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303 records returned

Hypermethylation and Transcriptional Downregulation of the TIMP3 Gene Is Associated with Allelic Loss on 22q12.3 and Malignancy in MeningiomasEmail this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
The gene for the tissue inhibitor of metalloproteinase 3 (TIMP3) on 22q12.3 had been reported to be inactivated by promoter methylation in various types of cancers, with controversial findings in meningiomas. We performed direct sodium bisulfite sequencing in a series of 50 meningiomas, including 27 benign meningiomas [World Health Organization (WHO) grade I], 11 atypical meningiomas (WHO grade II) and 12 anaplastic meningiomas (WHO grade III), and found hypermethylation of TIMP3 in 67% of anaplastic meningiomas, but only 22% of atypical and 17% of benign meningiomas. Moreover, TIMP3 methylation scores were significantly i...
Source: Brain Pathology - November 18, 2009 Category: Neurology Authors: Dimitri Barski, Marietta Wolter, Guido Reifenberger, Markus J. Riemenschneider Source Type: journals

Neuropathology of Olfactory Ensheathing Cell Transplantation into the Brain of Two Amyotrophic Lateral Sclerosis (ALS) PatientsEmail this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
In conclusion, the present neuropathologic analysis does not support a beneficial effect of fetal OEC implantation into the frontal lobes of ALS patients. (Source: Brain Pathology)
Source: Brain Pathology - November 17, 2009 Category: Neurology Authors: Maria Teresa Giordana, Silvia Grifoni, Barbara Votta, Michela Magistrello, Marco Vercellino, Alessia Pellerino, Roberto Navone, Consuelo Valentini, Andrea Calvo, Adriano Chiò Source Type: journals

Gene Expression Analysis of Tuberous Sclerosis Complex Cortical Tubers Reveals Increased Expression of Adhesion and Inflammatory FactorsEmail this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
Cortical tubers in patients with tuberous sclerosis complex are associated with disabling neurological manifestations, including intractable epilepsy. While these malformations are believed to result from the effects of TSC1 or TSC2 gene mutations, the molecular mechanisms leading to tuber formation, as well as the onset of seizures, remain largely unknown. We used the Affymetrix Gene Chip platform to provide the first genome-wide investigation of gene expression in surgically resected tubers, compared with histological normal perituberal tissue from the same patients or autopsy control tissue. We identified 2501 different...
Source: Brain Pathology - November 12, 2009 Category: Neurology Authors: Karin Boer, Peter B. Crino, Jan A. Gorter, Mark Nellist, Floor E. Jansen, Wim G.M. Spliet, Peter C. van Rijen, Floyd R.A. Wittink, Timo M. Breit, Dirk Troost, Wytse J. Wadman, Eleonora Aronica Source Type: journals

Characterization of R132H Mutation-specific IDH1 Antibody Binding in Brain TumorsEmail this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
Heterozygous point mutations of isocitrate dehydrogenase (IDH)1 codon 132 are frequent in grade II and III gliomas. Recently, we reported an antibody specific for the IDH1R132H mutation. Here we investigate the capability of this antibody to differentiate wild type and mutated IDH1 protein in central nervous system (CNS) tumors by Western blot and immunohistochemistry. Results of protein analysis are correlated to sequencing data. In Western blot, anti-IDH1R132H mouse monoclonal antibody mIDH1R132H detected a specific band only in mutated tumors. Immunohistochemistry of 345 primary brain tumors demonstrated a strong cytopl...
Source: Brain Pathology - November 9, 2009 Category: Neurology Authors: David Capper, Susanne Weißert, Jörg Balss, Antje Habel, Jochen Meyer, Diana Jäger, Ulrike Ackermann, Claudia Tessmer, Andrey Korshunov, Hanswalter Zentgraf, Christian Hartmann, Andreas von Deimling Source Type: journals

Purple Sweet Potato Color Alleviates D-galactose-induced Brain Aging in Old Mice by Promoting Survival of Neurons via PI3K Pathway and Inhibiting Cytochrome C-mediated ApoptosisEmail this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
Purple sweet potato color (PSPC), a class of naturally occurring anthocyanins, protects brain function against oxidative stress induced by D-galactose (D-gal) (Sigma-Aldrich, St. Louis, MO, USA). Our data showed that PSPC enhanced open-field activity, decreased step-through latency, and improved spatial learning and memory ability in D-gal-treated old mice by decreasing advanced glycation end-products' (AGEs) formation and the AGE receptor (RAGE) expression, and by elevating Cu,Zn-superoxide dismutase (Cu,Zn-SOD) (Sigma-Aldrich) and catalase (CAT) expression and activity. Cleavage of caspase-3 and increased terminal deoxyn...
Source: Brain Pathology - October 28, 2009 Category: Neurology Authors: Jun Lu, Dong-mei Wu, Yuan-lin Zheng, Bin Hu, Zi-feng Zhang Source Type: journals

Upregulation of Immunoglobulin-related Genes in Cortical Sections from Multiple Sclerosis PatientsEmail this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
This study demonstrates that genes involved in the synthesis of Igs are upregulated in MS patients and that this activation is caused by a small number of meningeal plasma cells that are not infected by EBV. The findings indicate that the Ig-producing B-cells found in the cerebrospinal fluid (CSF) of MS patients could have meningeal origin. (Source: Brain Pathology)
Source: Brain Pathology - October 16, 2009 Category: Neurology Authors: Øivind Torkildsen, Christine Stansberg, Solveig M. Angelskår, Evert-Jan Kooi, Jeroen J.G. Geurts, Paul van der Valk, Kjell-Morten Myhr, Vidar M. Steen, Lars Bø Source Type: journals

