Published in Neurobiol Dis on September 19, 2007
Downregulated microRNA-200a in meningiomas promotes tumor growth by reducing E-cadherin and activating the Wnt/beta-catenin signaling pathway. Mol Cell Biol (2009) 2.06
NF2/merlin is a novel negative regulator of mTOR complex 1, and activation of mTORC1 is associated with meningioma and schwannoma growth. Mol Cell Biol (2009) 1.78
Merlin: a tumour suppressor with functions at the cell cortex and in the nucleus. EMBO Rep (2012) 1.30
Missense mutations in the NF2 gene result in the quantitative loss of merlin protein and minimally affect protein intrinsic function. Proc Natl Acad Sci U S A (2011) 1.13
Comparative protein profiling reveals minichromosome maintenance (MCM) proteins as novel potential tumor markers for meningiomas. J Proteome Res (2010) 0.91
A high-throughput kinome screen reveals serum/glucocorticoid-regulated kinase 1 as a therapeutic target for NF2-deficient meningiomas. Oncotarget (2015) 0.79
NF2/Merlin mediates contact-dependent inhibition of EGFR mobility and internalization via cortical actomyosin. J Cell Biol (2015) 0.79
Global Proteome and Phospho-proteome Analysis of Merlin-deficient Meningioma and Schwannoma Identifies PDLIM2 as a Novel Therapeutic Target. EBioMedicine (2017) 0.75
2016 William Allan Award: Human Disease Research: Genetic Cycling and Re-cycling. Am J Hum Genet (2017) 0.75
PDGF activation in PGDS-positive arachnoid cells induces meningioma formation in mice promoting tumor progression in combination with Nf2 and Cdkn2ab loss. Oncotarget (2015) 0.75
A new patient-derived orthotopic malignant meningioma model treated with oncolytic herpes simplex virus. Neuro Oncol (2016) 0.75
A biomarker that identifies senescent human cells in culture and in aging skin in vivo. Proc Natl Acad Sci U S A (1995) 32.89
The small GTP-binding protein rac regulates growth factor-induced membrane ruffling. Cell (1992) 28.36
Rho GTPases and the actin cytoskeleton. Science (1998) 28.19
Crucial role of p53-dependent cellular senescence in suppression of Pten-deficient tumorigenesis. Nature (2005) 15.83
Tumour biology: senescence in premalignant tumours. Nature (2005) 10.15
The WHO classification of tumors of the nervous system. J Neuropathol Exp Neurol (2002) 8.62
ERM proteins and merlin: integrators at the cell cortex. Nat Rev Mol Cell Biol (2002) 8.41
The diagnostic evaluation and multidisciplinary management of neurofibromatosis 1 and neurofibromatosis 2. JAMA (1997) 8.30
Directed actin polymerization is the driving force for epithelial cell-cell adhesion. Cell (2000) 8.13
Lentivirus-delivered stable gene silencing by RNAi in primary cells. RNA (2003) 8.07
A negative feedback signaling network underlies oncogene-induced senescence. Cancer Cell (2006) 6.96
Alteration in a new gene encoding a putative membrane-organizing protein causes neuro-fibromatosis type 2. Nature (1993) 6.67
A novel moesin-, ezrin-, radixin-like gene is a candidate for the neurofibromatosis 2 tumor suppressor. Cell (1993) 6.29
Molecular complexity and dynamics of cell-matrix adhesions. J Cell Sci (2001) 5.77
The signals and pathways activating cellular senescence. Int J Biochem Cell Biol (2004) 5.00
The NF2 tumor suppressor gene product, merlin, mediates contact inhibition of growth through interactions with CD44. Genes Dev (2001) 3.65
Membrane organization and tumorigenesis--the NF2 tumor suppressor, Merlin. Genes Dev (2005) 3.59
Role of actin polymerization and adhesion to extracellular matrix in Rac- and Rho-induced cytoskeletal reorganization. J Cell Biol (1997) 3.12
The Nf2 tumor suppressor, merlin, functions in Rac-dependent signaling. Dev Cell (2001) 2.89
Optimized large-scale production of high titer lentivirus vector pseudotypes. J Virol Methods (2004) 2.84
Mice heterozygous for a mutation at the Nf2 tumor suppressor locus develop a range of highly metastatic tumors. Genes Dev (1998) 2.66
Cortical actin organization: lessons from ERM (ezrin/radixin/moesin) proteins. J Biol Chem (1999) 2.60
