Familial Danish dementia: a novel form of cerebral amyloidosis associated with deposition of both amyloid-Dan and amyloid-beta.

abstract：:The prognosis of gliomas is generally poor since these tumors elude established therapeutic approaches. Immunotherapy might present an effective therapy in particular because the glioma cells are diffusely dispersed in the infiltration zone of the tumor and show a strong propensity to invade the surrounding brain alon...

journal_title：Journal of neuropathology and experimental neurology

authors： Vetter M,Hofer MJ,Roth E,Pircher HP,Pagenstecher A

更新日期：2009-05-01 00:00:00

abstract：:Lysosomal storage disorders constitute a large group of genetic diseases, many of which are characterized by mental retardation and other neurologic symptoms. The mechanisms of neural dysfunction remain poorly understood. Because neural progenitor cells (NPCs) are fundamentally important to normal brain development an...

journal_title：Journal of neuropathology and experimental neurology

authors： Walton RM,Wolfe JH

更新日期：2007-08-01 00:00:00

abstract：:Oxidative stress has been proposed as a potential mechanism for neurodegenerative diseases, such as Alzheimer disease (AD), Parkinson disease (PD), and amyotrophic lateral sclerosis (ALS). In response to oxidative stress, the levels of numerous cytoprotective products are increased via alteration of the Kelch-like ECH...

journal_title：Journal of neuropathology and experimental neurology

authors： Tanji K,Maruyama A,Odagiri S,Mori F,Itoh K,Kakita A,Takahashi H,Wakabayashi K

更新日期：2013-01-01 00:00:00

abstract：:The aim of this study was to identify aberrantly expressed proteins in pediatric primitive neuroectodermal tumors (PNETs) and ependymomas. Tumor tissue of 29 PNET and 12 ependymoma patients was subjected to 2-dimensional difference gel electrophoresis. Gel analysis resulted in 79 protein spots being differentially exp...

journal_title：Journal of neuropathology and experimental neurology

authors： de Bont JM,den Boer ML,Kros JM,Passier MM,Reddingius RE,Smitt PA,Luider TM,Pieters R

更新日期：2007-06-01 00:00:00

abstract：:Mutations in dysferlin cause an inherited muscular dystrophy because of defective membrane repair. Three interacting partners of dysferlin are also implicated in membrane resealing: caveolin-3 (in limb girdle muscular dystrophy type 1C), annexin A1, and the newly identified protein mitsugumin 53 (MG53). Mitsugumin 53 ...

journal_title：Journal of neuropathology and experimental neurology

authors： Waddell LB,Lemckert FA,Zheng XF,Tran J,Evesson FJ,Hawkes JM,Lek A,Street NE,Lin P,Clarke NF,Landstrom AP,Ackerman MJ,Weisleder N,Ma J,North KN,Cooper ST

更新日期：2011-04-01 00:00:00

abstract：:In many degenerative and metabolic muscle diseases cells die without a marked inflammatory response. A potential mechanism how this could be accomplished is apoptotic cell death. Since there are no data available about apoptosis in cells of myogenic origin, we examined rat skeletal myoblasts for their potential to und...

journal_title：Journal of neuropathology and experimental neurology

authors： Stangel M,Zettl UK,Mix E,Zielasek J,Toyka KV,Hartung HP,Gold R

更新日期：1996-01-01 00:00:00

abstract：:The development and differentiation of oligodendrocytes (OC) in developing mouse spinal cord (MSC) were investigated by correlative analysis of light and electron microscopy (EM), and immunoperoxidase studies for glial fibrillary acidic protein (GFAP) and myelin basic protein (MBP). The sequential development of glial...

journal_title：Journal of neuropathology and experimental neurology

authors： Choi BH

更新日期：1986-09-01 00:00:00

abstract：:Growing evidence indicates that alterations within the peripheral nervous system (PNS) are involved at an early stage in the amyotrophic lateral sclerosis (ALS) pathogenetic cascade. In this study, magnetic resonance imaging (MRI), neurophysiologic analyses, and histologic analyses were used to monitor the extent of P...

