Ultrastructural analysis and TUNEL demonstrate motor neuron apoptosis in Werdnig-Hoffmann disease.

abstract：:Temporal lobe epilepsy (TLE) is characterized by hippocampal sclerosis together with profound losses and phenotypic changes of different classes of interneurons, including those expressing somatostatin (SRIF). To understand the functional significance of the plasticity of SRIF transmission in TLE, unraveling the statu...

journal_title：Journal of neuropathology and experimental neurology

authors： Csaba Z,Pirker S,Lelouvier B,Simon A,Videau C,Epelbaum J,Czech T,Baumgartner C,Sperk G,Dournaud P

更新日期：2005-11-01 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：:Whatever the cause of myelin damage of the peripheral nervous system, the initial attack on myelin by a dysimmune process may begin either at the internodal area or in the paranodal and nodal regions. The term "nodo-paranodopathy" was first applied to some "axonal Guillain-Barré syndrome" subtypes, then extended to ca...

journal_title：Journal of neuropathology and experimental neurology

authors： Vallat JM,Magy L,Corcia P,Boulesteix JM,Uncini A,Mathis S

更新日期：2020-03-01 00:00:00

abstract：:There are phenotypic and functional differences between vascular endothelium from different tissues and between microvascular and macrovascular endothelial cells (ECs) from the same tissue. Relatively little is known about the human blood-nerve barrier (BNB). We report the development of an in vitro BNB model using pr...

journal_title：Journal of neuropathology and experimental neurology

authors： Yosef N,Xia RH,Ubogu EE

更新日期：2010-01-01 00:00:00

abstract：:A single oral dose of clofibrate (30 mg/100 gm body weight) given to 250-300 gm male rats produced myotonic discharges from both the gastrocnemius and soleus muscles 2 hours after feeding. Rats fed the same dosage for 14 consecutive days exhibited electromyographic discharges consistent with profound myotonia. Fibers ...

journal_title：Journal of neuropathology and experimental neurology

authors： Ontell M,Paul HS,Adibi SA,Martin JL

更新日期：1979-11-01 00:00:00

abstract：:Most malignant human tumors display a high degree of intratumoral heterogeneity at the time of diagnosis that contributes to treatment failure. This also applies to malignant peripheral nerve sheath tumors (MPNSTs) and aggressive soft tissue sarcomas that arise sporadically or in the context of neurofibromatosis type ...

journal_title：Journal of neuropathology and experimental neurology

authors： Koelsch B,van den Berg L,Grabellus F,Fischer C,Kutritz A,Kindler-Röhrborn A

更新日期：2013-05-01 00:00:00

abstract：:Ca2+ currents are thought to enhance glutamate excitotoxicity. To investigate whether reduced expression of the Ca2+ limiting GluR2(B) subunit enhances seizure-induced vulnerability to either CA1 or CA3 neurons, we delivered GluR2(B) oligodeoxynucleotides (AS-ODNs) to the dorsal hippocampus of adult rats before induci...

journal_title：Journal of neuropathology and experimental neurology

authors： Friedman LK,Velísková J,Kaur J,Magrys BW,Liu H

更新日期：2003-07-01 00:00:00

abstract：:Axonal damage is the major cause of irreversible neurologic disability in patients with multiple sclerosis. Although axonal damage correlates with antibodies against neurofilament light (NF-L) protein, a major component of the axonal cytoskeleton, the possible pathogenic role of autoimmunity to axonal antigens such as...

journal_title：Journal of neuropathology and experimental neurology

authors： Huizinga R,Heijmans N,Schubert P,Gschmeissner S,'t Hart BA,Herrmann H,Amor S

更新日期：2007-04-01 00:00:00

abstract：:Pediatric and NF2-associated meningiomas are uncommon and poorly characterized in comparison to sporadic adult cases. In order to elucidate their molecular features, we analyzed MIB-1, progesterone receptor (PR), NF2, merlin, DAL-1, DAL-1 protein, and chromosomal arms 1p and 14q in 53 meningiomas from 40 pediatric/NF2...

journal_title：Journal of neuropathology and experimental neurology

authors： Perry A,Giannini C,Raghavan R,Scheithauer BW,Banerjee R,Margraf L,Bowers DC,Lytle RA,Newsham IF,Gutmann DH

更新日期：2001-10-01 00:00:00

abstract：:Lhermitte-Duclos disease (LDD) is a rare cerebellar tumor associated with Cowden disease (CD) and germline mutations in the PTEN gene. To further define these relationships, we reviewed clinical and pathologic findings in 31 LDD cases and analyzed the status of the PTEN pathway in 11 of them. We hypothesized that the ...

journal_title：Journal of neuropathology and experimental neurology

authors： Abel TW,Baker SJ,Fraser MM,Tihan T,Nelson JS,Yachnis AT,Bouffard JP,Mena H,Burger PC,Eberhart CG