NF2 deficiency promotes tumorigenesis and metastasis by destabilizing adherens junctions. Genes Dev (2003) 2.55
Merlin/NF-2 mediates contact inhibition of growth by suppressing recruitment of Rac to the plasma membrane. J Cell Biol (2005) 1.86
Ezrin regulates E-cadherin-dependent adherens junction assembly through Rac1 activation. Mol Biol Cell (2003) 1.67
Molecular genetic approach to human meningioma: loss of genes on chromosome 22. Proc Natl Acad Sci U S A (1987) 1.63
The NF2 tumor suppressor gene product, merlin, inhibits cell proliferation and cell cycle progression by repressing cyclin D1 expression. Mol Cell Biol (2005) 1.60
Protein 4.1 tumor suppressors: getting a FERM grip on growth regulation. J Cell Sci (2002) 1.59
Distinct cellular and subcellular patterns of expression imply distinct functions for the Drosophila homologues of moesin and the neurofibromatosis 2 tumor suppressor, merlin. J Cell Biol (1996) 1.56
Regulation of the neurofibromatosis type 2 tumor suppressor protein, merlin, by adhesion and growth arrest stimuli. J Biol Chem (1998) 1.54
Upregulation of the Rac1/JNK signaling pathway in primary human schwannoma cells. Hum Mol Genet (2003) 1.47
Nf2 gene inactivation in arachnoidal cells is rate-limiting for meningioma development in the mouse. Genes Dev (2002) 1.43
Molecular pathogenesis of meningiomas. J Neurooncol (2004) 1.42
Ruffling membrane, stress fiber, cell spreading and proliferation abnormalities in human Schwannoma cells. Oncogene (1998) 1.38
Frequency and distribution of NF2 mutations in schwannomas. Genes Chromosomes Cancer (1996) 1.34
A NHERF binding site links the betaPDGFR to the cytoskeleton and regulates cell spreading and migration. J Cell Sci (2004) 1.30
Increased expression of the NF2 tumor suppressor gene product, merlin, impairs cell motility, adhesionand spreading. Hum Mol Genet (1999) 1.27
Cellular transformation by a FERM domain mutant of the Nf2 tumor suppressor gene. Oncogene (2002) 1.23
The merlin tumor suppressor localizes preferentially in membrane ruffles. Oncogene (1996) 1.22
Expression level, subcellular distribution and rho-GDI binding affinity of merlin in comparison with Ezrin/Radixin/Moesin proteins. Oncogene (1999) 1.22
Management of the patient and family with neurofibromatosis 2: a consensus conference statement. Br J Neurosurg (2005) 1.21
Temporal control of Rac in Schwann cell-axon interaction is disrupted in NF2-mutant schwannoma cells. J Neurosci (2006) 1.17
Brain tumours: classification and genes. J Neurol Neurosurg Psychiatry (2004) 1.13
Isolation and characterization of Schwann cells from neurofibromatosis type 2 patients. Neurobiol Dis (1998) 1.12
Meningioma pathology, genetics, and biology. J Neuropathol Exp Neurol (2004) 1.06
The neurofibromatosis type 2 gene product, merlin, reverses the F-actin cytoskeletal defects in primary human Schwannoma cells. Mol Cell Biol (2002) 1.04
Multiple meningiomas: Investigating the molecular basis of sporadic and familial forms. Int J Cancer (2003) 1.02
Magicin, a novel cytoskeletal protein associates with the NF2 tumor suppressor merlin and Grb2. Oncogene (2004) 1.02
Mouse models of neurofibromatosis 1 and 2. Neoplasia (2002) 0.98
The neurofibromatosis type 2 gene product, schwannomin, suppresses growth of NIH 3T3 cells. Cancer Res (1995) 0.95
Mild familial neurofibromatosis 2 associates with expression of merlin with altered COOH-terminus. Neurology (2000) 0.90
Cytokeratin provides a specific marker for human arachnoid cells grown in vitro. Exp Cell Res (1983) 0.89
Characterization of normal human brain cultures. Evidence for the outgrowth of leptomeningeal cells. Lab Invest (1986) 0.86
Expression of epithelial and extracellular matrix protein markers in meningiomas. Histopathology (1993) 0.84
Expression of beta1 and beta4 integrins in normal arachnoid membrane and meningiomas. Cancer (1999) 0.82
Rearrangements of the intermediate filament GFAP in primary human schwannoma cells. Neurobiol Dis (2005) 0.80
Primary culture of human leptomeningeal cells in serum-free medium. Neurosci Lett (1994) 0.78
