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Neurofibromin 2 (0):
Recent article
Neurofibromin 2
Genetika (Moskva) (46)10 2010
Abstract
The Merlin gene of Drosophila is homologous to the human Neurofibromatosis 2 (NF2) gene an important regulator of proliferation and endocytosis of cell receptors. It was earlier shown that the Thr5 residue of the Drosophila Merlin protein was homologous to Ser518 of the human protein (which was alre...
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PMID: 21254560
PDF is available here.
PDF is available here.
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Genes & Development (24)16 2010
Abstract
We discuss these findings and the proposed molecular mechanisms involved within the context of our current understanding of the pathways regulated by NF2....
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PMID: 20713513
PDF is available here.
PDF is available here.
Abstract
PURPOSE. Neurofibromatosis type 2 (NF2) is an autosomal-dominant CNS tumor syndrome that affects 1:25,000 children and young adults. More than 50% of NF2 patients also develop posterior subcapsular cataracts (PSCs). The authors deleted Nf2 from the lens to determine its role in fiber cell differenti...
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PMID: 20181838
PDF is available here.
PDF is available here.
Abstract
We show that NF2 knockout mouse embryonic fibroblasts lost contact inhibition of cell proliferation and contained significantly increased canonical Wnt signaling. Inhibition of Rac1, the activity of which is inversely regulated by NF2, through the use of a dominant-negative mutant, small hairpin RNA...
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PMID: 20154721
PDF is available here.
PDF is available here.
Otology & Neurotology (31)3 2010
Abstract
We developed an in vitro model of human Schwann cell tumorigenesis by merlin knockdown.
Neurofibromatosis 2 (NF2)-related and sporadic vestibular schwannoma (VS) exhibit loss of functional merlin (schwannomin). After loss of merlin expression in the Schwann cell, the initial steps to...
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PMID: 20195187
PDF is available here.
PDF is available here.
Genetika (Moskva) (46)3 2010
Abstract
We found that ectopic expression in the wing pouch of the clathrin adapter protein Lap involved in clathrin-mediated receptor endocytosis resulted in the formation of extra vein materials. On the one hand, coexpression of wild-type Merlin and lap in the wing pouch restored normal venation, while ove...
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PMID: 20391775
PDF is available here.
PDF is available here.
Abstract
We show that the closed, growth-inhibitory form of Merlin accumulates in the nucleus, binds to the E3 ubiquitin ligase CRL4(DCAF1), and suppresses its activity. Depletion of DCAF1 blocks the promitogenic effect of inactivation of Merlin. Conversely, enforced expression of a Merlin-insensitive mutant...
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PMID: 20178741
PDF is available here.
PDF is available here.
Genetika (Moskva) (46)2 2010
Abstract
We showed that the Merlin mutation leads to depression of vein clonal restriction property. This means that this gene is involved not only in the control of cell proliferation, but also in the control of cell mobility and adhesion....
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PMID: 20297652
PDF is available here.
PDF is available here.
Genetika (Moskva) (46)2 2010
Abstract
Fluorescence of H3-p histone and DAPI was studied at different stages of interphase and mitosis in cells of imaginal disks of third-instar Drosophila melanogaster larvae. Three stages differing in the spatial organization of the chromosome set in mitosis were revealed. At the first stage (prophase,...
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PMID: 20297651
PDF is available here.
PDF is available here.
Abstract
We have developed a series of probes that measures merlin conformation by fluorescence resonance energy transfer, both as purified protein and in live cells. Using these tools, we find that merlin exists predominately as a monomer in a stable, closed conformation that is mediated by the central alph...
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PMID: 19884346
PDF is available here.
PDF is available here.
Abstract
Overall, the VSs demonstrated significant average growth (p < 0.001), and hearing worsened significantly (p < 0.001) over 1 year. The amount of change in the bigger tumors was not associated with the amount of change in the smaller tumors within each patient. Vestibular schwannoma size changes were...
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PMID: 19704365
PDF is available here.
PDF is available here.
Journal of Cell Science (122)Pt 14 2009
Abstract
We examined the effects of Mer, Ex and the Hippo pathway on the size of the apical membrane and on apical-basal polarity complexes. We found that mer;ex double mutant imaginal disc cells have significantly increased levels of apical membrane determinants, such as Crb, aPKC and Patj. These phenotypes...
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PMID: 19531584
PDF is available here.
PDF is available here.