更新日期：2005-04-01 00:00:00

abstract：:Herpes simplex virus type 1 (HSV-1) is the cause of a serious and often fatal encephalitis. Patients who survive herpes simplex encephalitis (HSE) experience behavioral abnormalities including profound cognitive dysfunctions. We have developed a rat model of acute HSE to investigate the cognitive impairments caused by...

journal_title：Journal of neuropathology and experimental neurology

authors： Beers DR,Henkel JS,Schaefer DC,Rose JW,Stroop WG

更新日期：1993-05-01 00:00:00

abstract：:To evaluate cognitive performance among persons who did and did not develop clinical Alzheimer disease (AD) but had AD neuropathology at autopsy, we examined neuropsychological performance in cognitively healthy (Clinical Dementia Rating [CDR] = 0) participants who returned for at least 1 follow-up and died within 2 y...

journal_title：Journal of neuropathology and experimental neurology

authors： Hassenstab J,Monsell SE,Mock C,Roe CM,Cairns NJ,Morris JC,Kukull W

更新日期：2015-11-01 00:00:00

abstract：:To determine if central nervous system (CNS) microvessel endothelial cells express class II major histocompatibility complex (MHC) molecules in early demyelinating lesions in humans, cerebral white matter (WM) biopsies from patients with acute inflammatory/demyelinating conditions, including 4 with multiple sclerosis ...

journal_title：Journal of neuropathology and experimental neurology

authors： van der Maesen K,Hinojoza JR,Sobel RA

更新日期：1999-04-01 00:00:00

abstract：:Since first described, amyotrophic lateral sclerosis/parkinsonism-dementia complex (ALS/PDC) of Guam has represented an important model of age-related neurodegenerative disease. ALS/PDC is characterized neuropathologically by severe widespread involvement by neurofibrillary tangles (NFTs). Over the past 30 years there...

journal_title：Journal of neuropathology and experimental neurology

authors： Perl DP,Hof PR,Purohit DP,Loerzel AJ,Kakulas BA

更新日期：2003-04-01 00:00:00

abstract：:Spirochetal infections are an important cause of neurological disease. In previous studies of the pathogenesis of spirochetal brain infection, mice inoculated with Borrelia turicatae, an agent of tick-borne relapsing fever in North America, developed mild meningitis and parenchymal activation/infiltration by interleuk...

journal_title：Journal of neuropathology and experimental neurology

authors： Londoño D,Carvajal J,Arguelles-Grande C,Marques A,Cadavid D

更新日期：2008-10-01 00:00:00

abstract：:Although stroke is among the most common causes of death and chronic disability worldwide, the proteome of the ischemic human brain remains unknown. Only a few studies have investigated the ischemic brain proteome in rodent stroke models. We performed a proteomic study of the human brain after ischemic stroke using a ...

journal_title：Journal of neuropathology and experimental neurology

authors： Cuadrado E,Rosell A,Colomé N,Hernández-Guillamon M,García-Berrocoso T,Ribo M,Alcazar A,Ortega-Aznar A,Salinas M,Canals F,Montaner J

更新日期：2010-11-01 00:00:00

abstract：:Neuropathologists use anti-glial fibrillary acidic protein (GFAP) antibodies as specific markers for glial cells, and neurobiologists use GFAP for targeting transgenes to glial cells. Since GFAP has also been detected in non-glial cells, we systematically analyzed GFAP expression in human and murine non-CNS tissues us...

journal_title：Journal of neuropathology and experimental neurology

authors： Hainfellner JA,Voigtländer T,Ströbel T,Mazal PR,Maddalena AS,Aguzzi A,Budka H

更新日期：2001-05-01 00:00:00

abstract：:Nemaline myopathy is the most common congenital myopathy and is caused by mutations in various genes such as ACTA1 (encoding skeletal α-actin). It is associated with limb and respiratory muscle weakness. Despite increasing clinical and scientific interest, the molecular and cellular events leading to such weakness rem...

journal_title：Journal of neuropathology and experimental neurology

authors： Lindqvist J,Cheng AJ,Renaud G,Hardeman EC,Ochala J

更新日期：2013-06-01 00:00:00

abstract：:Sibling concurrence of pathologically confirmed prion disease has only been reported in association with pathogenic mutation of the prion protein gene (PRNP). Here, we report 2 siblings with classic neuropathologic features of sporadic Creutzfeldt-Jakob disease unexplained by PRNP mutation or known risk factors for ia...

journal_title：Journal of neuropathology and experimental neurology

authors： Webb TE,Pal S,Siddique D,Heaney DC,Linehan JM,Wadsworth JD,Joiner S,Beck J,Wroe SJ,Stevenson V,Brandner S,Mead S,Collinge J

更新日期：2008-09-01 00:00:00

abstract：:Abnormalities were detected by two-dimensional gel electrophoresis in the protein composition of both the dorsal and ventral roots of three of six patients who succumbed to amyotrophic lateral sclerosis (ALS). The abnormalities consisted of a cascade of acidic protein spots on silver-stained gels which were shown by i...