FUS-Immunoreactive Intranuclear Inclusions in Neurodegenerative DiseaseEmail this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
The objective of the present study was to determine the range of neurodegenerative disorders characterized by FUS-positive NIIs. Immunostaining for FUS revealed intense reactivity of NIIs in FTLD-IF and FTLD-UPS as well as in Huntington's disease, spinocerebellar ataxias 1 and 3, and neuronal intranuclear inclusion body disease. In contrast, there was no FUS staining of NIIs in inherited forms of FTLD-TDP caused by GRN and VCP mutations, fragile-X-associated tremor ataxia syndrome, or oculopharyngeal muscular dystrophy. In a cell culture model of Huntington's disease, NIIs were intensely FUS-positive. NII-bearing cells dis...
Source: Brain Pathology - October 12, 2009 Category: Neurology Authors: John Woulfe, Douglas A. Gray, Ian R.A. Mackenzie Source Type: journals

Unique Molecular Characteristics of Pediatric Myxopapillary EpendymomaEmail this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
Myxopapillary ependymoma (MEPN) generally can be cured by gross total surgical resection and usually manifest a favorable prognosis. However, surgery is less curative in tumors that are large, multifocal or extend outside the thecal sac. Late recurrences may occur, particularly in pediatric patients. The role of adjuvant therapy is unclear in the clinical management of recurrent tumors. Clinical trial design requires a better understanding of tumor biology. Unique molecular features of MEPN were investigated by using microarray technology to compare the gene expression of five pediatric MEPN to 24 pediatric intracranial ep...
Source: Brain Pathology - September 29, 2009 Category: Neurology Authors: Valerie N. Barton, Andrew M. Donson, Bette K. Kleinschmidt-DeMasters, Diane K. Birks, Michael H. Handler, Nicholas K. Foreman Source Type: journals

Recent Insights into PDGF-Induced GliomagenesisEmail this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
Gliomas are aggressive and almost incurable glial brain tumors which frequently display abnormal platelet-derived growth factor (PDGF) signaling. Evidence gained from studies on several in vivo animal models has firmly established a causal connection between aberrant PDGF signaling and the formation of some gliomas. However, only recently has significant knowledge been gained regarding crucial issues such as the glioma cell of origin and the relationship between the transforming stimulus and the cellular characteristics of the resulting tumor. Based on recent evidence, we propose that PDGF can bias cell-fate decisions, dri...
Source: Brain Pathology - September 24, 2009 Category: Neurology Authors: Filippo Calzolari, Paolo Malatesta Source Type: journals

Identification and Functional Characterization of microRNAs Involved in the Malignant Progression of GliomasEmail this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
Diffuse astrocytoma of World Health Organization (WHO) grade II has an inherent tendency to spontaneously progress to anaplastic astrocytoma WHO grade III or secondary glioblastoma WHO grade IV. We explored the role of microRNAs (miRNAs) in glioma progression by investigating the expression profiles of 157 miRNAs in four patients with primary WHO grade II gliomas that spontaneously progressed to WHO grade IV secondary glioblastomas. Thereby, we identified 12 miRNAs (miR-9, miR-15a, miR-16, miR-17, miR-19a, miR-20a, miR-21, miR-25, miR-28, miR-130b, miR-140 and miR-210) showing increased expression, and two miRNAs (miR-184 ...
Source: Brain Pathology - September 23, 2009 Category: Neurology Authors: Bastian Malzkorn, Marietta Wolter, Franziska Liesenberg, Michael Grzendowski, Kai Stühler, Helmut E. Meyer, Guido Reifenberger Source Type: journals

Brain Neurons Express Ornithine Decarboxylase-Activating Antizyme Inhibitor 2 with Accumulation in Alzheimer's DiseaseEmail this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
Polyamines are small cationic molecules that in adult brain are connected to neuronal signaling by regulating inward-rectifier K+-channels and different glutamate receptors. Antizyme inhibitors (AZINs) regulate the cellular uptake of polyamines and activate ornithine decarboxylase (ODC), the rate-limiting enzyme of polyamine synthesis. Elevated levels of ODC activity and polyamines are detected in various brain disorders including stroke and Alzheimer's disease (AD). We originally reported a novel brain- and testis-specific AZIN, called AZIN2, the distribution of which we have now studied in normal and diseased human brain...
Source: Brain Pathology - September 21, 2009 Category: Neurology Authors: Laura T. Mäkitie, Kristiina Kanerva, Tuomo Polvikoski, Anders Paetau, Leif C. Andersson Source Type: journals

Impact of Morphology, MIB-1, p53 and MGMT on Outcome in Pilocytic AstrocytomasEmail this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
Pilocytic astrocytoma (PA) is the most common glioma in the pediatric population. PAs can exhibit variable behavior that does not always correlate with location, yet at present there is no way to predict which tumors will be more aggressive. To address this problem, an institutional cohort of 147 PAs (118 with outcome data) from both cerebellar and noncerebellar locations (spine, diencephalon, midbrain, brainstem and cortex) was utilized. Parameters included quantification of characteristic morphologic variables as well as genes previously shown to be of relevance in high-grade gliomas, including MIB-1, p53 and MGMT. In th...
Source: Brain Pathology - September 20, 2009 Category: Neurology Authors: Craig Horbinski, Ronald L. Hamilton, Colleen Lovell, Judith Burnham, Ian F. Pollack Source Type: journals

PCR- and Restriction Endonuclease-Based Detection of IDH1 MutationsEmail this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
Hotspot mutations in codon 132 of the gene encoding isocitrate dehydrogenase 1 (IDH1) have emerged as the most frequent DNA alteration in astrocytomas, oligodendrogliomas and oligoastrocytomas. These mutations have been shown to be of significant diagnostic and prognostic value. So far, assessment of IDH1 mutation relied on DNA sequencing techniques. We generated a set of primers suitable for endonuclease-based detection of hotspot mutations in codon 132 of IDH1. This primer set will allow determining these mutations without the need of DNA sequencing. One set of primer sets is designed to detect the presence or absence of...
Source: Brain Pathology - September 10, 2009 Category: Neurology Authors: Jochen Meyer, Stefan Pusch, Jörg Balss, David Capper, Wolf Mueller, Arne Christians, Christian Hartmann, Andreas von Deimling Source Type: journals