Molecular and Cellular Biochemistry (324)1-2 2009
Abstract
We analyze the clinical and biological behaviors of seven randomly selected sporadic vestibular Schwannomas removed from the patients. We find that merlin was commonly lost in these Schwannomas, due to loss of merlin expression or phosphorylation status of merlin expression. Heightened CDKs/cyclins...
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PMID: 19142715
PDF is available here.
PDF is available here.
Abstract
We describe a case of brainstem stroke in a child with NF2....
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PMID: 18406647
PDF is available here.
PDF is available here.
Brain Pathology (19)1 2009
Abstract
We here show that Schwannoma cells display an increased number of mature and stable focal contacts. In addition to an involvement of RhoA signaling via the Rho kinase ROCK, Rac1 plays a significant role in the pathological adhesion of Schwannoma cells. The Rac1 guanine exchange factor- beta-Pix, loc...
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PMID: 18445079
PDF is available here.
PDF is available here.
BioFactors (35)4 2009
Abstract
We summarize current knowledge of Merlin/MST/SAV/MOB/LATS/NDR/YAP/TAZ networks (also termed mammalian Hippo signaling) and their roles in mammalian cellular transformation....
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PMID: 19484742
PDF is available here.
PDF is available here.
Genetika (Moskva) (44)11 2008
[Role of the porcupine gene in the development of the wing imaginal disk of Drosophila melanogaster]
Abstract
A search for the genes interacting with the Merlin tumor suppressor gene revealed a Merlin-porcupine interaction during wing morphogenesis. Ectopic expression of the porcupine gene in the wing imaginal disk reduced the adult wing, while addition of an UAS construct with a full-length or truncated co...
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PMID: 19137731
PDF is available here.
PDF is available here.
Methods in Molecular Biology (493)493 2008
Abstract
Recent advances in molecular biology have led to a better understanding of the etiology of vestibular schwannomas. The underlying purpose of vestibular schwannoma research is the development of new treatment options; however, such options have not yet been established. A fundamental understanding of...
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PMID: 18839347
PDF is available here.
PDF is available here.
Brain Pathology (18)4 2008
Abstract
We performed an immunohistochemistry analysis on 45 schwannomas from patients with multiple schwannoma syndromes and on 38 solitary, sporadic schwannomas from non-syndromic patients. A mosaic pattern of INI1 expression was seen in 93% of tumors from familial schwannomatosis patients, 55% of tumors f...
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PMID: 18422762
PDF is available here.
PDF is available here.
Cancer Research (68)14 2008
Abstract
We show that merlin expression is dramatically reduced in human malignant gliomas and that reexpression of functional merlin dramatically inhibits both subcutaneous and intracranial growth of human glioma cells in mice. We further show that merlin reexpression inhibits glioma cell proliferation and...
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PMID: 18632626
PDF is available here.
PDF is available here.
Experimental Cell Research (314)11-12 2008
Abstract
We previously demonstrated that c-Jun N-terminal kinase (JNK) phosphorylation of the focal adhesion protein paxillin mediates differentiation in N1E-115 neuroblastoma cells. Here, we show that VPA up-regulates the neurofibromatosis type 2 (NF2) tumor suppressor, merlin, to regulate neurite outgrowth...
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PMID: 18486129
PDF is available here.
PDF is available here.
Abstract
We provide evidence to show that Merlin is regulated in a Roc1-Cullin4A-DDB1-dependent manner. Following serum stimulation, Merlin is recruited to the E3 ligase complex through a direct interaction with the WD40-containing adaptor protein VprBP. Loading of Merlin to the E3 ubiquitin ligase complex r...
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PMID: 18332868
PDF is available here.
PDF is available here.
Abstract
Merlin is inactivated in DU145 prostate cancer cells by PAK-mediated constitutive phosphorylation, identifying a novel mechanism of merlin inactivation in neoplastic cells....
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PMID: 18361411
PDF is available here.
PDF is available here.
Abstract
We identified germline mutations in SMARCB1 in 5 of 15 (33.3%) families with schwannomatosis and 2 of 28 (7.1%) individuals with sporadic schwannomatosis. In all individuals with a germline mutation in SMARCB1 in whom tumour tissue was available, we detected a second hit with loss of SMARCB1. In add...
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PMID: 18285426
PDF is available here.
PDF is available here.