journal_title：Journal of neuropathology and experimental neurology

authors： Riva N,Chaabane L,Peviani M,Ungaro D,Domi T,Dina G,Bianchi F,Spano G,Cerri F,Podini P,Corbo M,Carro UD,Comi G,Bendotti C,Quattrini A

更新日期：2014-07-01 00:00:00

abstract：:Selenoprotein P (Sepp1) is involved in selenium homeostasis. Mice with a deletion of Sepp1, replacement of it by the shortened form Sepp1(Delta240-361), or deletion of its receptor apolipoprotein E receptor 2 develop severe neurologic dysfunction when fed low-selenium diet. Because the brainstems of Sepp1(-/-) mice ha...

journal_title：Journal of neuropathology and experimental neurology

authors： Valentine WM,Abel TW,Hill KE,Austin LM,Burk RF

更新日期：2008-01-01 00:00:00

abstract：:Cutaneous and leptomeningeal vascular malformations are hallmarks of the Sturge-Weber Syndrome (SWS), resulting in chronic ischemic tissue damage. The mechanisms underlying the pathobiology of these progressive lesions are unknown. Aberrant expression of angiogenic factors has been implicated in the genesis and mainte...

journal_title：Journal of neuropathology and experimental neurology

authors： Comati A,Beck H,Halliday W,Snipes GJ,Plate KH,Acker T

更新日期：2007-01-01 00:00:00

abstract：:Friedreich's Ataxia (FRDA) is caused by a homozygous intronic GAA expansion in the FXN gene. FRDA affects primarily the peripheral nervous system (PNS) with cumulative evidence from postmortem studies and in vitro models suggesting a developmental component of its pathology. In the present study, we aimed at gaining f...

journal_title：Journal of neuropathology and experimental neurology

authors： Indelicato E,Nachbauer W,Eigentler A,Rudzki D,Wanschitz J,Boesch S

更新日期：2018-12-01 00:00:00

abstract：:To develop improved therapies for medulloblastoma, we studied the ability of a neuroattenuated HSV-1 ICP34.5 mutant (variant-1716) to replicate within and destory an authentic medulloblastoma cell line known as Med 283 (D283) using immunohistochemistry, in situ hybridization, and viral titrations. In vitro studies sho...

journal_title：Journal of neuropathology and experimental neurology

authors： Lasner TM,Kesari S,Brown SM,Lee VM,Fraser NW,Trojanowski JQ

更新日期：1996-12-01 00:00:00

abstract：:Little is known about the molecular features of desmoplastic infantile ganglioglioma (DIG) and desmoplastic infantile astrocytoma (DIA). We performed a genome-wide DNA copy number analysis in combination with a multiplex ligation-dependent probe amplification-based analysis of copy number changes of candidate genes in...

journal_title：Journal of neuropathology and experimental neurology

authors： Gessi M,Zur Mühlen A,Hammes J,Waha A,Denkhaus D,Pietsch T

更新日期：2013-09-01 00:00:00

abstract：:A working group supported by the Office of Rare Diseases of the National Institutes of Health formulated neuropathologic criteria for corticobasal degeneration (CBD) that were subsequently validated by an independent group of neuropathologists. The criteria do not require a specific clinical phenotype, since CBD can h...

journal_title：Journal of neuropathology and experimental neurology

authors： Dickson DW,Bergeron C,Chin SS,Duyckaerts C,Horoupian D,Ikeda K,Jellinger K,Lantos PL,Lippa CF,Mirra SS,Tabaton M,Vonsattel JP,Wakabayashi K,Litvan I,Office of Rare Diseases of the National Institutes of Health.

