774 | GEO

Runx1 and Runx3 cooperatively repress Pmp22 to drive neurofibromagenesis

Project description:This SuperSeries is composed of the SubSeries listed below.

2019-05-01 | GSE122777 | GEO

RUNX represses <i>Pmp22</i> to drive neurofibromagenesis.

Project description:Patients with neurofibromatosis type 1 (NF1) are predisposed to develop neurofibromas, but the underlying molecular mechanisms of neurofibromagenesis are not fully understood. We showed dual genetic deletion of Runx1 and Runx3 in Schwann cells (SCs) and SC precursors delayed neurofibromagenesis and prolonged mouse survival. We identified peripheral myelin protein 22 (Pmp22/Gas3) related to neurofibroma initiation. Knockdown of Pmp22 with short hairpin RNAs increased Runx1fl/fl;Runx3fl/fl;Nf1fl/fl;DhhCre tumor-derived sphere numbers and enabled significantly more neurofibroma-like microlesions on transplantation. Conversely, overexpression of Pmp22 in mouse neurofibroma SCs decreased cell proliferation. Mechanistically, RUNX1/3 regulated alternative promoter usage and induced levels of protein expression of Pmp22 to control SC growth. Last, pharmacological inhibition of RUNX/core-binding factor β (CBFB) activity significantly reduced neurofibroma volume in vivo. Thus, we identified a signaling pathway involving RUNX1/3 suppression of Pmp22 in neurofibroma initiation and/or maintenance. Targeting disruption of RUNX/CBFB interaction might provide a novel therapy for patients with neurofibroma.

| S-EPMC6482019 | biostudies-literature

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