Abstract
Merlin, the protein product of the neurofibromatosis type 2 gene (NF2) acts as a tumor suppressor in mice and humans. In this study, melanoma B16F10 cells were engineered to overexpress the NF2 gene by establishing stable transductants. A cell line overexpressing Merlin (B16F10-M) was generated. Whe...
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PMID: 18497985
PDF is available here.
PDF is available here.
Abstract
We identify a novel PKA phosphorylation site, serine 10, in the N terminus of merlin. We show that a non-phosphorylatable form of serine 10 (S10A) affects cellular morphology. Regulation of this site also influences actin cytoskeleton organization and dynamics in vivo, as merlin S10A reduces the amo...
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PMID: 18071304
PDF is available here.
PDF is available here.
Abstract
We demonstrate in primary Schwann cells (SCs) that Schwannomin is rapidly phosphorylated on S518 by Pak following laminin-1 binding to beta1 integrin, and by protein kinase A following neuregulin-1beta (NRG1beta) binding to ErbB2/ErbB3 receptors. These receptors, together with phosphorylated Schwann...
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PMID: 17998937
PDF is available here.
PDF is available here.
Abstract
We have shown that human primary schwannoma cells display activation of the RhoGTPases Rac1 and Cdc42 which results in highly dynamic and ongoing protrusive activity like ruffling. Ruffling is an initial and temporally limited step in the formation of intercellular contacts like adherens junctions t...
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PMID: 18240308
PDF is available here.
PDF is available here.
Genetika (Moskva) (44)3 2008
Abstract
The effect of mutation for gene Merlin on chromosome disjunction in Drosophila during meiosis was genetically studied. Chromosome nondisjunction was not registered in females heterozygous for this mutation and containing structurally normal X chromosomes. In cases when these females additionally con...
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PMID: 18664148
PDF is available here.
PDF is available here.
Abstract
I will first describe the phenotypes associated with 'merlin' mutations and consider differential diagnosis, in particular Schwannomatosis, for which a gene defect has been described recently. Existing therapeutic options, surgery and radiosurgery, including new data on the latter will be reviewed....
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PMID: 17940085
PDF is available here.
PDF is available here.
Abstract
This review explores possible mechanisms by which the neurofibromatosis type-2 tumour suppressor Merlin regulates contact-dependent inhibition of proliferation. Starting from an evolutionary perspective, the concurrent emergence of intercellular contacts and proliferation control in multicellular or...
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PMID: 17971776
PDF is available here.
PDF is available here.
Abstract
We show that the extreme amino (N) terminus directs merlin biochemically to an insoluble membrane compartment and physically to the cortical actin network, with a marked concentration along cell-cell boundaries. This insoluble-membrane distribution is required for the growth-suppressing function of...
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PMID: 18086884
PDF is available here.
PDF is available here.
Human Mutation (29)2 2008
Abstract
We report on the molecular analysis of the SMARCB1 and NF2 genes in a series of 21 patients with schwannomatosis and in eight schwannomatosis-associated tumors from four different patients. A novel germline SMARCB1 mutation was found in one patient; inactivating somatic mutations of NF2, associated...
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PMID: 18072270
PDF is available here.
PDF is available here.
Neurobiology of Disease (29)2 2008
Abstract
We established primary cultures from genetically-matched meningioma and normal arachnoidal tissues. Our studies revealed novel and distinct cell biological and biochemical properties unique to merlin-deficient meningioma cells compared to merlin-expressing arachnoidal and meningioma cells, and other...
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PMID: 17962031
PDF is available here.
PDF is available here.
Abstract
This review chapter is a synthesis of the recent literature about pathogenesis of schwannomas with emphasis on vestibular schwannomas (VSs). The cornerstone of cellular transformation and proliferation of Schwann cells toward schwannomas has been attributed to the nonexpression of normal schwannomin...
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PMID: 18810196
PDF is available here.
PDF is available here.
Mutation Research (637)1-2 2008
Abstract
We have examined the NF2 mutation in HEI-193 cells, an immortalized cell line derived from the schwannoma of an NF2 patient. Previous work showed that the NF2 mutation in HEI-193 cells causes a splicing defect in the NF2 transcript. We have confirmed this result and further identified the resultant...
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PMID: 17868749
PDF is available here.
PDF is available here.
Abstract
We compared NF2 gene expression in 5 cases with gastrointestinal stromal tumors by quantitative real-time polymerase chain reaction analysis. NF2 gene mRNA expression was assessed in fresh tissue of stomach from 5 consecutive patients. We detected no alterations in NF2 gene expression in the quantit...
