Published in Acta Neuropathol on December 11, 2011
Pathology of peripheral nerve sheath tumors: diagnostic overview and update on selected diagnostic problems. Acta Neuropathol (2012) 2.35
Canonical Wnt/β-catenin signaling drives human schwann cell transformation, progression, and tumor maintenance. Cancer Discov (2013) 1.10
Co-targeting the MAPK and PI3K/AKT/mTOR pathways in two genetically engineered mouse models of schwann cell tumors reduces tumor grade and multiplicity. Oncotarget (2014) 1.04
The Challenge of Cancer Genomics in Rare Nervous System Neoplasms: Malignant Peripheral Nerve Sheath Tumors as a Paradigm for Cross-Species Comparative Oncogenomics. Am J Pathol (2015) 0.99
Genetic predisposition to peripheral nerve neoplasia: diagnostic criteria and pathogenesis of neurofibromatoses, Carney complex, and related syndromes. Acta Neuropathol (2011) 0.95
Transgenic mice overexpressing neuregulin-1 model neurofibroma-malignant peripheral nerve sheath tumor progression and implicate specific chromosomal copy number variations in tumorigenesis. Am J Pathol (2013) 0.89
Survivin expression and prognostic significance in pediatric malignant peripheral nerve sheath tumors (MPNST). PLoS One (2013) 0.82
PleiotRHOpic: Rho pathways are essential for all stages of Neural Crest development. Small GTPases (2014) 0.81
Wilms' tumor gene 1 (WT1) silencing inhibits proliferation of malignant peripheral nerve sheath tumor sNF96.2 cell line. PLoS One (2014) 0.79
Methylated RASSF1A in malignant peripheral nerve sheath tumors identifies neurofibromatosis type 1 patients with inferior prognosis. Neuro Oncol (2014) 0.79
Classic Ras Proteins Promote Proliferation and Survival via Distinct Phosphoproteome Alterations in Neurofibromin-Null Malignant Peripheral Nerve Sheath Tumor Cells. J Neuropathol Exp Neurol (2015) 0.78
TAGLN expression is upregulated in NF1-associated malignant peripheral nerve sheath tumors by hypomethylation in its promoter and subpromoter regions. Oncol Rep (2014) 0.77
Stressing out the Hippo/YAP signaling pathway: toward a new role in Schwann cells. Cell Death Dis (2015) 0.77
Neuregulin-1 overexpression and Trp53 haploinsufficiency cooperatively promote de novo malignant peripheral nerve sheath tumor pathogenesis. Acta Neuropathol (2014) 0.77
Tumor suppressor Nf2/merlin drives Schwann cell changes following electromagnetic field exposure through Hippo-dependent mechanisms. Cell Death Discov (2015) 0.76
Clinicopathologic implications of NF1 gene alterations in diffuse gliomas. Hum Pathol (2015) 0.76
ERBB3: A potential serum biomarker for early detection and therapeutic target for devil facial tumour 1 (DFT1). PLoS One (2017) 0.75
Combinatorial therapy with tamoxifen and trifluoperazine effectively inhibits malignant peripheral nerve sheath tumor growth by targeting complementary signaling cascades. J Neuropathol Exp Neurol (2014) 0.75
Fatal acute retropharyngeal hemorrhage in neurofibromatosis type 1. Forensic Sci Med Pathol (2017) 0.75
Targeting group I p21-activated kinases to control malignant peripheral nerve sheath tumor growth and metastasis. Oncogene (2017) 0.75
CNS Tumors in Neurofibromatosis. J Clin Oncol (2017) 0.75
The Ras-RasGAP complex: structural basis for GTPase activation and its loss in oncogenic Ras mutants. Science (1997) 9.21
Type 1 neurofibromatosis gene: identification of a large transcript disrupted in three NF1 patients. Science (1990) 8.91
Truncating mutations of hSNF5/INI1 in aggressive paediatric cancer. Nature (1998) 7.55
A negative feedback signaling network underlies oncogene-induced senescence. Cancer Cell (2006) 6.96
Deletions and a translocation interrupt a cloned gene at the neurofibromatosis type 1 locus. Cell (1990) 6.94
The tumour-suppressor genes NF2/Merlin and Expanded act through Hippo signalling to regulate cell proliferation and apoptosis. Nat Cell Biol (2005) 6.74