Protein Targets of Oxidative Damage in Human Neurodegenerative Diseases with Abnormal Protein AggregatesEmail this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
Human neurodegenerative diseases with abnormal protein aggregates are associated with aberrant post-translational modifications, solubility, aggregation and fibril formation of selected proteins which cannot be degraded by cytosolic proteases, ubiquitin[ndash]protesome system and autophagy, and, therefore, accumulate in cells and extracellular compartments as residual debris. In addition to the accumulation of "primary" proteins, several other mechanisms are involved in the degenerative process and probably may explain crucial aspects such as the timing, selective cellular vulnerability and progression of the disease in pa...
Source: Brain Pathology - September 2, 2009 Category: Neurology Authors: Anna Martínez, Manuel Portero-Otin, Reinald Pamplona, Isidre Ferrer Source Type: journals

Axonal Pathology and Loss Precede Demyelination and Accompany Chronic Lesions in a Spontaneously Occurring Animal Model of Multiple SclerosisEmail this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
Axonal damage has been highlighted recently as a cause of neurological disability in various demyelinating diseases, including multiple sclerosis, either as a primary pathological change or secondary due to myelin loss. To characterize and quantify axonal damage and loss in canine distemper demyelinating leukoencephalomyelitis (DL), formalin-fixed paraffin-embedded cerebella were investigated histochemically and immunohistochemically using the modified Bielschowsky's silver stain as well as antibodies against nonphosphorylated (n-NF), phosphorylated neurofilament (p-NF) and [beta]-amyloid precursor protein ([beta]-APP). In...
Source: Brain Pathology - August 25, 2009 Category: Neurology Authors: Frauke Seehusen, Wolfgang Baumgärtner Source Type: journals

Loss of Inhibitor of Growth (ING-4) Is Implicated in the Pathogenesis and Progression of Human AstrocytomasEmail this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
Inhibitor of growth 4 (ING-4) is a tumor suppressor gene that interacts with nuclear factor-kappaB (NF-[kappa]B) and represses its transcriptional activity. Several lines of evidence suggest that the tumor suppressor gene ING-4, the transcription factor NF-[kappa]B and its target genes matrix metalloproteases MMP-2, MMP-9 and urokinase plasminogen activator (u-PA) are critically involved in tumor invasion. The aim of the present study was to investigate immunohistochemically the expression pattern of ING-4, NF-[kappa]B and the NF-[kappa]B downstream targets MMP-2, MMP-9 and u-PA in human astrocytomas from 101 patients. We ...
Source: Brain Pathology - August 5, 2009 Category: Neurology Authors: George Klironomos, Vasiliki Bravou, Dionysios J Papachristou, George Gatzounis, John Varakis, Ekaterini Parassi, Maria Repanti, Helen Papadaki Source Type: journals

Leptin and Its Receptor Are Overexpressed in Brain Tumors and Correlate with the Degree of MalignancyEmail this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
Although leptin and its receptor (ObR) have emerged as important cancer biomarkers, the role of the leptin system in brain tumor development remains unknown. We screened 87 human brain tumor biopsies using immunohistochemistry and detected leptin and ObR in 55.2% and 60.9% cases, respectively. In contrast, leptin and ObR were absent in 14 samples of normal brain tissue. The presence of leptin correlated with ObR with overall concordance 80.5%. The leptin/ObR system was highly expressed in glioblastomas and anaplastic astrocytomas, while lower expression of both markers was noted in low-grade astrocytomas and gangliogliomas...
Source: Brain Pathology - July 19, 2009 Category: Neurology Authors: Mirko Riolfi, Rita Ferla, Luis Del Valle, Sergio Piña-Oviedo, Laura Scolaro, Rocco Micciolo, Micol Guidi, Marianna Terrasi, Gian Luigi Cetto, Eva Surmacz Source Type: journals

Oligomeric Aβ in Alzheimer's Disease: Relationship to Plaque and Tangle Pathology, APOE Genotype and Cerebral Amyloid AngiopathyEmail this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
Despite accumulating evidence of a central role for oligomeric amyloid [beta] (A[beta]) in the pathogenesis of Alzheimer's Disease (AD), there is scant information on the relationship between the levels and distribution of oligomeric A[beta] and those of other neurodegenerative abnormalities in AD. In the present study, we have found oligomeric A[beta] to be associated with both diffuse and neuritic plaques (mostly co-localized with A[beta]1[ndash]42) and with cerebrovascular deposits of A[beta] in paraffin sections of formalin-fixed human brain tissue. The amount of oligomeric A[beta] that was labeled in the sections corr...
Source: Brain Pathology - July 15, 2009 Category: Neurology Authors: Zoë van Helmond, James S. Miners, Patrick G. Kehoe, Seth Love Source Type: journals

Cerebral Microinfarcts Associated with Severe Cerebral β-Amyloid AngiopathyEmail this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
Cerebral amyloid angiopathy (CAA) is common in elderly individuals, especially those affected with Alzheimer's disease. Eighteen brains with severe SCAA (SCAA) were compared with 21 brains with mild CAA (MCAA) to investigate whether the presence of SCAA in the brains of demented patients was associated with a higher burden of old microinfarcts than those with MCAA. Immunohistochemistry for CD68 was employed to highlight old microinfarcts in tissue blocks from various brain regions. Old microinfarcts, manually counted by light microscopy, were present in 14 of 18 SCAA brains and in 7 of 21 MCAA brains (P = 0.01, two-tailed ...
Source: Brain Pathology - July 15, 2009 Category: Neurology Authors: Virawudh Soontornniyomkij, Matthew D. Lynch, Sherin Mermash, Justine Pomakian, Haleh Badkoobehi, Ryan Clare, Harry V. Vinters Source Type: journals

