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Open AccessNf1 mutation disrupts activity-dependent oligodendroglial plasticity and motor learning in mice
Neurogenetic disorders, such as neurofibromatosis type 1 (NF1), can cause cognitive and motor impairments, traditionally attributed to intrinsic neuronal defects such as disruption of synaptic function. Activi...
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Article
Low-grade glioma in children with neurofibromatosis type 1: surveillance, treatment indications, management, and future directions
Neurofibromatosis type 1 (NF1) is an autosomal dominant cancer predisposition syndrome characterized by the development of both central and peripheral nervous system tumors. Low-grade glioma (LGG) is the most ...
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Article
Open AccessBrain injury drives optic glioma formation through neuron-glia signaling
Tissue injury and tumorigenesis share many cellular and molecular features, including immune cell (T cells, monocytes) infiltration and inflammatory factor (cytokines, chemokines) elaboration. Their common pat...
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Article
Open AccessMonocyte depletion enhances neutrophil influx and proneural to mesenchymal transition in glioblastoma
Myeloid cells comprise the majority of immune cells in tumors, contributing to tumor growth and therapeutic resistance. Incomplete understanding of myeloid cells response to tumor driver mutation and therapeut...
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Article
Open AccessNeurofibromatosis type 1-dependent alterations in mouse microglia function are not cell-intrinsic
We previously discovered a sex-by-genotype defect in microglia function using a heterozygous germline knockout mouse model of Neurofibromatosis type 1 (Nf1 ± mice), in which only microglia from male Nf1 ± mice ex...
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MRI-guided laser interstitial thermal therapy for deep-seated gliomas in children with neurofibromatosis type 1: report of two cases
Nearly a quarter of neurofibromatosis type 1 (NF 1)- associated diencephalic low-grade tumors are refractory to chemotherapy. Addition of alternative treatment options with laser interstitial thermal therapy w...
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Article
Open AccessHuman induced pluripotent stem cell engineering establishes a humanized mouse platform for pediatric low-grade glioma modeling
A major obstacle to identifying improved treatments for pediatric low-grade brain tumors (gliomas) is the inability to reproducibly generate human xenografts. To surmount this barrier, we leveraged human induc...
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Article
Open AccessNeuronal hyperexcitability drives central and peripheral nervous system tumor progression in models of neurofibromatosis-1
Neuronal activity is emerging as a driver of central and peripheral nervous system cancers. Here, we examined neuronal physiology in mouse models of the tumor predisposition syndrome Neurofibromatosis-1 (NF1),...
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Article
Open AccessAsthma reduces glioma formation by T cell decorin-mediated inhibition of microglia
To elucidate the mechanisms underlying the reduced incidence of brain tumors in children with Neurofibromatosis type 1 (NF1) and asthma, we leverage Nf1 optic pathway glioma (Nf1OPG) mice, human and mouse RNAseq ...
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Article
Open AccessBRAF mutations may identify a clinically distinct subset of glioblastoma
Glioblastoma (GBM) is the most common primary malignant brain tumor in adults. Prior studies examining the mutational landscape of GBM revealed recurrent alterations in genes that regulate the same growth cont...
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Article
NF1 mutation drives neuronal activity-dependent initiation of optic glioma
Neurons have recently emerged as essential cellular constituents of the tumour microenvironment, and their activity has been shown to increase the growth of a diverse number of solid tumours1. Although the role o...
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Article
Integrated molecular and clinical analysis of low-grade gliomas in children with neurofibromatosis type 1 (NF1)
Low-grade gliomas (LGGs) are the most common childhood brain tumor in the general population and in individuals with the Neurofibromatosis type 1 (NF1) cancer predisposition syndrome. Surgical biopsy is rarely...
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Article
Open AccessShared developmental gait disruptions across two mouse models of neurodevelopmental disorders
Motor deficits such as abnormal gait are an underappreciated yet characteristic phenotype of many neurodevelopmental disorders (NDDs), including Williams Syndrome (WS) and Neurofibromatosis Type 1 (NF1). Compa...
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Article
Cabozantinib for neurofibromatosis type 1–related plexiform neurofibromas: a phase 2 trial
Neurofibromatosis type 1 (NF1) plexiform neurofibromas (PNs) are progressive, multicellular neoplasms that cause morbidity and may transform to sarcoma. Treatment of Nf1fl/fl;Postn-Cre mice with cabozantinib, an ...
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Open AccessMidkine activation of CD8+ T cells establishes a neuron–immune–cancer axis responsible for low-grade glioma growth
Brain tumors (gliomas) are heterogeneous cellular ecosystems, where non-neoplastic monocytic cells have emerged as key regulators of tumor maintenance and progression. However, relative to macrophages/microgli...
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Open AccessCorrection to: Comprehensive gene expression meta-analysis identifies signature genes that distinguish microglia from peripheral monocytes/macrophages in health and glioma
The original publication of this article [1] contained 3 minor errors in Figs. 1, 3 and 5. In this correction article the updated figures are published. The figure captions describe the updated information in ...
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The Sociobiology of Brain Tumors
Brain tumors are complex cellular ecosystems, composed of populations of both neoplastic and non-neoplastic cell types. While the contributions of the cancer cells in low-grade and high-grade gliomas have been...
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Article
Variability of Betweenness Centrality and Its Effect on Identifying Essential Genes
This paper begins to build a theoretical framework that would enable the pharmaceutical industry to use network complexity measures as a way to identify drug targets. The variability of a betweenness measure f...
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Reply to ‘Assembling the brain trust: the multidisciplinary imperative in neuro-oncology’
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Open AccessChallenges to curing primary brain tumours
Despite decades of research, brain tumours remain among the deadliest of all forms of cancer. The ability of these tumours to resist almost all conventional and novel treatments relates, in part, to the unique...