更新日期：2002-11-01 00:00:00

abstract：:The frontotemporal dementias (FTDs) are a heterogeneous group of neurodegenerative disorders that are characterized clinically by dementia, personality changes, language impairment, and occasionally extrapyramidal movement disorders. Historically, the diagnosis and classification of FTDs has been fraught with difficul...

journal_title：Journal of neuropathology and experimental neurology

authors： Mott RT,Dickson DW,Trojanowski JQ,Zhukareva V,Lee VM,Forman M,Van Deerlin V,Ervin JF,Wang DS,Schmechel DE,Hulette CM

更新日期：2005-05-01 00:00:00

abstract：:Because the presence of carbonic anhydrase C (CA C) has been demonstrated in the oligodendrocytes of the mouse, rat and man, anti-CA C serum has been considered to be a possible specific marker for these cells. In order to determine its value in human neurooncology, specimens from 110 human tumors from the central and...

journal_title：Journal of neuropathology and experimental neurology

authors： Nakagawa Y,Perentes E,Rubinstein LJ

更新日期：1987-07-01 00:00:00

abstract：:To determine whether arterial wall degeneration, in combination with hemodynamic insult, causes cerebral artery aneurysms in a dog model, we simulated the geometry and hemodynamics of a human artery by surgical reconstruction of both common carotid arteries in 12 dogs. The dogs were then randomly assigned to one of th...

journal_title：Journal of neuropathology and experimental neurology

authors： Zhu YQ,Li MH,Yan L,Tan HQ,Cheng YS

更新日期：2014-09-01 00:00:00

abstract：:Hereditary diffuse leukoencephalopathy with spheroids (HDLS) is a familial neurodegenerative disease clinically characterized by progressive cognitive and motor dysfunction. Mutations in the colony-stimulating factor 1 receptor (CSF1R) gene have recently been identified in HDLS patients. The presence of diffuse axonal...

journal_title：Journal of neuropathology and experimental neurology

authors： Riku Y,Ando T,Goto Y,Mano K,Iwasaki Y,Sobue G,Yoshida M

更新日期：2014-12-01 00:00:00

abstract：:Versican is a large extracellular proteoglycan and is expressed in a variety of tissues including the central nervous system. A malignant astrocytoma cell line U87 with high motility expressed a higher level of versican than another malignant astrocytoma cell line U343 with lower motility. We observed that the U87 cel...

journal_title：Journal of neuropathology and experimental neurology

authors： Ang LC,Zhang Y,Cao L,Yang BL,Young B,Kiani C,Lee V,Allan K,Yang BB

更新日期：1999-06-01 00:00:00

abstract：:In situ hybridization (ISH) for JC virus (JCV) is generally applied for the diagnosis of progressive multifocal leukoencephalopathy (PML). To explore the usefulness of immunohistochemistry (IHC) for JCV early proteins, 14 paraffin-embedded postmortem brain specimens with histologic features compatible with PML were te...

journal_title：Journal of neuropathology and experimental neurology

authors： Muñoz-Mármol AM,Mola G,Fernández-Vasalo A,Vela E,Mate JL,Ariza A

更新日期：2004-11-01 00:00:00

abstract：:Mucopolysaccharidosis IIID (MPS IIID) is one of the rarest of the MPS-III syndromes. To date, the clinical manifestations of 10 patients have been reported, the deficient N-acetylglucosamine 6-sulfatase (G6S) enzyme has been purified, and the G6S gene has been cloned, sequenced and localized. However, morphological ma...

journal_title：Journal of neuropathology and experimental neurology

authors： Jones MZ,Alroy J,Rutledge JC,Taylor JW,Alvord EC Jr,Toone J,Applegarth D,Hopwood JJ,Skutelsky E,Ianelli C,Thorley-Lawson D,Mitchell-Herpolsheimer C,Arias A,Sharp P,Evans W,Sillence D,Cavanagh KT

更新日期：1997-10-01 00:00:00

abstract：:Histopathologic evaluation of brain tissue derived from surgically treated patients with medically refractory temporal lobe epilepsy (TLE) frequently reveals structural brain lesions in the surgical specimen. While several of the most commonly encountered lesions such as low-grade neoplasms or vascular malformations a...