Alteration in a new gene encoding a putative membrane-organizing protein causes neuro-fibromatosis type 2. Nature (1993) 6.67
Mutations of the gene encoding the protein kinase A type I-alpha regulatory subunit in patients with the Carney complex. Nat Genet (2000) 6.26
The neurofibromatosis type 1 gene encodes a protein related to GAP. Cell (1990) 5.24
Neurofibromas in NF1: Schwann cell origin and role of tumor environment. Science (2002) 5.08
Malignant peripheral nerve sheath tumours in neurofibromatosis 1. J Med Genet (2002) 5.06
Aberrant regulation of ras proteins in malignant tumour cells from type 1 neurofibromatosis patients. Nature (1992) 4.42
GDIs: central regulatory molecules in Rho GTPase activation. Trends Cell Biol (2005) 4.13
A dermal niche for multipotent adult skin-derived precursor cells. Nat Cell Biol (2004) 4.06
Tumour predisposition in mice heterozygous for a targeted mutation in Nf1. Nat Genet (1994) 3.97
Exhaustive mutation analysis of the NF1 gene allows identification of 95% of mutations and reveals a high frequency of unusual splicing defects. Hum Mutat (2000) 3.87
The NF2 tumor suppressor gene product, merlin, mediates contact inhibition of growth through interactions with CD44. Genes Dev (2001) 3.65
The catalytic domain of the neurofibromatosis type 1 gene product stimulates ras GTPase and complements ira mutants of S. cerevisiae. Cell (1990) 3.56
The NF1 tumor suppressor critically regulates TSC2 and mTOR. Proc Natl Acad Sci U S A (2005) 3.49
Neurofibromatosis 1 and neurofibromatosis 2: a twenty first century perspective. Lancet Neurol (2007) 3.44
Diagnostic criteria for schwannomatosis. Neurology (2005) 3.41
Mouse models of tumor development in neurofibromatosis type 1. Science (1999) 3.40
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
Loss of the tumor suppressor gene NF2, encoding merlin, constitutively activates integrin-dependent mTORC1 signaling. Mol Cell Biol (2009) 3.25
A clinical study of type 2 neurofibromatosis. Q J Med (1992) 3.23
Direct interaction of the Rho GDP dissociation inhibitor with ezrin/radixin/moesin initiates the activation of the Rho small G protein. J Biol Chem (1997) 3.08
Loss of Apc+ in intestinal adenomas from Min mice. Cancer Res (1994) 3.00
Haploinsufficiency of Snf5 (integrase interactor 1) predisposes to malignant rhabdoid tumors in mice. Proc Natl Acad Sci U S A (2000) 2.91
Mouse tumor model for neurofibromatosis type 1. Science (1999) 2.89
The Nf2 tumor suppressor, merlin, functions in Rac-dependent signaling. Dev Cell (2001) 2.89
Nf1-dependent tumors require a microenvironment containing Nf1+/-- and c-kit-dependent bone marrow. Cell (2008) 2.84
Schwannomatosis: a clinical and pathologic study. Neurology (1996) 2.82
An analysis of variation in expression of neurofibromatosis (NF) type 1 (NF1): evidence for modifying genes. Am J Hum Genet (1993) 2.79
Genetic heterogeneity and spectrum of mutations of the PRKAR1A gene in patients with the carney complex. Hum Mol Genet (2000) 2.73
Conditional biallelic Nf2 mutation in the mouse promotes manifestations of human neurofibromatosis type 2. Genes Dev (2000) 2.72
Clinical and mutational spectrum of neurofibromatosis type 1-like syndrome. JAMA (2009) 2.72
Mice heterozygous for a mutation at the Nf2 tumor suppressor locus develop a range of highly metastatic tumors. Genes Dev (1998) 2.66
NF2 deficiency promotes tumorigenesis and metastasis by destabilizing adherens junctions. Genes Dev (2003) 2.55
CD-34 is expressed by a distinctive cell population in peripheral nerve, nerve sheath tumors, and related lesions. Am J Surg Pathol (1993) 2.54
Germline mutation of INI1/SMARCB1 in familial schwannomatosis. Am J Hum Genet (2007) 2.53
Chromosome 17p deletions and p53 gene mutations associated with the formation of malignant neurofibrosarcomas in von Recklinghausen neurofibromatosis. Proc Natl Acad Sci U S A (1990) 2.52