Telomerase in intracranial meningiomas. Int J Mol Med (2003) 0.78
On Arachnoid Villi and Meningiomas: Functional Implication of Ultrastructure, Cell Adhesion Mechanisms, and Extracellular Matrix Composition. Pathol Oncol Res (1996) 0.77
Elevated p53 expression in benign meningiomas protects against recurrence and may be indicative of senescence. Neuropathol Appl Neurobiol (2001) 0.76
Mouse models of neurofibromatosis. Biochim Biophys Acta (2001) 0.76
Association between microdeletion and microduplication at 16p11.2 and autism. N Engl J Med (2008) 19.71
The GPR54 gene as a regulator of puberty. N Engl J Med (2003) 9.54
Reversal of learning deficits in a Tsc2+/- mouse model of tuberous sclerosis. Nat Med (2008) 5.15
Genomewide association study for susceptibility genes contributing to familial Parkinson disease. Hum Genet (2008) 5.04
Disruption of neurexin 1 associated with autism spectrum disorder. Am J Hum Genet (2008) 5.04
Hearing improvement after bevacizumab in patients with neurofibromatosis type 2. N Engl J Med (2009) 3.85
Phase II study of cediranib, an oral pan-vascular endothelial growth factor receptor tyrosine kinase inhibitor, in patients with recurrent glioblastoma. J Clin Oncol (2010) 3.43
Sequencing chromosomal abnormalities reveals neurodevelopmental loci that confer risk across diagnostic boundaries. Cell (2012) 3.21
Genomic sequencing of meningiomas identifies oncogenic SMO and AKT1 mutations. Nat Genet (2013) 3.19
Human chromosome 7: DNA sequence and biology. Science (2003) 3.02
Admixture mapping of an allele affecting interleukin 6 soluble receptor and interleukin 6 levels. Am J Hum Genet (2007) 2.91
Clinical genetic testing for patients with autism spectrum disorders. Pediatrics (2010) 2.87
Assessment of 2q23.1 microdeletion syndrome implicates MBD5 as a single causal locus of intellectual disability, epilepsy, and autism spectrum disorder. Am J Hum Genet (2011) 2.85
Proteasomal and genetic inactivation of the NF1 tumor suppressor in gliomagenesis. Cancer Cell (2009) 2.73
Mirror extreme BMI phenotypes associated with gene dosage at the chromosome 16p11.2 locus. Nature (2011) 2.59
KCTD13 is a major driver of mirrored neuroanatomical phenotypes of the 16p11.2 copy number variant. Nature (2012) 2.47
Complex reorganization and predominant non-homologous repair following chromosomal breakage in karyotypically balanced germline rearrangements and transgenic integration. Nat Genet (2012) 2.45
Mutations in CHD7, encoding a chromatin-remodeling protein, cause idiopathic hypogonadotropic hypogonadism and Kallmann syndrome. Am J Hum Genet (2008) 2.33
Improved tumor oxygenation and survival in glioblastoma patients who show increased blood perfusion after cediranib and chemoradiation. Proc Natl Acad Sci U S A (2013) 2.14
Deletions of NRXN1 (neurexin-1) predispose to a wide spectrum of developmental disorders. Am J Med Genet B Neuropsychiatr Genet (2010) 2.12
Next-generation sequencing strategies enable routine detection of balanced chromosome rearrangements for clinical diagnostics and genetic research. Am J Hum Genet (2011) 2.12
Case records of the Massachusetts General Hospital. Case 11-2007. A 59-year-old man with neck pain, weakness in the arms, and cranial-nerve palsies. N Engl J Med (2007) 2.11
Oligogenic basis of isolated gonadotropin-releasing hormone deficiency. Proc Natl Acad Sci U S A (2010) 2.10
The Gly2019Ser mutation in LRRK2 is not fully penetrant in familial Parkinson's disease: the GenePD study. BMC Med (2008) 2.10
Downregulated microRNA-200a in meningiomas promotes tumor growth by reducing E-cadherin and activating the Wnt/beta-catenin signaling pathway. Mol Cell Biol (2009) 2.06
Characterization of apparently balanced chromosomal rearrangements from the developmental genome anatomy project. Am J Hum Genet (2008) 2.06
Functions of MUC16 in corneal epithelial cells. Invest Ophthalmol Vis Sci (2007) 2.01
Use of video to facilitate end-of-life discussions with patients with cancer: a randomized controlled trial. J Clin Oncol (2009) 2.00