journal_title：Journal of neuropathology and experimental neurology

authors： Kasper BS,Stefan H,Buchfelder M,Paulus W

更新日期：1999-01-01 00:00:00

abstract：:Injury to neonatal nerves, unlike adult nerves, results in poor regeneration and extensive motoneuron death. We examined whether exposure to a more mature nerve environment could rescue axotomized motoneurons following neonatal injury. The sciatic nerve in 1 hindlimb of 3-day-old (P3) rats was transected and the cut e...

journal_title：Journal of neuropathology and experimental neurology

authors： Low HL,Nogradi A,Vrbová G,Greensmith L

更新日期：2003-01-01 00:00:00

abstract：:The diagnosis of Alzheimer disease (AD) according to current criteria is a combined clinical and pathological exercise. The clinical discrimination of AD from other types of dementia may be complicated when the patient suffers from more than one disease. In particular the concomitant presence of other neurological con...

journal_title：Journal of neuropathology and experimental neurology

authors： Nagy Z,Esiri MM,Jobst KA,Morris JH,King EM,McDonald B,Joachim C,Litchfield S,Barnetson L,Smith AD

更新日期：1997-02-01 00:00:00

abstract：:Maternal cigarette smoking during pregnancy is associated with a significantly increased risk of Sudden Infant Death Syndrome (SIDS). This study investigated the effects of prenatal exposure to carbon monoxide (CO), a major component of cigarette smoke, on the neuroglial and neurochemical development of the medulla in...

journal_title：Journal of neuropathology and experimental neurology

authors： Tolcos M,McGregor H,Walker D,Rees S

更新日期：2000-03-01 00:00:00

abstract：:Side population (SP) cells identified using the Hoechst 33342 fluorescent dye efflux technique overlap with the tumor stem cell fraction that is responsible for tumorigenesis and recurrence in malignant gliomas. Because diffuse invasion of glioma cells represents the main obstacle to successful therapy and underlies r...

journal_title：Journal of neuropathology and experimental neurology

authors： Weber K,Paulus W,Senner V

更新日期：2010-06-01 00:00:00

abstract：:The phenotypic identities and characterization of neural networks disrupted after neonatal hypoxia-ischemia (HI) in the preterm brain remain to be elucidated. Interruption of the central serotonergic (5-hydroxytryptamine [5-HT]) system can lead to numerous functional deficits, many of which match those in human preter...

journal_title：Journal of neuropathology and experimental neurology

authors： Wixey JA,Reinebrant HE,Buller KM

更新日期：2011-01-01 00:00:00

abstract：:Loss of chromosome 1p/19q in oligodendrogliomas represents a powerful predictor of good prognosis. Expression of internexin (INA), a neuronal specific intermediate filament protein, has recently been proposed as a surrogate marker for 1p/19q deletion based on the high degree of correlation between both parameters in o...

journal_title：Journal of neuropathology and experimental neurology

authors： Buckley PG,Alcock L,Heffernan J,Woods J,Brett F,Stallings RL,Farrell MA

更新日期：2011-03-01 00:00:00

abstract：:A spontaneous spongy degeneration of the brain stem and spinal cord was discovered in a murine model of accelerated senescence (SAM), cared for under both conventional (SAM-P/8) and specific pathogen-free (SAM-P/8/Ta) conditions. SAM-P/8 and SAM-P/8/Ta showed no clinical neurological abnormalities, yet there was a det...

journal_title：Journal of neuropathology and experimental neurology

authors： Yagi H,Irino M,Matsushita T,Katoh S,Umezawa M,Tsuboyama T,Hosokawa M,Akiguchi I,Tokunaga R,Takeda T

更新日期：1989-09-01 00:00:00

abstract：:Fas-apoptotic inhibitory molecule 2 (Faim2) is a neuron-specific membrane protein and a member of the evolutionary conserved lifeguard apoptosis regulatory gene family. Its neuroprotective effect in acute neurological diseases has been demonstrated in an in vivo model of focal cerebral ischemia. Here we show that Faim...

journal_title：Journal of neuropathology and experimental neurology

authors： Tauber SC,Harms K,Falkenburger B,Weis J,Sellhaus B,Nau R,Schulz JB,Reich A

更新日期：2014-01-01 00:00:00