RhoGDI: multiple functions in the regulation of Rho family GTPase activities. Biochem J (2005) 2.50
Differential regulation of Raf-1, A-Raf, and B-Raf by oncogenic ras and tyrosine kinases. J Biol Chem (1997) 2.47
Merlin/NF2 suppresses tumorigenesis by inhibiting the E3 ubiquitin ligase CRL4(DCAF1) in the nucleus. Cell (2010) 2.38
Constitutional mutations of the hSNF5/INI1 gene predispose to a variety of cancers. Am J Hum Genet (1999) 2.35
An absence of cutaneous neurofibromas associated with a 3-bp inframe deletion in exon 17 of the NF1 gene (c.2970-2972 delAAT): evidence of a clinically significant NF1 genotype-phenotype correlation. Am J Hum Genet (2006) 2.23
Merlin, the product of the Nf2 tumor suppressor gene, is an inhibitor of the p21-activated kinase, Pak1. Mol Cell (2003) 2.23
Cell of origin and microenvironment contribution for NF1-associated dermal neurofibromas. Cell Stem Cell (2009) 2.12
Somatic deletion of the neurofibromatosis type 1 gene in a neurofibrosarcoma supports a tumour suppressor gene hypothesis. Nat Genet (1993) 2.10
TORC1 is essential for NF1-associated malignancies. Curr Biol (2007) 2.08
Common pathogenetic mechanism for three tumor types in bilateral acoustic neurofibromatosis. Science (1987) 2.06
p21-activated kinase links Rac/Cdc42 signaling to merlin. J Biol Chem (2001) 2.05
Mutagenesis of the phosphatidylinositol 4,5-bisphosphate (PIP(2)) binding site in the NH(2)-terminal domain of ezrin correlates with its altered cellular distribution. J Cell Biol (2000) 1.97
A mouse model for the Carney complex tumor syndrome develops neoplasia in cyclic AMP-responsive tissues. Cancer Res (2005) 1.94
Molecular characterisation of SMARCB1 and NF2 in familial and sporadic schwannomatosis. J Med Genet (2008) 1.87
Merlin/NF-2 mediates contact inhibition of growth by suppressing recruitment of Rac to the plasma membrane. J Cell Biol (2005) 1.86
Plexiform and dermal neurofibromas and pigmentation are caused by Nf1 loss in desert hedgehog-expressing cells. Cancer Cell (2008) 1.86
Neurofibromin-deficient Schwann cells secrete a potent migratory stimulus for Nf1+/- mast cells. J Clin Invest (2003) 1.85
Merlin phosphorylation by p21-activated kinase 2 and effects of phosphorylation on merlin localization. J Biol Chem (2002) 1.82
Large-scale molecular comparison of human schwann cells to malignant peripheral nerve sheath tumor cell lines and tissues. Cancer Res (2006) 1.80
The neurofibromatosis-2 homologue, Merlin, and the tumor suppressor expanded function together in Drosophila to regulate cell proliferation and differentiation. Development (2000) 1.78
NHE-RF, a regulatory cofactor for Na(+)-H+ exchange, is a common interactor for merlin and ERM (MERM) proteins. J Biol Chem (1998) 1.75
Genetic and biochemical evidence that haploinsufficiency of the Nf1 tumor suppressor gene modulates melanocyte and mast cell fates in vivo. J Exp Med (2000) 1.71
Type of mutation in the neurofibromatosis type 2 gene (NF2) frequently determines severity of disease. Am J Hum Genet (1996) 1.70
The Nf2 tumor suppressor gene product is essential for extraembryonic development immediately prior to gastrulation. Genes Dev (1997) 1.67
Minireview: PRKAR1A: normal and abnormal functions. Endocrinology (2004) 1.64
Paternal origin of new mutations in von Recklinghausen neurofibromatosis. Nature (1990) 1.64
How does the Schwann cell lineage form tumors in NF1? Glia (2008) 1.63
Cancer-associated mutations in chromatin remodeler hSNF5 promote chromosomal instability by compromising the mitotic checkpoint. Genes Dev (2005) 1.61
Serine 518 phosphorylation modulates merlin intramolecular association and binding to critical effectors important for NF2 growth suppression. Oncogene (2004) 1.61
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