A Topographic Study of Minicolumnar Core Width by Lamina Comparison between Autistic Subjects and Controls: Possible Minicolumnar Disruption due to an Anatomical Element In-Common to Multiple LaminaeEmail this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
This study further characterizes this cortical deficit by comparing minicolumnar widths across layers. Brains from seven autistic patients and an equal number of age-matched controls were celloidin embedded, serially sectioned at 200 µm and Nissl stained with gallocyanin. Photomicrograph mosaics of the cortex were analyzed with computerized imaging methods to determine minicolumnar width at nine separate neocortical areas: Brodmann Area's (BA) 3b, 4, 9, 10, 11, 17, 24, 43 and 44. Each area was assessed at supragranular, granular and infragranular levels. Autistic subjects had smaller minicolumns whose dimensions varied ac...
Source: Brain Pathology - July 7, 2009 Category: Neurology Authors: Manuel F. Casanova, Ayman El-Baz, Eric Vanbogaert, Praveen Narahari, Andrew Switala Source Type: journals

N-CAM Dysfunction and Unexpected Accumulation of PSA-NCAM in Brain of Adult-Onset Autosomal-Dominant LeukodystrophyEmail this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
Previously, myelin from cerebral white matter (CWM) of two subjects of a family with orthochromatic adult-onset autosomal-dominant leukodystrophy (ADLD) was disclosed to exhibit defective large isoform of myelin-associated glycoprotein (L-MAG) and patchy distribution only in the elder subject. L-MAG and neural cell adhesion molecule (N-CAM) (N-CAM 180, 140, and 120) are structurally related and concur to myelin/axon interaction. In early developmental stages, in neurons and glia N-CAM is converted into polysialylated (PSA)-NCAM by two sialyltransferases sialyltransferase-X (STX) and polysialyltransferase-1 (PST). Notably, ...
Source: Brain Pathology - June 24, 2009 Category: Neurology Authors: Marco Piccinini, Barbara Buccinnà, Giovanni De Marco, Elisa Lupino, Cristina Ramondetti, Silvia Grifoni, Barbara Votta, Maria Teresa Giordana, Maria Teresa Rinaudo Source Type: journals

Central Nervous System Primitive Neuroectodermal Tumors: A Clinicopathologic and Genetic Study of 33 CasesEmail this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
Central nervous system (CNS) primitive neuroectodermal tumors (PNETs) include supratentorial, brain stem, and spinal cord tumors with medulloblastoma-like histopathology. The prognostic impact of various pathologic and genetic features has not been thoroughly investigated. After re-diagnosis of three infantile cases as atypical teratoid/rhabdoid tumor (AT/RT), 33 remaining CNS PNETs were retrieved for clinicopathologic and fluorescence in situ hybridization studies. Anaplastic and/or large cell features were seen in 18 of 33 (55%) examples and survival was decreased in these patients (P = 0.036). MYCN or MYCC gene amplific...
Source: Brain Pathology - June 24, 2009 Category: Neurology Authors: Amir Behdad, Arie Perry Source Type: journals

Association Between Lifetime Cigarette Smoking and Lewy Body AccumulationEmail this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
Cigarette smoking has been associated repeatedly in observational studies with decreased risk of Parkinson's disease (PD), but its relationship to the risk of dementia or Alzheimer's disease (AD) is inconsistent. All of these studies have used clinical diagnoses of disease. We tested the hypothesis that lifetime cigarette use might be associated with reduced risk of neuropathologic changes of Lewy-related pathology (LRP) in multiple brain regions or with reduced risk of consensus neuropathologic changes of AD in a prospective community-based study of brain aging and dementia, the Adult Changes in Thought (ACT) study. We ob...
Source: Brain Pathology - June 16, 2009 Category: Neurology Authors: Debby Tsuang, Eric B. Larson, Ge Li, Jane B. Shofer, Kathleen S. Montine, Mary Lou Thompson, Joshua A. Sonnen, Paul K. Crane, James B. Leverenz, Thomas J. Montine Source Type: journals

PET of Brain Prion Protein Amyloid in Gerstmann–Sträussler–Scheinker DiseaseEmail this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
In vivo amyloid PET imaging was carried out on six symptomatic and asymptomatic carriers of PRNP mutations associated with the Gerstmann[ndash]Sträussler[ndash]Scheinker (GSS) disease, a rare familial neurodegenerative brain disorder demonstrating prion amyloid neuropathology, using 2-(1-{6-[(2-[F-18]fluoroethyl)(methyl)amino]-2-naphthyl}ethylidene)malononitrile ([F-18]FDDNP). 2-Deoxy-2-[F-18]fluoro-d-glucose PET ([F-18]FDG) and magnetic resonance imaging (MRI) scans were also performed in each subject. Increased [F-18]FDDNP binding was detectable in cerebellum, neocortex and subcortical areas of all symptomatic gene carr...
Source: Brain Pathology - June 8, 2009 Category: Neurology Authors: Vladimir Kepe, Bernardino Ghetti, Martin R. Farlow, Mara Bresjanac, Karen Miller, Sung-Cheng Huang, Koon-Pong Wong, Jill R. Murrell, Pedro Piccardo, Francine Epperson, Grega Repov[scaron], Lojze M. Smid, Andrej Petrič, Prabha Siddarth, Jie Liu, Nag Source Type: journals