journal_title：Journal of neuropathology and experimental neurology

authors： Brock TO,McIlwain DL

更新日期：1984-11-01 00:00:00

abstract：:Neoplastic transformation is often associated with aberrant gap junctional intercellular communication. We assessed mutations and expression of the connexin43 (Cx43) gene in 49 intracranial meningiomas. SSCP analyses followed by direct DNA sequencing showed GCG-->GTG (Ala-->Val) transition mutation in codon 253 of the...

journal_title：Journal of neuropathology and experimental neurology

authors： Sato K,Gratas C,Lampe J,Biernat W,Kleihues P,Yamasaki H,Ohgaki H

更新日期：1997-07-01 00:00:00

abstract：:The aim of our study is to evaluate the extent and distribution of grey matter demyelinating lesions in multiple sclerosis (MS), addressing also neuronal loss and synaptic loss. Whole coronal sections of 6 MS brains and 6 control brains were selected. Immunohistochemistry was performed for myelin basic protein, neurof...

journal_title：Journal of neuropathology and experimental neurology

authors： Vercellino M,Plano F,Votta B,Mutani R,Giordana MT,Cavalla P

更新日期：2005-12-01 00:00:00

abstract：:During brain development and blood-brain barrier (BBB) differentiation the expression of P-glycoprotein (P-gp) may complement the protective function of the placental barrier against xenobiotic substances. To establish an immunohistochemical procedure for P-gp detection, different anti-P-gp monoclonal antibodies were ...

journal_title：Journal of neuropathology and experimental neurology

authors： Virgintino D,Errede M,Girolamo F,Capobianco C,Robertson D,Vimercati A,Serio G,Di Benedetto A,Yonekawa Y,Frei K,Roncali L

更新日期：2008-01-01 00:00:00

abstract：:Recent studies have shown that the Alzheimer disease (AD) neocortex is characterized by a loss of large neurons, the presence of dilated terminal axons, widespread loss of synapses, and a disruption of the dendritic cytoskeleton which is manifested as Tau immunoreactive threads. In the present study we have investigat...

journal_title：Journal of neuropathology and experimental neurology

authors： Masliah E,Ellisman M,Carragher B,Mallory M,Young S,Hansen L,DeTeresa R,Terry RD

更新日期：1992-07-01 00:00:00

abstract：:Lewy body-like hyaline inclusions in the soma and swollen, cord-like cell processes are characteristic alterations of the anterior horn cells in familial amyotrophic lateral sclerosis (ALS) with posterior column and spinocerebellar tract involvement. A fine structural analysis of these two structures has been performe...

journal_title：Journal of neuropathology and experimental neurology

authors： Hirano A,Nakano I,Kurland LT,Mulder DW,Holley PW,Saccomanno G

更新日期：1984-09-01 00:00:00

abstract：:We review the newest advances related to seeking the pathogenic mechanism(s) of sporadic inclusion-body myositis (s-IBM) and present the pathologic diagnostic criteria of s-IBM. We discuss the possible pathogenic role of several themes, such as 1) increased amyloid-beta precursor protein (AbetaPP) and of its fragment ...

journal_title：Journal of neuropathology and experimental neurology

authors： Askanas V,Engel WK

更新日期：2001-01-01 00:00:00

abstract：:Mouse central nervous system tissue cultured for different lengths of time was analyzed for the proliferation of macrophages. These cells were identified and characterized by ultrastructural features, cell surface determinants and their ability to ingest latex particles and bacteria. Under the experimental conditions ...

journal_title：Journal of neuropathology and experimental neurology

authors： Raedler E,Raedler A

更新日期：1984-09-01 00:00:00

abstract：:The DNA repair protein O6-methylguanine-DNA methyltransferase (MGMT) removes temozolomide-induced alkylation, thereby preventing DNA damage and cytotoxicity. We investigated the prognostic effect of different MGMT methylation levels on overall and progression-free survival in 327 patients with primary glioblastoma und...

journal_title：Journal of neuropathology and experimental neurology

authors： Dahlrot RH,Larsen P,Boldt HB,Kreutzfeldt MS,Hansen S,Hjelmborg JB,Kristensen BW

更新日期：2019-07-01 00:00:00

abstract：:The substantia nigra is not the induction site in the brain of the neurodegenerative process underlying Parkinson disease (PD). Instead, the results of this semi-quantitative study of 30 autopsy cases with incidental Lewy body pathology indicate that PD in the brain commences with the formation of the very first immun...

journal_title：Journal of neuropathology and experimental neurology

authors： Del Tredici K,Rüb U,De Vos RA,Bohl JR,Braak H

更新日期：2002-05-01 00:00:00

abstract：:The external segment of the globus pallidus (GPe) in humans and the equivalent structure in rodents, the globus pallidus (GP), influence signal processing in the basal ganglia under normal and pathological conditions. Parvalbumin (PV) immunoreactivity defines 2 main neuronal subpopulations in the GP/GPe: PV-immunoposi...

journal_title：Journal of neuropathology and experimental neurology

authors： Fernández-Suárez D,Celorrio M,Lanciego JL,Franco R,Aymerich MS

更新日期：2012-11-01 00:00:00