Published in PLoS One on June 10, 2013
RASopathies: unraveling mechanisms with animal models. Dis Model Mech (2015) 0.84
A Mutation Associated with Stuttering Alters Mouse Pup Ultrasonic Vocalizations. Curr Biol (2016) 0.81
Behavioral and cognitive outcomes for clinical trials in children with neurofibromatosis type 1. Neurology (2015) 0.80
Response inhibition in Attention deficit disorder and neurofibromatosis type 1 - clinically similar, neurophysiologically different. Sci Rep (2017) 0.76
Conflict processing in juvenile patients with neurofibromatosis type 1 (NF1) and healthy controls - Two pathways to success. Neuroimage Clin (2017) 0.75
The diagnostic evaluation and multidisciplinary management of neurofibromatosis 1 and neurofibromatosis 2. JAMA (1997) 8.30
Ablation of NF1 function in neurons induces abnormal development of cerebral cortex and reactive gliosis in the brain. Genes Dev (2001) 4.68
Targeted disruption of the neurofibromatosis type-1 gene leads to developmental abnormalities in heart and various neural crest-derived tissues. Genes Dev (1994) 3.40
The neurobiological basis of spontaneous alternation. Neurosci Biobehav Rev (2002) 2.81
Neurofibromin regulation of ERK signaling modulates GABA release and learning. Cell (2008) 2.65
The HMG-CoA reductase inhibitor lovastatin reverses the learning and attention deficits in a mouse model of neurofibromatosis type 1. Curr Biol (2005) 2.62
Validity of head-dipping as a measure of exploration in a modified hole-board. Psychopharmacologia (1975) 2.46
Optic nerve glioma in mice requires astrocyte Nf1 gene inactivation and Nf1 brain heterozygosity. Cancer Res (2003) 2.30
Apoptotic neurodegeneration induced by ethanol in neonatal mice is associated with profound learning/memory deficits in juveniles followed by progressive functional recovery in adults. Neurobiol Dis (2004) 2.24
The nature and frequency of cognitive deficits in children with neurofibromatosis type 1. Neurology (2005) 1.98
Hippocampal long-term depression and long-term potentiation encode different aspects of novelty acquisition. Proc Natl Acad Sci U S A (2004) 1.93
Novelty acquisition is associated with induction of hippocampal long-term depression. Proc Natl Acad Sci U S A (1999) 1.85
A role in learning for SRF: deletion in the adult forebrain disrupts LTD and the formation of an immediate memory of a novel context. Neuron (2006) 1.83
Dopamine D1/D5 receptors gate the acquisition of novel information through hippocampal long-term potentiation and long-term depression. J Neurosci (2006) 1.74
Development of a mouse test for repetitive, restricted behaviors: relevance to autism. Behav Brain Res (2007) 1.71
Altered stress-induced anxiety in adenylyl cyclase type VIII-deficient mice. J Neurosci (2000) 1.65
Cognitive function and academic performance in neurofibromatosis. 1: consensus statement from the NF1 Cognitive Disorders Task Force. Neurology (1997) 1.52
Olfaction in Parkinson's disease and related disorders. Neurobiol Dis (2011) 1.51
Treatment of ADHD in neurofibromatosis type 1. Dev Med Child Neurol (2002) 1.47
Learning disabilities in children with neurofibromatosis type 1: subtypes, cognitive profile, and attention-deficit-hyperactivity disorder. Dev Med Child Neurol (2006) 1.42
Impaired spatial learning and defective theta burst induced LTP in mice lacking fibroblast growth factor 14. Neurobiol Dis (2007) 1.34
Reduced striatal dopamine underlies the attention system dysfunction in neurofibromatosis-1 mutant mice. Hum Mol Genet (2010) 1.18
The immediate early gene early growth response gene 3 mediates adaptation to stress and novelty. Neuroscience (2007) 1.17
Restored plasticity in a mouse model of neurofibromatosis type 1 via inhibition of hyperactive ERK and CREB. Eur J Neurosci (2007) 1.10
Core features of frontotemporal dementia recapitulated in progranulin knockout mice. Neurobiol Dis (2011) 1.10
The disruption of Celf6, a gene identified by translational profiling of serotonergic neurons, results in autism-related behaviors. J Neurosci (2013) 1.05
Dopamine systems in the forebrain. Adv Exp Med Biol (2009) 1.00
Loss of RBPj in postnatal excitatory neurons does not cause neurodegeneration or memory impairments in aged mice. PLoS One (2012) 0.98
Dopamine deficiency underlies learning deficits in neurofibromatosis-1 mice. Ann Neurol (2012) 0.97