HD CAG repeat implicates a dominant property of huntingtin in mitochondrial energy metabolism. Hum Mol Genet (2005) 1.96
X-linked protocadherin 19 mutations cause female-limited epilepsy and cognitive impairment. Nat Genet (2008) 1.95
Influence of heterozygosity for parkin mutation on onset age in familial Parkinson disease: the GenePD study. Arch Neurol (2006) 1.94
Biotin-responsive basal ganglia disease maps to 2q36.3 and is due to mutations in SLC19A3. Am J Hum Genet (2005) 1.94
Plexiform and dermal neurofibromas and pigmentation are caused by Nf1 loss in desert hedgehog-expressing cells. Cancer Cell (2008) 1.86
A genome scan for modifiers of age at onset in Huntington disease: The HD MAPS study. Am J Hum Genet (2003) 1.85
Disruption of ROBO2 is associated with urinary tract anomalies and confers risk of vesicoureteral reflux. Am J Hum Genet (2007) 1.85
Maintenance of primary tumor phenotype and genotype in glioblastoma stem cells. Neuro Oncol (2011) 1.80
NF2/merlin is a novel negative regulator of mTOR complex 1, and activation of mTORC1 is associated with meningioma and schwannoma growth. Mol Cell Biol (2009) 1.78
Update from the 2011 International Schwannomatosis Workshop: From genetics to diagnostic criteria. Am J Med Genet A (2013) 1.72
Specific progressive cAMP reduction implicates energy deficit in presymptomatic Huntington's disease knock-in mice. Hum Mol Genet (2003) 1.72
Glioblastoma recurrence after cediranib therapy in patients: lack of "rebound" revascularization as mode of escape. Cancer Res (2011) 1.69
Clinical diagnosis by whole-genome sequencing of a prenatal sample. N Engl J Med (2012) 1.67
Early phenotypes that presage late-onset neurodegenerative disease allow testing of modifiers in Hdh CAG knock-in mice. Hum Mol Genet (2002) 1.67
Translocations disrupting PHF21A in the Potocki-Shaffer-syndrome region are associated with intellectual disability and craniofacial anomalies. Am J Hum Genet (2012) 1.64
Depletion of peripheral macrophages and brain microglia increases brain tumor titers of oncolytic viruses. Cancer Res (2007) 1.60
Immunohistochemical analysis supports a role for INI1/SMARCB1 in hereditary forms of schwannomas, but not in solitary, sporadic schwannomas. Brain Pathol (2008) 1.59
Rescue of a human mRNA splicing defect by the plant cytokinin kinetin. Hum Mol Genet (2004) 1.58
miRNA-7 attenuation in Schwannoma tumors stimulates growth by upregulating three oncogenic signaling pathways. Cancer Res (2010) 1.54
WDR11, a WD protein that interacts with transcription factor EMX1, is mutated in idiopathic hypogonadotropic hypogonadism and Kallmann syndrome. Am J Hum Genet (2010) 1.49
Role of resection of malignant peripheral nerve sheath tumors in patients with neurofibromatosis type 1. J Neurosurg (2012) 1.45
TorsinA in PC12 cells: localization in the endoplasmic reticulum and response to stress. J Neurosci Res (2003) 1.42
Huntington CAG repeat size does not modify onset age in familial Parkinson's disease: the GenePD study. Mov Disord (2008) 1.41
TorsinA protein and neuropathology in early onset generalized dystonia with GAG deletion. Neurobiol Dis (2003) 1.39
Tumor burden in patients with neurofibromatosis types 1 and 2 and schwannomatosis: determination on whole-body MR images. Radiology (2009) 1.36
Reversal of a full-length mutant huntingtin neuronal cell phenotype by chemical inhibitors of polyglutamine-mediated aggregation. BMC Neurosci (2005) 1.35
Histology-based expression profiling yields novel prognostic markers in human glioblastoma. J Neuropathol Exp Neurol (2005) 1.34
Huntingtin facilitates polycomb repressive complex 2. Hum Mol Genet (2009) 1.32
Genome-wide significance for a modifier of age at neurological onset in Huntington's disease at 6q23-24: the HD MAPS study. BMC Med Genet (2006) 1.30
NFIA haploinsufficiency is associated with a CNS malformation syndrome and urinary tract defects. PLoS Genet (2007) 1.30
Three-dimensional volumetrics for tracking vestibular schwannoma growth in neurofibromatosis type II. Neurosurgery (2008) 1.30
A NHERF binding site links the betaPDGFR to the cytoskeleton and regulates cell spreading and migration. J Cell Sci (2004) 1.30