Familial schwannomatosis: exclusion of the NF2 locus as the germline event. Neurology (2003) 1.57
Merlin regulates transmembrane receptor accumulation and signaling at the plasma membrane in primary mouse Schwann cells and in human schwannomas. Oncogene (2008) 1.57
Expression of neuregulins and their putative receptors, ErbB2 and ErbB3, is induced during Wallerian degeneration. J Neurosci (1997) 1.57
Epidermal growth factor receptor expression in neurofibromatosis type 1-related tumors and NF1 animal models. J Clin Invest (2000) 1.56
Dissecting and targeting the growth factor-dependent and growth factor-independent extracellular signal-regulated kinase pathway in human schwannoma. Cancer Res (2008) 1.55
Interdomain binding mediates tumor growth suppression by the NF2 gene product. Oncogene (1997) 1.55
Regulation of the neurofibromatosis type 2 tumor suppressor protein, merlin, by adhesion and growth arrest stimuli. J Biol Chem (1998) 1.54
The loss of Nf1 transiently promotes self-renewal but not tumorigenesis by neural crest stem cells. Cancer Cell (2008) 1.54
Role for the epidermal growth factor receptor in neurofibromatosis-related peripheral nerve tumorigenesis. Cancer Cell (2005) 1.53
Malignant transformation of neurofibromas in neurofibromatosis 1 is associated with CDKN2A/p16 inactivation. Am J Pathol (1999) 1.52
Hyperactivation of p21(ras) and the hematopoietic-specific Rho GTPase, Rac2, cooperate to alter the proliferation of neurofibromin-deficient mast cells in vivo and in vitro. J Exp Med (2001) 1.50
Individual rate constants for the interaction of Ras proteins with GTPase-activating proteins determined by fluorescence spectroscopy. Biochemistry (1997) 1.49
Nf1+/- mast cells induce neurofibroma like phenotypes through secreted TGF-beta signaling. Hum Mol Genet (2006) 1.48
Expression of the human hematopoietic progenitor cell antigen CD34 in vascular and spindle cell tumors. J Cutan Pathol (1993) 1.47
NF1 microdeletions in neurofibromatosis type 1: from genotype to phenotype. Hum Mutat (2010) 1.47
Genotype/phenotype correlations in type 2 neurofibromatosis (NF2): evidence for more severe disease associated with truncating mutations. J Med Genet (1998) 1.47
Induction of abnormal proliferation by nonmyelinating schwann cells triggers neurofibroma formation. Cancer Cell (2008) 1.46
New mechanisms and functions of actin nucleation. Curr Opin Cell Biol (2010) 1.45
Effective in vivo targeting of the mammalian target of rapamycin pathway in malignant peripheral nerve sheath tumors. Mol Cancer Ther (2008) 1.44
Development of the Schwann cell lineage: from the neural crest to the myelinated nerve. Glia (2008) 1.42
Regulatory proteins of R-Ras, TC21/R-Ras2, and M-Ras/R-Ras3. J Biol Chem (2000) 1.42
Spectrum of single- and multiexon NF1 copy number changes in a cohort of 1,100 unselected NF1 patients. Genes Chromosomes Cancer (2006) 1.41
Integrative genomic analyses of neurofibromatosis tumours identify SOX9 as a biomarker and survival gene. EMBO Mol Med (2009) 1.41
Phase I trial and pharmacokinetic study of the farnesyltransferase inhibitor tipifarnib in children with refractory solid tumors or neurofibromatosis type I and plexiform neurofibromas. J Clin Oncol (2006) 1.40
Loss of NF1 allele in Schwann cells but not in fibroblasts derived from an NF1-associated neurofibroma. Genes Chromosomes Cancer (1999) 1.39
In vitro functional studies of naturally occurring pathogenic PRKAR1A mutations that are not subject to nonsense mRNA decay. Hum Mutat (2008) 1.39
Ruffling membrane, stress fiber, cell spreading and proliferation abnormalities in human Schwannoma cells. Oncogene (1998) 1.38
A novel moesin-, ezrin-, radixin-like gene is a candidate for the neurofibromatosis 2 tumor suppressor. Cell (1993) 1.38
TP53 mutations are frequent in malignant NF1 tumors. Genes Chromosomes Cancer (1994) 1.37