PET imaging for attention deficit preclinical drug testing in neurofibromatosis-1 mice. Exp Neurol (2011) 0.94
A systematic review on olfaction in child and adolescent psychiatric disorders. J Neural Transm (Vienna) (2012) 0.94
Hippocampal long-term depression as an index of spatial working memory. Eur J Neurosci (2002) 0.93
Activity, non-selective attention and emotionality in dopamine D2/D3 receptor knock-out mice. Behav Brain Res (2002) 0.87
Non-selective attention in a rat model of hyperactivity and attention deficit: subchronic methylphenydate and nitric oxide synthesis inhibitor treatment. Neurosci Biobehav Rev (2000) 0.86
A rotating holeboard procedure for testing drug effects on spatial learning and memory in mice. Brain Res Brain Res Protoc (1997) 0.81
Early exposure to common anesthetic agents causes widespread neurodegeneration in the developing rat brain and persistent learning deficits. J Neurosci (2003) 7.42
Subtypes of medulloblastoma have distinct developmental origins. Nature (2010) 5.94
Astrocytes as determinants of disease progression in inherited amyotrophic lateral sclerosis. Nat Neurosci (2008) 5.89
Rapamycin prevents epilepsy in a mouse model of tuberous sclerosis complex. Ann Neurol (2008) 5.55
International consensus statement on malignant peripheral nerve sheath tumors in neurofibromatosis. Cancer Res (2002) 4.27
Neurofibromatosis 2. Curr Opin Neurol (2003) 3.46
Astrocyte-specific TSC1 conditional knockout mice exhibit abnormal neuronal organization and seizures. Ann Neurol (2002) 3.37
Neurofibromatosis type 1 revisited. Pediatrics (2009) 3.06
Distinct genetic signatures among pilocytic astrocytomas relate to their brain region origin. Cancer Res (2007) 3.05
Sex Is a major determinant of neuronal dysfunction in neurofibromatosis type 1. Ann Neurol (2014) 3.01
Optic pathway gliomas in neurofibromatosis-1: controversies and recommendations. Ann Neurol (2007) 2.75
Pten loss causes hypertrophy and increased proliferation of astrocytes in vivo. Cancer Res (2004) 2.75
Astrocyte-specific inactivation of the neurofibromatosis 1 gene (NF1) is insufficient for astrocytoma formation. Mol Cell Biol (2002) 2.65
Neurofibromin regulation of ERK signaling modulates GABA release and learning. Cell (2008) 2.65
Oncogenic BRAF mutation with CDKN2A inactivation is characteristic of a subset of pediatric malignant astrocytomas. Cancer Res (2010) 2.56
Cardiovascular disease in neurofibromatosis 1: report of the NF1 Cardiovascular Task Force. Genet Med (2002) 2.39
Optic nerve glioma in mice requires astrocyte Nf1 gene inactivation and Nf1 brain heterozygosity. Cancer Res (2003) 2.30
Acquired deficit of forebrain glucocorticoid receptor produces depression-like changes in adrenal axis regulation and behavior. Proc Natl Acad Sci U S A (2004) 2.11
Proteomic analysis reveals hyperactivation of the mammalian target of rapamycin pathway in neurofibromatosis 1-associated human and mouse brain tumors. Cancer Res (2005) 2.02
Neurosteroid access to the GABAA receptor. J Neurosci (2005) 1.95
Neurofibromatosis-1 regulates neuronal and glial cell differentiation from neuroglial progenitors in vivo by both cAMP- and Ras-dependent mechanisms. Cell Stem Cell (2007) 1.92
Treatment with an amyloid-beta antibody ameliorates plaque load, learning deficits, and hippocampal long-term potentiation in a mouse model of Alzheimer's disease. J Neurosci (2005) 1.90
Inactivation of NF1 in CNS causes increased glial progenitor proliferation and optic glioma formation. Development (2005) 1.85
Neurofibromatosis-1 (Nf1) heterozygous brain microglia elaborate paracrine factors that promote Nf1-deficient astrocyte and glioma growth. Hum Mol Genet (2007) 1.80
Large-scale molecular comparison of human schwann cells to malignant peripheral nerve sheath tumor cell lines and tissues. Cancer Res (2006) 1.80
Impaired glial glutamate transport in a mouse tuberous sclerosis epilepsy model. Ann Neurol (2003) 1.72
Spatiotemporal differences in CXCL12 expression and cyclic AMP underlie the unique pattern of optic glioma growth in neurofibromatosis type 1. Cancer Res (2007) 1.69
Nucleophosmin mediates mammalian target of rapamycin-dependent actin cytoskeleton dynamics and proliferation in neurofibromin-deficient astrocytes. Cancer Res (2007) 1.65
Nectin-like proteins mediate axon Schwann cell interactions along the internode and are essential for myelination. J Cell Biol (2007) 1.63