NG2+/Olig2+ Cells Are the Major Cycle-Related Cell Population of the Adult Human Normal BrainEmail this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
A persistent cycling cell population in the normal adult human brain is well established. Neural stem cells or neural progenitors have been identified in the subventricular zone and the dentate gyrus subgranular layer (SGL), two areas of persistent neurogenesis. Cycling cells in other human normal brain areas, however, remains to be established. Here, we determined the distribution and identity of these cells in the cortex, the white matter and the hippocampal formation of adult patients with and without chronic temporal lobe epilepsy using immunohistochemistry for the cell cycle markers Ki-67 (Mib-1) and minichromosome ma...
Source: Brain Pathology - May 28, 2009 Category: Neurology Authors: Sameh Geha, Johan Pallud, Marie-Pierre Junier, Bertrand Devaux, Nadine Leonard, Francine Chassoux, Hervé Chneiweiss, Catherine Daumas-Duport, Pascale Varlet Source Type: journals

Diffuse Leptomeningeal Glioneuronal Tumors: A New Entity?Email this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
The peculiar radiological and pathological findings of four pediatric cases admitted to the University Hospital of Padua between 1990 and 2007 are described. In all cases, the contrast-enhanced head and spine magnetic resonance images revealed thickened and abnormally enhancing subarachnoid spaces particularly at the level of basal cisterns and interhemispheric fissure. Furthermore, small cystic lesions scattered throughout the brain and mainly in the cerebellum were also visible. All patients were missing a well-defined intraparenchymal mass, although during the follow-up a small intramedullary lesion appeared within the ...
Source: Brain Pathology - May 27, 2009 Category: Neurology Authors: Marina Paola Gardiman, Matteo Fassan, Enrico Orvieto, Domenico D'Avella, Luca Denaro, Milena Calderone, Mariasavina Severino, Giovanni Scarsello, Elisabetta Viscardi, Giorgio Perilongo Source Type: journals

Differential Expression of Utrophin-A and -B Promoters in the Central Nervous System (CNS) of Normal and Dystrophic mdx MiceEmail this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
Utrophin (Utrn) is the autosomal homolog of dystrophin, the Duchene Muscular Dystrophy (DMD) locus product and of therapeutic interest, as its overexpression can compensate dystrophin's absence. Utrn is transcribed by Utrn-A and -B promoters with mRNAs differing at their 5' ends. However, previous central nervous system (CNS) studies used C-terminal antibodies recognizing both isoforms. As this distinction may impact upregulation strategies, we generated Utrn-A and -B promoter-specific antibodies, Taqman Polymerase chain reaction (PCR)-based absolute copy number assays, and luciferase-reporter constructs to study CNS of no...
Source: Brain Pathology - May 27, 2009 Category: Neurology Authors: Santhosh M. Baby, Sasha Bogdanovich, Gabriel Willmann, Utpal Basu, Olga Lozynska, Tejvir S. Khurana Source Type: journals

Neuropathogenesis of Naturally Occurring Encephalitis Caused by Listeria monocytogenes in RuminantsEmail this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
This study investigates the neuropathogenesis of listeric encephalitis in over 200 natural cases in cattle, sheep and goats by analyzing anatomical distribution, severity, bacterial load and temporal evolution of the lesions. Our results suggest that LM gains access to the brainstem of all three species via axonal migration not only along the trigeminal nerve, but also along other nerves. The ensuing encephalitis does not remain restricted to the brainstem. Rather, LM spreads further from the brainstem into rostral brain regions likely by intracerebral axonal migration. Significant differences in severity of the lesions an...
Source: Brain Pathology - May 21, 2009 Category: Neurology Authors: Anna Oevermann, Stefano Di Palma, Marcus G. Doherr, Carlos Abril, Andreas Zurbriggen, Marc Vandevelde Source Type: journals

Mitotic Epitopes Are Incorporated into Age-dependent Neurofibrillary Tangles in Niemann–Pick Disease Type CEmail this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
The mechanism underlying neurofibrillary tangles (NFTs) in Alzheimer's disease (AD) and other neurodegenerative disorders remains elusive. Niemann[ndash]Pick disease type C (NPC) is a kind of genetic neurovisceral disorder in which the intracellular sequestration of cholesterol and other lipids in neurons, NFT formation and neuronal degeneration in brain are the neuropathology hallmarks. The age of onset and progression of the disease vary dramatically. We have analyzed the hippocampus from 17 NPC cases, aged from 7 months to 55 years, to depict the temporal characteristics of NFT formation. Unexpectedly, classic NFT was o...
Source: Brain Pathology - May 20, 2009 Category: Neurology Authors: Min Zhang, Xuezhen Wang, Feng Jiang, Wei Wang, Inez Vincent, Bitao Bu Source Type: journals

Fetal Hydrocephalus Caused by Cryptic Intraventricular HemorrhageEmail this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
Cryptic intracerebral hemorrhage as an etiological factor in fetal hydrocephalus has been postulated but not described at autopsy. Four fetuses with overt hydrocephalus diagnosed by in utero ultrasound examination were examined at autopsy at 19[ndash]22 weeks gestation. Although a hemorrhagic etiology was not evident on ultrasound, hemosiderin-containing macrophages and associated reactive changes were found to obstruct the otherwise well-formed cerebral aqueduct in all four. Coagulopathy due to thrombocytopenia was implicated in one case. Anomalies involving other parts of the body were identified in two cases, although a...
Source: Brain Pathology - May 20, 2009 Category: Neurology Authors: Belinda Lategan, Bernard N. Chodirker, Marc R. Del Bigio Source Type: journals