Value of PET in the assessment of patients with neurofibromatosis type 1. AJR Am J Roentgenol (2007) 1.28
Clinical features of schwannomatosis: a retrospective analysis of 87 patients. Oncologist (2012) 1.27
Pam and its ortholog highwire interact with and may negatively regulate the TSC1.TSC2 complex. J Biol Chem (2003) 1.26
Tissue-specific reduction in splicing efficiency of IKBKAP due to the major mutation associated with familial dysautonomia. Am J Hum Genet (2003) 1.26
The relationship between CAG repeat length and age of onset differs for Huntington's disease patients with juvenile onset or adult onset. Ann Hum Genet (2006) 1.26
An ovine transgenic Huntington's disease model. Hum Mol Genet (2010) 1.24
The predominantly HEAT-like motif structure of huntingtin and its association and coincident nuclear entry with dorsal, an NF-kB/Rel/dorsal family transcription factor. BMC Neurosci (2002) 1.24
Anti-vascular endothelial growth factor therapies as a novel therapeutic approach to treating neurofibromatosis-related tumors. Cancer Res (2010) 1.24
Genomewide association study for onset age in Parkinson disease. BMC Med Genet (2009) 1.24
Consensus recommendations for current treatments and accelerating clinical trials for patients with neurofibromatosis type 2. Am J Med Genet A (2011) 1.24
YKL-40 is a differential diagnostic marker for histologic subtypes of high-grade gliomas. Clin Cancer Res (2005) 1.23
Exonic deletions in AUTS2 cause a syndromic form of intellectual disability and suggest a critical role for the C terminus. Am J Hum Genet (2013) 1.22
Huntington's disease. Neuromolecular Med (2003) 1.22
A novel approach to investigate tissue-specific trinucleotide repeat instability. BMC Syst Biol (2010) 1.20
Genetic causes of brain tumors: neurofibromatosis, tuberous sclerosis, von Hippel-Lindau, and other syndromes. Neurol Clin (2007) 1.19
PARK3 influences age at onset in Parkinson disease: a genome scan in the GenePD study. Am J Hum Genet (2002) 1.19
Evidence for a modifier of onset age in Huntington disease linked to the HD gene in 4p16. Neurogenetics (2004) 1.19
Exacerbation of cerebral radiation necrosis by bevacizumab. J Clin Oncol (2010) 1.18
Development of a focused oligonucleotide-array comparative genomic hybridization chip for clinical diagnosis of genomic imbalance. Clin Chem (2007) 1.18
Phosphorylation of tuberin as a novel mechanism for somatic inactivation of the tuberous sclerosis complex proteins in brain lesions. Cancer Res (2004) 1.18
Candidate DNA replication initiation regions at human trinucleotide repeat disease loci. Hum Mol Genet (2003) 1.17
Therapeutic potential and mechanism of kinetin as a treatment for the human splicing disease familial dysautonomia. J Mol Med (Berl) (2007) 1.17
Unbiased gene expression analysis implicates the huntingtin polyglutamine tract in extra-mitochondrial energy metabolism. PLoS Genet (2007) 1.17
Increase in tumor-associated macrophages after antiangiogenic therapy is associated with poor survival among patients with recurrent glioblastoma. Neuro Oncol (2013) 1.16
Expanding the phenotype and genotype of female GnRH deficiency. J Clin Endocrinol Metab (2011) 1.16
The early onset dystonia protein torsinA interacts with kinesin light chain 1. J Biol Chem (2004) 1.16
Peripheral and cranial nerve sheath tumors. Curr Opin Neurol (2005) 1.16
Linkage of eye movement dysfunction to chromosome 6p in schizophrenia: additional evidence. Am J Med Genet B Neuropsychiatr Genet (2004) 1.11
Understanding relationships between autism, intelligence, and epilepsy: a cross-disorder approach. Dev Med Child Neurol (2012) 1.11
Sacrococcygeal chordomas in patients with tuberous sclerosis complex show somatic loss of TSC1 or TSC2. Genes Chromosomes Cancer (2004) 1.10
A humanized IKBKAP transgenic mouse models a tissue-specific human splicing defect. Genomics (2007) 1.08
Pam (Protein associated with Myc) functions as an E3 ubiquitin ligase and regulates TSC/mTOR signaling. Cell Signal (2008) 1.07