Integrative genomic analysis identifies NDRG2 as a candidate tumor suppressor gene frequently inactivated in clinically aggressive meningioma. Cancer Res (2005) 1.63
Serine 518 phosphorylation modulates merlin intramolecular association and binding to critical effectors important for NF2 growth suppression. Oncogene (2004) 1.61
Mechanisms of neurosteroid interactions with GABA(A) receptors. Pharmacol Ther (2007) 1.60
Protein 4.1 tumor suppressors: getting a FERM grip on growth regulation. J Cell Sci (2002) 1.59
Immunohistochemical analysis supports a role for INI1/SMARCB1 in hereditary forms of schwannomas, but not in solitary, sporadic schwannomas. Brain Pathol (2008) 1.59
Circadian clock proteins regulate neuronal redox homeostasis and neurodegeneration. J Clin Invest (2013) 1.57
RAS pathway activation and an oncogenic RAS mutation in sporadic pilocytic astrocytoma. Neurology (2005) 1.55
Tsc2 gene inactivation causes a more severe epilepsy phenotype than Tsc1 inactivation in a mouse model of tuberous sclerosis complex. Hum Mol Genet (2010) 1.53
Predictive value of café au lait macules at initial consultation in the diagnosis of neurofibromatosis type 1. Arch Dermatol (2009) 1.52
Epileptogenesis and reduced inward rectifier potassium current in tuberous sclerosis complex-1-deficient astrocytes. Epilepsia (2005) 1.48
Merlin is a potent inhibitor of glioma growth. Cancer Res (2008) 1.47
Loss of tuberous sclerosis complex 1 (Tsc1) expression results in increased Rheb/S6K pathway signaling important for astrocyte cell size regulation. Glia (2004) 1.47
Preclinical cancer therapy in a mouse model of neurofibromatosis-1 optic glioma. Cancer Res (2008) 1.47
Microarray analyses reveal regional astrocyte heterogeneity with implications for neurofibromatosis type 1 (NF1)-regulated glial proliferation. Glia (2009) 1.46
Defining future directions in spinal cord tumor research: proceedings from the National Institutes of Health workshop. J Neurosurg Spine (2010) 1.45
Neurofibromatosis-1 regulates neuroglial progenitor proliferation and glial differentiation in a brain region-specific manner. Genes Dev (2010) 1.43
Perspective: Upcoming paradigm shifts for psychiatry in clinical care, research, and education. Acad Med (2012) 1.43
Nf2 gene inactivation in arachnoidal cells is rate-limiting for meningioma development in the mouse. Genes Dev (2002) 1.43
High-grade glioma formation results from postnatal pten loss or mutant epidermal growth factor receptor expression in a transgenic mouse glioma model. Cancer Res (2006) 1.42
Molecular pathogenesis of meningiomas. J Neurooncol (2004) 1.42
Gliomas in neurofibromatosis type 1: a clinicopathologic study of 100 patients. J Neuropathol Exp Neurol (2008) 1.41
Visual function and optic pathway glioma: a critical response. JAMA Ophthalmol (2013) 1.41
Neurofibromatosis 1: from lab bench to clinic. Pediatr Neurol (2005) 1.40
Abnormal glutamate homeostasis and impaired synaptic plasticity and learning in a mouse model of tuberous sclerosis complex. Neurobiol Dis (2007) 1.37
Proceedings from the 2009 genetic syndromes of the Ras/MAPK pathway: From bedside to bench and back. Am J Med Genet A (2010) 1.36
Differential NF1, p16, and EGFR patterns by interphase cytogenetics (FISH) in malignant peripheral nerve sheath tumor (MPNST) and morphologically similar spindle cell neoplasms. J Neuropathol Exp Neurol (2002) 1.36
Zinc modulates bidirectional hippocampal plasticity by effects on NMDA receptors. J Neurosci (2006) 1.35
Mixed-lineage kinase 3 regulates B-Raf through maintenance of the B-Raf/Raf-1 complex and inhibition by the NF2 tumor suppressor protein. Proc Natl Acad Sci U S A (2006) 1.35
Identification of dominant negative mutants of Rheb GTPase and their use to implicate the involvement of human Rheb in the activation of p70S6K. J Biol Chem (2003) 1.35
Neurofibromatosis 2 (NF2) tumor suppressor merlin inhibits phosphatidylinositol 3-kinase through binding to PIKE-L. Proc Natl Acad Sci U S A (2004) 1.34
Gliomas in patients with neurofibromatosis type 1. Expert Rev Neurother (2009) 1.33
Neurofibromatosis type 1 - a model for nervous system tumour formation? Nat Rev Cancer (2005) 1.33
Neurofibromin regulates neural stem cell proliferation, survival, and astroglial differentiation in vitro and in vivo. J Neurosci (2005) 1.33