Alterations of Zinc Transporter Proteins ZnT-1, ZnT-4 and ZnT-6 in Preclinical Alzheimer's Disease BrainEmail this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
Our previous studies demonstrate alterations of zinc (Zn) transporter proteins ZnT-1, ZnT-4 and ZnT-6 in vulnerable brain regions of subjects with mild cognitive impairment (MCI), and early and late stage Alzheimer's disease (AD), suggesting disruptions of Zn homeostasis may play a role in the pathogenesis of AD. A preclinical stage of AD (PCAD) has been described in which subjects show no overt clinical manifestations of AD, but demonstrate significant AD pathology at autopsy. To determine if alterations of ZnT proteins occur in PCAD, we measured ZnT-1, ZnT-4 and ZnT-6 in the hippocampus/parahippocampal gyrus (HPG) and ce...
Source: Brain Pathology - April 18, 2009 Category: Neurology Authors: Ganna Lyubartseva, Jennifer L. Smith, William R. Markesbery, Mark A. Lovell Source Type: journals

Galectins and GliomasEmail this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
Malignant gliomas, especially glioblastomas, are associated with a dismal prognosis. Despite advances in diagnosis and treatment, glioblastoma patients still have a median survival expectancy of only 14 months. This poor prognosis can be at least partly explained by the fact that glioma cells diffusely infiltrate the brain parenchyma and exhibit decreased levels of apoptosis, and thus resistance to cytotoxic drugs. Galectins are a family of mammalian beta-galactoside-binding proteins characterized by a shared characteristic amino acid sequence. They are expressed differentially in normal vs. neoplastic tissues and are know...
Source: Brain Pathology - April 9, 2009 Category: Neurology Authors: Marie Le Mercier, Shannon Fortin, Véronique Mathieu, Robert Kiss, Florence Lefranc Source Type: journals

Pi3K-mTOR Signaling and AMOG Expression in Epilepsy-associated Glioneuronal TumorsEmail this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
Gangliogliomas (GGs) and dysembryoplastic neuroepithelial tumors (DNTs) represent the most frequent type of neoplasms in pediatric medically intractable epilepsy. Several data suggest a pathogenetic relationship between GGs and other glioneuronal malformations of cortical development (MCDs), including activation of the Pi3K-mTOR signaling pathway. To further reveal these pathogenetic similarities, we investigated immunocytochemically the expression of phosphorylated (p)-PDK1, p-AKT, p-mTOR, p-4E-BP1, p-eIF4G, p-p70S6K and p-S6, the effector proteins ERM (ezrin/radixin/moesin) and the pathway regulator AMOG (adhesion molecu...
Source: Brain Pathology - April 9, 2009 Category: Neurology Authors: Karin Boer, Dirk Troost, Wendy Timmermans, Peter C. van Rijen, Wim G.M. Spliet, Eleonora Aronica Source Type: journals

Cerebellar Cortical Demyelination in the Murine Cuprizone ModelEmail this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
In multiple sclerosis, demyelination occurs beside the white-matter structures and in the cerebral and cerebellar cortex. We have previously shown that, in the cuprizone model, demyelination is present not only in the corpus callosum but also in the cerebral cortex. Here, we have performed a detailed analysis of the dynamics of de- and remyelination in the cerebellar cortex and white matter at nine timepoints in two cerebellar regions. To induce demyelination, C57BL/6 mice were fed with 0.2% cuprizone for 12 weeks followed by a recovery of 8 weeks. Both cortex and white-matter structures were significantly demyelinated aft...
Source: Brain Pathology - April 9, 2009 Category: Neurology Authors: Thomas Skripuletz, Jens-Heiko Bussmann, Viktoria Gudi, Paraskevi N. Koutsoudaki, Refik Pul, Darius Moharregh-Khiabani, Maren Lindner, Martin Stangel Source Type: journals

TDP-43 Redistribution Is an Early Event in Sporadic Amyotrophic Lateral SclerosisEmail this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
Amyotrophic lateral sclerosis (ALS) is a neurodegenerative disorder consisting of progressive loss of motor neurons. TDP-43 has been identified as a component of ubiquitin-immunoreactive inclusions of motor neurons in ALS. We focused on the diffuse cytoplasmic TDP-43 immunoreactivity in ALS neurons, and quantitatively assessed it in comparison with skein/round TDP-43 and ubiquitin immunostaining in motor neurons of 30 sporadic ALS cases. The percentage of spinal motor neurons with cytoplasmic TDP-43 immunoreactivity was higher than that of ubiquitin-immunoreactive ones. The percentage of TDP-43-positive motor neurons was i...
Source: Brain Pathology - March 17, 2009 Category: Neurology Authors: Maria Teresa Giordana, Marco Piccinini, Silvia Grifoni, Giovanni De Marco, Marco Vercellino, Michela Magistrello, Alessia Pellerino, Barbara Buccinnà, Elisa Lupino, Maria Teresa Rinaudo Source Type: journals

Neuroprotective Effects of Calmodulin Peptide 76-121aa: Disruption of Calmodulin Binding to Mutant HuntingtinEmail this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
Huntington's disease (HD) is a neurodegenerative disease caused by mutant huntingtin protein containing an expanded polyglutamine tract, which may cause abnormal protein[ndash]protein interactions such as increased association with calmodulin (CaM). We previously demonstrated in HEK293 cells that a peptide containing amino acids 76-121 of CaM (CaM-peptide) interrupted the interaction between CaM and mutant huntingtin, reduced mutant huntingtin-induced cytotoxicity and reduced transglutaminase (TG)-modified mutant huntingtin. We now report that adeno-associated virus (AAV)-mediated expression of CaM-peptide in differentiate...
Source: Brain Pathology - March 11, 2009 Category: Neurology Authors: Nichole L. Dudek, Ying Dai, Nancy A. Muma Source Type: journals

Array-Based Genomics in Glioma ResearchEmail this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
Over the years, several relevant biomarkers with a potential clinical interest have been identified in gliomas using various techniques, such as karyotype, microsatellite analysis, fluorescent in situ hybridization and chromosome comparative genomic hybridization. Despite their pivotal contribution to our understanding of gliomas biology, clinical application of these approaches has been limited by technological and clinical complexities. In contrast, genomic arrays (array-based comparative genomic hybridization and single nucleotide polymorphisms array) have emerged as promising technologies for clinical use in the settin...
Source: Brain Pathology - March 6, 2009 Category: Neurology Authors: Ahmed Idbaih, Emmanuelle Crinière, Keith L. Ligon, Olivier Delattre, Jean-Yves Delattre Source Type: journals