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PMID: 18822692
PDF is available here.
PDF is available here.
Arkhiv Patologii (70)4 2008
Abstract
The authors describe 4 cases of clinically, morphologically, and immunohistochemically typical intraneural perineuriomas that occurred in women aged 17 to 24 years. The tumors involved median, radial, ulnar, and palmar nerves and were 3, 2.5, 1.5, and 1.2 cm in the largest diameter. None patients ha...
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PMID: 18807521
PDF is available here.
PDF is available here.
Biochimica et Biophysica Acta (1785)1 2008
Abstract
The neurofibromatosis 2 (NF2) tumor suppressor protein merlin is commonly mutated in human benign brain tumors. The gene altered in NF2 was located on human chromosome 22q12 in 1993 and the encoded protein named merlin and schwannomin. Merlin has homology to ERM family proteins, ezrin, radixin, and...
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PMID: 17980164
PDF is available here.
PDF is available here.
Clinical Neuropathology (27)5 2008
Abstract
We measured levels of mRNAs coding for these genes by qRT-PCR in 51 cases and levels ofc-Myc protooncogene and sst2 protein by immunohistochemistry in 26 cases of meningiomas of various grades and histotypes. C-Myc mRNA and protein levels were not grade-related, but validated subdivision of the 36 b...
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PMID: 18808065
PDF is available here.
PDF is available here.
BMC Cell Biology (9)1 2008
Abstract
Testis examination reveals that hemizygous Mer3 mutant males have small seminal vesicles that contain only a few immotile sperm. By cytological and electron microscopy analyses of the Mer3, Mer4 (Gln170-->stop), and control testes at various stages of spermatogenesis, we show that Merlin mutations a...
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PMID: 18186933
PDF is available here.
PDF is available here.
Abstract
We analyzed 179 co-isogenic single P[GT1]-element insertion lines of Drosophila melanogaster to identify novel genes affecting developmental time in flies reared at 25 degrees C. Sixty percent of the lines showed a heterochronic phenotype, suggesting that a large number of genes affect this trait. M...
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PMID: 18687152
PDF is available here.
PDF is available here.
Laryngoscope (118)1 2008
Abstract
We found that merlin appeared in 98% of the VS tissue samples, with a mean cellular positivity of 46.66 +/- 5.75%. Merlin is inversely correlated with cyclin D1 in regard to subcellular localization. Merlin locates in the cytoplasm during G0/G1 phase, moves to the nucleus at S phase, and accumulates...
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PMID: 17989580
PDF is available here.
PDF is available here.
Brain Pathology (18)1 2008
Abstract
We inactivated Nf2 in homozygous conditional knockout mice by adenoviral Cre delivery and showed that Nf2 loss in arachnoid cells is rate-limiting for meningioma formation. Here, we report that additional nullizygosity for p16(Ink4a) increases the frequency of meningioma and meningothelial prolifera...
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PMID: 17924978
PDF is available here.
PDF is available here.
Genes to Cells (12)12 2007
Abstract
P-selectin glycoprotein ligand-1 (PSGL-1), an adhesion molecule with O-glycosylated extracellular sialomucins, is involved in leukocyte inflammatory responses. On activation, ezrin-radixin-moesin (ERM) proteins mediate the redistribution of PSGL-1 on polarized cell surfaces to facilitate binding to...
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PMID: 18076570
PDF is available here.
PDF is available here.
European Spine Journal (16 Suppl 3)s3 2007
Abstract
In this paper authors present two cases of multiple schwannomas without the features of neurofibromatosis (NF). The authors retrospectively reviewed the hospital charts, radiology films, operative notes and pathology slides of these two patients. There was no family history of neurofibromatosis. The...
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PMID: 17216226
PDF is available here.
PDF is available here.
Nature Cell Biology (9)10 2007
Abstract
We show that the protein kinase Akt directly binds to and phosphorylates merlin on residues Thr 230 and Ser 315, which abolishes merlin NTD/CTD interactions and binding to merlin's effector protein PIKE-L and other binding partners. Furthermore, Akt-mediated phosphorylation leads to merlin degradati...
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PMID: 17891137
PDF is available here.
PDF is available here.
Abstract
The role of the neurofibromatosis type 2 protein, denoted as merlin or schwannomin, in embryonic development, cellular adherence, and in cell proliferation has become better elucidated in the past year. Likewise, the role of merlin in Schwann cell-axon interaction has been studied. Additionally, two...
