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Temporal, spatial, and genetic constraints contribute to the patterning and penetrance of murine Neurofibromatosis-1 optic glioma

ABSTRACT: Temporal, spatial, and genetic constraints contribute to the patterning and penetrance of murine Neurofibromatosis-1 optic glioma

PROVIDER: PRJNA631206 | ENA |

REPOSITORIES: ENA

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			Action	DRS
	SRR11734210.fastq.gz	Fastqsanger.gz
	SRR11734211.fastq.gz	Fastqsanger.gz
	SRR11734212.fastq.gz	Fastqsanger.gz
	SRR11734213.fastq.gz	Fastqsanger.gz
	SRR11734214.fastq.gz	Fastqsanger.gz

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Temporal, spatial, and genetic constraints contribute to the patterning and penetrance of murine neurofibromatosis-1 optic glioma.

Project description:BackgroundBrain tumors are the most common solid tumors of childhood, but little is understood about the factors that influence their development. Pediatric low-grade gliomas in particular display unique temporal and spatial localization associated with different genetic mutations (eg, BRAF genomic alterations, mutations in the neurofibromatosis type 1 [NF1] gene) for reasons that remain unclear. NF1 low-grade gliomas typically arise in the optic pathway of young children as optic pathway gliomas (OPGs), likely from a cell of origin that resides within the third ventricular zone (TVZ). However, the factors that contribute to their distinct temporal patterning and penetrance have not been adequately explored.MethodsTVZ neuroglial progenitor cells (NPCs) were analyzed over the course of mouse brain development. Progenitors isolated by fluorescence-activated cell sorting (FACS) were assessed for functional and molecular differences. The impact of different germline Nf1 mutations on TVZ NPC properties was analyzed using genetically engineered mice.ResultsWe identify 3 individual factors that could each contribute to Nf1 optic glioma temporal patterning and penetrance. First, there are 3 functionally and molecularly distinct populations of mouse TVZ NPCs, one of which ("M" cells) exhibits the highest clonogenic incidence, proliferation, and abundance during embryogenesis. Second, TVZ NPC proliferation dramatically decreases after birth. Third, germline Nf1 mutations differentially increase TVZ NPC proliferation during embryogenesis.ConclusionsThe unique temporal patterning and penetrance of Nf1 optic glioma reflects the combined effects of TVZ NPC population composition, time-dependent changes in progenitor proliferation, and the differential impact of the germline Nf1 mutation on TVZ NPC expansion.

| S-EPMC8041339 | biostudies-literature

Temporal, spatial, and genetic constraints contribute to the patterning and penetrance of murine Neurofibromatosis-1 optic glioma

Project description:Contributing factors to pediatric glioma formation

2021-04-21 | GSE149946 | GEO

Effect of maternal obesogenic diet exposure on murine Neurofibromatosis Type 1 optic pathway glioma

Project description:Pediatric low-grade glioma incidence has been rising in the U.S., mirroring the rising rates of pediatric and maternal obesity. Recently, children of obese mothers were demonstrated to develop brain tumors at higher rates. Importantly, obesity in the U.S. is largely driven by diet, given the abudnance of high fat and high sugar food choices. We identified that combined maternal and postnatal obesogenic (Ob) diet exposure accelerated glioma formation in murine Neurofibromatosis type 1 (Nf1) optic pathway glioma (OPG)-forming mice, a model of pediatric glioma formation. Postnatal dietary exposure alone could not fully recaptiulate these effects, indicating maternal exposure was critical for this phenotype. To determine how Ob exposure affected gene expression in the optic nerve, we isolated Ob-exposed versus CD-exposed optic nerves and performed RNASeq. Enriched pathways included nervous system development, neuronal signaling and appetite regulation.

2025-05-27 | GSE269517 | GEO

OXPHOS bioenergetic compensation does not explain disease penetrance in Leber hereditary optic neuropathy

Project description:OXPHOS bioenergetic compensation does not explain disease penetrance in Leber hereditary optic neuropathy

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Effect of maternal obesogenic diet exposure on murine Neurofibromatosis Type 1 optic pathway glioma

Project description:Effect of maternal obesogenic diet exposure on murine Neurofibromatosis Type 1 optic pathway glioma

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Fine-tuning mechanical constraints reveals uncoupled patterning and gene expression programs in murine gastruloids

Project description:Fine-tuning mechanical constraints reveals uncoupled patterning and gene expression programs in murine gastruloids

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Head-to-head preclinical treatment design prioritizes promising therapies for Neurofibromatosis 1 optic glioma clinical translation

Project description:Head-to-head preclinical treatment design prioritizes promising therapies for Neurofibromatosis 1 optic glioma clinical translation

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Fine-tuning mechanical constraints reveals uncoupled patterning and gene expression programs in murine gastruloids [RNAseq_concentration]

Project description:Fine-tuning mechanical constraints reveals uncoupled patterning and gene expression programs in murine gastruloids [RNAseq_concentration]

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Fine-tuning mechanical constraints reveals uncoupled patterning and gene expression programs in murine gastruloids [RNAseq_time]

Project description:Fine-tuning mechanical constraints reveals uncoupled patterning and gene expression programs in murine gastruloids [RNAseq_time]

| PRJNA1216285 | ENA

Transcription profiling of human neurofibromatosis type 1

Project description:Genes involved in tumorigenesis associated with neurofibromatosis type 1.

2007-01-12 | E-TABM-69 | biostudies-arrayexpress