Lithium Restores Neurogenesis in the Subventricular Zone of the Ts65Dn Mouse, a Model for Down SyndromeEmail this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
Down syndrome (DS), a high-incidence genetic pathology, involves brain hypoplasia and mental retardation. Emerging evidence suggests that reduced neurogenesis may be a major determinant of brain underdevelopment in DS. To establish whether it is possible to improve neurogenesis in DS, Ts65Dn mice[mdash]the most widely used model for DS[mdash]and euploid mice were treated with control or lithium chow for 1 month. During the last 3 days animals received one daily injection of 5-bromo-2-deoxyuridine (BrdU)[mdash]a marker of proliferating cells[mdash]and were sacrificed 24 h after the last injection. Neurogenesis was examined ...
Source: Brain Pathology - March 4, 2009 Category: Neurology Authors: Patrizia Bianchi, Elisabetta Ciani, Andrea Contestabile, Sandra Guidi, Renata Bartesaghi Source Type: journals

Mitochondrial ATP-Synthase in the Entorhinal Cortex Is a Target of Oxidative Stress at Stages I/II of Alzheimer's Disease PathologyEmail this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
In this study, we investigate whether brain proteins are locally modified by oxidative stress at the first stages of AD-related pathology when morphological lesions are restricted to the entorhinal and transentorhinal cortices of neurofibrillary pathology (stages I/II of Braak). Using a proteomic approach, we show that the [alpha] subunit of the mitochondrial adenosine triphosphate (ATP)-synthase is distinctly lipoxidized in the entorhinal cortex at Braak stages I/II compared with age-matched controls. In addition, ATP-synthase activity is significantly lower in Braak stages I/II than age-matched control, while electron tr...
Source: Brain Pathology - February 28, 2009 Category: Neurology Authors: Beatrice Terni, Jordi Boada, Manuel Portero-Otin, Reinald Pamplona, Isidro Ferrer Source Type: journals

Morpho-Physiologic Characteristics of Dorsal Subicular Network in Mice after Pilocarpine-Induced Status EpilepticusEmail this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
The goal of this study was to examine the morpho-physiologic changes in the dorsal subiculum network in the mouse model of temporal lobe epilepsy using extracellular recording, juxtacellular and immunofluorescence double labeling, and anterograde tracing methods. A significant loss of total dorsal subicular neurons, particularly calbindin, parvalbumin (PV) and immunopositive interneurons, was found at 2 months after pilocarpine-induced status epilepticus (SE). However, the sprouting of axons from lateral entorhinal cortex (LEnt) was observed to contact with surviving subicular neurons. These neurons had two predominant dis...
Source: Brain Pathology - February 27, 2009 Category: Neurology Authors: De Fu He, Dong Liang Ma, Yong Cheng Tang, Jerome Engel Jr, Anatol Bragin, Feng Ru Tang Source Type: journals

Lesion Profiling at Primary Isolation in RIII Mice Is Insufficient in Distinguishing BSE from Classical ScrapieEmail this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
Primary isolation of bovine spongiform encephalopathy (BSE) in RIII mice generates a lesion profile believed to be reproducible and distinct from that produced by classical scrapie. This profile, which is characterized by peaks at gray matter areas 1, 4 and 7 (dorsal medulla, hypothalamus and septal nuclei), is used to diagnose BSE on primary isolation. The aim of this study was to investigate whether the BSE agent could be present in sheep diagnosed with classical scrapie, using lesion profiles in RIII mice as a discriminatory method. Sixty-two positive scrapie field cases were collected from individual farms between 1996...
Source: Brain Pathology - February 26, 2009 Category: Neurology Authors: Katy E. Beck, Melanie Chaplin, Michael Stack, Rosemary E. Sallis, Sarah Simonini, Richard Lockey, John Spiropoulos Source Type: journals

Global Expression Profiling in Epileptogenesis: Does It Add to the Confusion?Email this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
Since the inception of global gene expression profiling platforms in the mid-1990s, there has been a significant increase in publications of differentially expressed genes in the process of epileptogenesis. In particular for mesial temporal lobe epilepsy, the presence of a latency period between the first manifestation of seizures to chronic epilepsy provides the opportunity for therapeutic interventions at the molecular biology level. Using global expression profiling techniques, approximately 2000 genes have been published demonstrating differential expression in mesial temporal epilepsy. The majority of these changes, h...
Source: Brain Pathology - February 26, 2009 Category: Neurology Authors: Yi Yuen Wang, Paul Smith, Michael Murphy, Mark Cook Source Type: journals

A2B5 Cells from Human Glioblastoma have Cancer Stem Cell PropertiesEmail this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
Glioblastomas, like other cancers, harbor small cell populations with the capability of sustaining tumor formation. These cells are referred to as cancer stem cells. We isolated cells expressing the surface marker A2B5 from three human glioblastomas (GBM) and showed that after grafting into nude mice, they generated dense and highly infiltrative tumors. Then, we extensively studied A2B5+ cells isolated from 11 human GBM. These cells display neurosphere-like, self-renewal, asymmetrical cell division properties and have multipotency capability. Stereotactic xenografts of dissociated A2B5+-derived secondary spheres revealed t...
Source: Brain Pathology - February 22, 2009 Category: Neurology Authors: Aurélie Tchoghandjian, Nathalie Baeza, Carole Colin, Myriam Cayre, Philippe Metellus, Christophe Beclin, L'Houcine Ouafik, Dominique Figarella-Branger Source Type: journals

