Structural and Functional Abnormalities of the Neuromuscular Junction in the Trembler-J Homozygote Mouse Model of Congenital Hypomyelinating Neuropathy.

abstract：:The endoneurial lead and water content was serially evaluated in the nerves of rats fed lead carbonate and related to the onset and severity of segmental demyelination and remyelination. Lead began to accumulate significantly in the endoneurium by 5 days, reached a maximum level (71 microgram/g dry weight) by 34 days,...

journal_title：Journal of neuropathology and experimental neurology

authors： Windebank AJ,McCall JT,Hunder HG,Dyck PJ

更新日期：1980-11-01 00:00:00

abstract：:The roles of bone morphogenetic proteins (BMPs) and their receptors (BMPRs) in meningioma biology are not known. In this study, frozen tissues from 26 World Health Organization Grades I to III meningiomas were analyzed by Western blot for BMP-2/4, BMPR IA, and BMPR II, and activation of downstream p-Smad1, p38 mitogen...

journal_title：Journal of neuropathology and experimental neurology

authors： Johnson MD,O'Connell MJ,Vito F,Pilcher W

更新日期：2009-11-01 00:00:00

abstract：:Hydrocephalic hyh mice are born with moderate hydrocephalus and a normal cerebral aqueduct. At about the fifth postnatal day the aqueduct becomes obliterated and severe hydrocephalus develops. The aim of the present investigation was to investigate the mechanism of this hydrocephalus, probably starting during fetal li...

journal_title：Journal of neuropathology and experimental neurology

authors： Jiménez AJ,Tomé M,Páez P,Wagner C,Rodríguez S,Fernández-Llebrez P,Rodríguez EM,Pérez-Fígares JM

更新日期：2001-11-01 00:00:00

abstract：:The most common histologic feature in patients with frontotemporal lobar degeneration (FTLD) is intracellular brain inclusions of yet uncharacterized proteins that react with antiubiquitin (Ub) antibodies, but not with tau or synuclein (FTLD-U). We identified a four-generation Belgian FTLD family in which 8 patients h...

journal_title：Journal of neuropathology and experimental neurology

authors： Pirici D,Vandenberghe R,Rademakers R,Dermaut B,Cruts M,Vennekens K,Cuijt I,Lübke U,Ceuterick C,Martin JJ,Van Broeckhoven C,Kumar-Singh S

更新日期：2006-03-01 00:00:00

abstract：:Brain trauma in humans increases the risk for developing Alzheimer disease (AD) and may induce the acute formation of AD-like plaques containing amyloid beta (A beta). To further explore the potential link between brain trauma and neurodegeneration, we conducted neuropathological studies using a pig model of diffuse b...

journal_title：Journal of neuropathology and experimental neurology

authors： Smith DH,Chen XH,Nonaka M,Trojanowski JQ,Lee VM,Saatman KE,Leoni MJ,Xu BN,Wolf JA,Meaney DF

更新日期：1999-09-01 00:00:00

abstract：:This morphometric study explores temporal and topographic changes in the estimated neuronal number in human neocortex during the latter half of gestation and early infancy. Neuronal estimates are calculated from standardized measurements of cortical layer thickness and neuronal density in 6 neocortical regions in 9 hu...

journal_title：Journal of neuropathology and experimental neurology

authors： Rabinowicz T,de Courten-Myers GM,Petetot JM,Xi G,de los Reyes E

更新日期：1996-03-01 00:00:00

abstract：:We have previously studied in nonhuman primates several aspects of the acute rejection of myofibers, including the histological characteristics, the mechanisms of myofiber elimination by the T cells, and the development of anti-donor antibodies. Here, we report the participation of the complement membrane attack compl...

journal_title：Journal of neuropathology and experimental neurology

authors： Skuk D,Tremblay JP

更新日期：2019-01-01 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：:A primary cerebellar rhabdomyosarcoma (RMS) in a six and a half year old boy is reported. Microscopy of the surgical material revealed lobules of closely packed cells with a high mitotic rate, pleomorphic hyperchromatic nuclei and scant cytoplasm. At their periphery, the lobules merged with rounded cells with similar ...