Frequent promoter hypermethylation and transcriptional downregulation of the NDRG2 gene at 14q11.2 in primary glioblastoma. Int J Cancer (2008) 1.33
Effect of merlin phosphorylation on neurofibromatosis 2 (NF2) gene function. Oncogene (2004) 1.32
Detection and measurement of neurofibromatosis-1 mouse optic glioma in vivo. Neuroimage (2007) 1.32
Neurosteroid analogues. 12. Potent enhancement of GABA-mediated chloride currents at GABAA receptors by ent-androgens. Eur J Med Chem (2007) 1.31
Akt phosphorylation regulates the tumour-suppressor merlin through ubiquitination and degradation. Nat Cell Biol (2007) 1.31
Glioma formation in neurofibromatosis 1 reflects preferential activation of K-RAS in astrocytes. Cancer Res (2005) 1.30
Neurofibromatosis 1. Neurol Clin (2002) 1.30
Pediatric glioma-associated KIAA1549:BRAF expression regulates neuroglial cell growth in a cell type-specific and mTOR-dependent manner. Genes Dev (2012) 1.30
Molecular characterization of human meningiomas by gene expression profiling using high-density oligonucleotide microarrays. Am J Pathol (2002) 1.29
Optic pathway gliomas in neurofibromatosis type 1: the effect of presenting symptoms on outcome. Am J Med Genet A (2003) 1.28
Visual outcomes in children with neurofibromatosis type 1-associated optic pathway glioma following chemotherapy: a multicenter retrospective analysis. Neuro Oncol (2012) 1.27
The natural history and treatment of epilepsy in a murine model of tuberous sclerosis. Epilepsia (2007) 1.26
Advances in the treatment of neurofibromatosis-associated tumours. Nat Rev Clin Oncol (2013) 1.26
Expression of ICAM-1, TNF-alpha, NF kappa B, and MAP kinase in tubers of the tuberous sclerosis complex. Neurobiol Dis (2003) 1.26
Innate neural stem cell heterogeneity determines the patterning of glioma formation in children. Cancer Cell (2012) 1.26
Defective cAMP generation underlies the sensitivity of CNS neurons to neurofibromatosis-1 heterozygosity. J Neurosci (2010) 1.25
Diverse voltage-sensitive dyes modulate GABAA receptor function. J Neurosci (2010) 1.24
Slow actions of neuroactive steroids at GABAA receptors. J Neurosci (2004) 1.24
Repetitive hypoglycemia in young rats impairs hippocampal long-term potentiation. Pediatr Res (2003) 1.24
Increased c-Jun-NH2-kinase signaling in neurofibromatosis-1 heterozygous microglia drives microglia activation and promotes optic glioma proliferation. Cancer Res (2008) 1.24
The neurofibromatosis 2 tumor suppressor gene product, merlin, regulates human meningioma cell growth by signaling through YAP. Neoplasia (2008) 1.23
Selective antagonism of 5alpha-reduced neurosteroid effects at GABA(A) receptors. Mol Pharmacol (2004) 1.23
Recent advances in neurofibromatosis type 1. Curr Opin Neurol (2004) 1.23
Neurofibromatosis type 1 (NF1): diagnosis and management. Handb Clin Neurol (2013) 1.22
Neuroexcitatory actions of Tamiflu and its carboxylate metabolite. Neurosci Lett (2007) 1.22
Astrocyte loss of mutant SOD1 delays ALS disease onset and progression in G85R transgenic mice. Hum Mol Genet (2010) 1.21
The neurofibromatosis 1 gene product neurofibromin regulates pituitary adenylate cyclase-activating polypeptide-mediated signaling in astrocytes. J Neurosci (2003) 1.21
The sticky issue of neurosteroids and GABA(A) receptors. Trends Neurosci (2010) 1.21
Neurofibromatosis-1 heterozygosity increases microglia in a spatially and temporally restricted pattern relevant to mouse optic glioma formation and growth. J Neuropathol Exp Neurol (2011) 1.20
Neurofibromatosis-1 regulates mTOR-mediated astrocyte growth and glioma formation in a TSC/Rheb-independent manner. Proc Natl Acad Sci U S A (2011) 1.20
Dimethyl sulfoxide (DMSO) produces widespread apoptosis in the developing central nervous system. Neurobiol Dis (2008) 1.20
Oligodendrogliomas result from the expression of an activated mutant epidermal growth factor receptor in a RAS transgenic mouse astrocytoma model. Cancer Res (2003) 1.20
Cyclic AMP suppression is sufficient to induce gliomagenesis in a mouse model of neurofibromatosis-1. Cancer Res (2010) 1.20
Array-based comparative genomic hybridization identifies CDK4 and FOXM1 alterations as independent predictors of survival in malignant peripheral nerve sheath tumor. Clin Cancer Res (2011) 1.20
Reduced striatal dopamine underlies the attention system dysfunction in neurofibromatosis-1 mutant mice. Hum Mol Genet (2010) 1.18