Reduced Activity of CD13/Aminopeptidase N (APN) in Aggressive Meningiomas Is Associated with Increased Levels of SPARCEmail this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
In this study, we examined the expression and function of the membrane alanyl-aminopeptidase [mAAP, aminopeptidase N (APN), CD13, EC3.4.11.2] zinc-dependent ectopeptidase in meningiomas and meningioma cell lines, based on its prior association with tumor invasion in colorectal and renal carcinomas. We found a significant reduction of APNmRNA and protein expression, as well as enzymatic activity, in high-grade meningiomas. While meningioma tumor cell proliferation was not affected by either pharmacologic APN inhibition or siRNA-mediated APN silencing, APN pharmacologic and siRNA knockdown significantly reduced meningioma ce...
Source: Brain Pathology - February 20, 2009 Category: Neurology Authors: Christian Mawrin, Carmen Wolke, Daniela Haase, Sabine Krüger, Raimund Firsching, Gerburg Keilhoff, Werner Paulus, David H. Gutmann, Anita Lal, Uwe Lendeckel Source Type: journals

Claudin 6 Is a Positive Marker for Atypical Teratoid/Rhabdoid TumorsEmail this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
Atypical teratoid/rhabdoid tumors (AT/RTs) are highly aggressive pediatric brain tumors characterized by the presence of rhabdoid cells and negative immunostaining for INI1 (BAF47). Histogenesis is unknown and diagnosis can be challenging because of their extreme morphological and immunophenotypic heterogeneity. Currently no signature markers other than INI1 loss have been identified. To search for possible candidate proteins of interest in AT/RTs, Affymetrix GeneChip® microarrays were utilized to investigate nine AT/RTs vs. 124 other tumor samples. The most distinctive gene identified was claudin 6 (CLDN6), a key compone...
Source: Brain Pathology - February 11, 2009 Category: Neurology Authors: Diane K. Birks, Bette K. Kleinschmidt-DeMasters, Andrew M. Donson, Valerie N. Barton, Sean A. McNatt, Nicholas K. Foreman, Michael H. Handler Source Type: journals

Computer- and Internet-Based Tools for Neuropathology in the 21st CenturyEmail this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
(Source: Brain Pathology)
Source: Brain Pathology - February 4, 2009 Category: Neurology Authors: Alexander R. Judkins Source Type: journals

Modified C-Reactive Protein Is Expressed by Stroke Neovessels and Is a Potent Activator of Angiogenesis In VitroEmail this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
Native C-reactive protein (nCRP) is a pentameric oligo-protein and an acute phase reactant whose serum expression is increased in patients with inflammatory disease. We have identified by immunohistochemistry, significant expression of a tissue-binding insoluble modified version or monomeric form of CRP (mCRP) associated with angiogenic microvessels in peri-infarcted regions of patients studied with acute ischaemic stroke. mCRP, but not nCRP was expressed in the cytoplasm and nucleus of damaged neurons. mCRP co-localized with CD105, a marker of angiogenesis in regions of revascularisation. In vitro investigations demonstra...
Source: Brain Pathology - January 22, 2009 Category: Neurology Authors: Mark Slevin, Sabine Matou-Nasri, Marta Turu, Ana Luque, Norma Rovira, Lina Badimon, Susana Boluda, Lawrence Potempa, Coral Sanfeliu, Nuria de Vera, Jerzy Krupinski Source Type: journals

Immunolocalization of Fascin, an Actin-Bundling Protein and Glial Fibrillary Acidic Protein in Human Astrocytoma CellsEmail this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
In this report, we investigate fascin in astrocytomas. We show that fascin is expressed in astrocytes and in a panel of human astrocytoma cell lines. Immunofluorescence analysis demonstrates that fascin and the intermediate filament protein, glial fibrillary acidic protein (GFAP), are both expressed in the perinuclear region and within cytoplasmic processes of astrocytes and astrocytoma cells. Amino acid residues within the NH2 terminus of GFAP can undergo phosphorylation; these modifications regulate intermediate filament disassembly and occur during cytokinesis. We show that fascin and specific phosphorylated species of ...
Source: Brain Pathology - January 15, 2009 Category: Neurology Authors: Soma Mondal, Peter Dirks, James T. Rutka Source Type: journals

Reduction of Cerebral Oxidative Stress Following Environmental Enrichment in Mice with Alzheimer-Like PathologyEmail this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
This study identifies a thus far undescribed antagonizing effect of environmental stimulation on Alzheimer's disease-related oxidative damage. (Source: Brain Pathology)
Source: Brain Pathology - January 8, 2009 Category: Neurology Authors: Arne Herring, Mareike Blome, Oliver Ambrée, Norbert Sachser, Werner Paulus, Kathy Keyvani Source Type: journals

Analysis of the Repressor Element-1 Silencing Transcription Factor/Neuron-Restrictive Silencer Factor Occupancy of Non-Neuronal Genes in Peripheral Lymphocytes from Patients with Huntington's DiseaseEmail this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
We have previously demonstrated that the transcription of neuronal repressor element-1/neuron-restrictive silencer element (RE1/NRSE)-regulated genes is reduced in the brain of subjects with Huntington's disease (HD) as a result of increased binding of the repressor element-1 silencing transcription factor/neuron-restrictive silencer factor (REST/NRSF) to its RE1/NRSE targets. As specific non-neuronal REST/NRSF-regulated genes have been identified in the human genome, we exploited the possibility that the binding of REST/NRSF to its target RE1/NRSE sites may also be altered in the peripheral tissues of HD patients. Our res...
Source: Brain Pathology - January 7, 2009 Category: Neurology Authors: Manuela Marullo, Marta Valenza, Caterina Mariotti, Stefano Di Donato, Elena Cattaneo, Chiara Zuccato Source Type: journals