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PMID: 17823551
PDF is available here.
PDF is available here.
Brain Pathology (17)4 2007
Abstract
We set out to investigate the underlying genetics in peripheral nerve of NF2 patients with polyneuropathy. We identified NF2 patients with polyneuropathy in which we could detect the germline mutation and analyzed NF2 gene dosage in archived nerve biopsies from these patients using a newly developed...
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PMID: 17655741
PDF is available here.
PDF is available here.
Abstract
Our purpose was to investigate if secretory meningiomas, analogous to meningothelial meningiomas, follow a molecular route of pathogenesis independent of the neurorofibromatosis 2 gene-associated pathway. All meningiomas showed positive immunocoloration involving the majority of the hyaline inclusio...
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PMID: 17721284
PDF is available here.
PDF is available here.
Human Molecular Genetics (16)14 2007
Abstract
We identify merlin as a novel microtubule-organizing protein. We identify two tubulin-binding sites in merlin, one residing at the N-terminal FERM-domain and another at the C-terminal domain. Merlin's intramolecular association and phosphorylation of serine 518 regulate the interaction between merli...
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PMID: 17566081
PDF is available here.
PDF is available here.
Carcinogenesis (28)7 2007
Abstract
We found that Nf2(+/-) mice developed mesotheliomas after intra-peritoneal inoculation of a RCF sample (RCF1). Clinical features in exposed mice were similar to those observed in HMM, showing association between ascite and mesothelioma. Early passages of 12 mesothelioma cell cultures from ascites de...
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PMID: 17272307
PDF is available here.
PDF is available here.
Abstract
We determined the expression levels of VEGF, and vascular endothelial growth factor receptor (VEGFR)-1 and -2 mRNA in 46 intracranial schwannomas by quantitative real-time PCR, and correlated these with various clinical factors or other molecular markers. We found that these tumors expressed signifi...
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PMID: 17570036
PDF is available here.
PDF is available here.
Abstract
We show that Cdc42, important in filopodia formation, is activated. Both Rac1 and Cdc42 are found all around the cell periphery and in colocalization with their effector phospho-p21 activated kinase in human schwannoma cells. We therefore claim that Rac1 and Cdc42 are activated in a nonlocalized man...
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PMID: 17620986
PDF is available here.
PDF is available here.
Journal of Cell Biology (177)5 2007
Abstract
We show that upon cell-cell contact Merlin coordinates the processes of adherens junction stabilization and negative regulation of epidermal growth factor receptor (EGFR) signaling by restraining the EGFR into a membrane compartment from which it can neither signal nor be internalized. In confluent...
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PMID: 17548515
PDF is available here.
PDF is available here.
Trends in Cell Biology (17)5 2007
Abstract
We review these recent findings and their relevance to the tumor suppressor function of Merlin....
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PMID: 17442573
PDF is available here.
PDF is available here.
Developmental Biology (304)1 2007
Abstract
We have found important qualitative and quantitative differences in the ways Mer and Ex function to regulate cell proliferation and cell survival. Though both mer and ex restrict cell and tissue growth, ex clones exhibit delayed cell cycle exit in the developing eye, while mer clones do not. Convers...
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PMID: 17258190
PDF is available here.
PDF is available here.
Abstract
We studied a group of pediatric meningiomas, including neurofibromatosis type II-associated, sporadic, and radiation-induced cases. We found NF2 gene deletion in about 72% of the cases, with corresponding absent or minimal merlin protein expression by immunohistochemistry. Our findings confirm that...
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PMID: 17478763
PDF is available here.
PDF is available here.
Journal of Biological Chemistry (282)11 2007
Abstract
We investigated the involvement of miRNA in malignant cholangiocytes stably transfected to overexpress IL-6, which enhances tumor growth in vivo by inhibition of apoptosis. We provide evidence that (i) miRNA expression both in vitro and in vivo is altered by overexpression of IL-6; (ii) selective mi...
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PMID: 17220301
PDF is available here.
PDF is available here.
Abstract
We found that in the mouse embryo, expression of the Nf2 tumor suppressor, merlin, is dynamically regulated during tissue fusion: Nf2 expression is low at the leading front before fusion and high across the fused tissue bridge. Mosaic Nf2 mutants exhibit a global defect in tissue fusion characterize...
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PMID: 17360635
PDF is available here.
PDF is available here.