journal_title：Journal of neuropathology and experimental neurology

authors： Hinton DR,Halliday WC

更新日期：1984-07-01 00:00:00

abstract：:Protein aggregate myopathies, including myofibrillar myopathies and sporadic inclusion body myositis (sIBM), are characterized by abnormal protein aggregates composed of various muscular and ectopic proteins. Previous studies have shown the crucial role ofdysregulated transcription factors such as neuron-restrictive s...

journal_title：Journal of neuropathology and experimental neurology

authors： Olivé M,Janué A,Moreno D,Gámez J,Torrejón-Escribano B,Ferrer I

更新日期：2009-03-01 00:00:00

abstract：:The kinin B1 receptor is an inducible receptor expressed in response to inflammatory mediators. We sought to determine whether kinin B1 receptor can be expressed on human brain endothelial cells (HBECs) in vitro and whether signaling via this receptor can regulate permeability and chemokine production properties of th...

journal_title：Journal of neuropathology and experimental neurology

authors： Prat A,Biernacki K,Pouly S,Nalbantoglu J,Couture R,Antel JP

更新日期：2000-10-01 00:00:00

abstract：:The role of anti-myelin antibodies in the pathogenesis of experimental autoimmune neuritis (EAN) induced in the Lewis rat by immunization with peripheral nerve myelin has been assessed. Passive transfer with lymph node cells (LNC) or purified serum immunoglobulin from rats with EAN was employed to directly measure the...

journal_title：Journal of neuropathology and experimental neurology

authors： Taylor JM,Pollard JD

更新日期：2001-06-01 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：:The triplication of the DYRK1A gene encoding proline-directed serine/threonine kinase and located in the critical region of Down syndrome (DS) has been implicated in cognitive deficits and intellectual disability of individuals with DS. We investigated the effect of abnormal levels of this kinase on the cytoskeleton i...

journal_title：Journal of neuropathology and experimental neurology

authors： Dowjat K,Adayev T,Kaczmarski W,Wegiel J,Hwang YW

更新日期：2012-12-01 00:00:00

abstract：:A superoxide dismutase 1 (SOD-1)genetic defect has been identified in familial amyotrophic lateral sclerosis (ALS) and motor neuron degeneration has been described in SOD-1 transgenic mice. Because an excitotoxic mechanism has been implicated in ALS, we undertook studies to provide a description of excitotoxic degener...

journal_title：Journal of neuropathology and experimental neurology

authors： Ikonomidou C,Qin Qin Y,Labruyere J,Olney JW

更新日期：1996-02-01 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：:Due to recent biological and technical advances, the list of potentially useful candidate genes is rapidly expanding in the study of brain tumors. However, traditional methods of screening individual genes in individual samples are slow and tedious, often with consumption of precious resources after only a few experim...

journal_title：Journal of neuropathology and experimental neurology

authors： Fuller CE,Wang H,Zhang W,Fuller GN,Perry A

更新日期：2002-12-01 00:00:00

abstract：:Recent studies have shown that deficient functioning of glutamate transporters (GTs) in Alzheimer disease (AD) might lead to neurodegeneration. The main objectives of the present study were to determine which GT subtype is most affected in AD and to asses to what extent altered GT function is associated with abnormal ...

journal_title：Journal of neuropathology and experimental neurology

authors： Li S,Mallory M,Alford M,Tanaka S,Masliah E

更新日期：1997-08-01 00:00:00

abstract：:Glial lipopigment appears in the globus pallidus without accumulating in neurons (except for late adolescence) in multiple chronic childhood diseases. In this observational study, we compared the age-related development of glial pigmentation in children with the chronic illness (cystic fibrosis) and children dying acu...

journal_title：Journal of neuropathology and experimental neurology

authors： Gilles FH,Tavaré CJ

更新日期：2002-04-01 00:00:00

abstract：:The brainstem and cervical spinal cord were sampled from 45 cases of sudden infant death syndrome (SIDS), from 17 control cases without neurological disease, and from three negative control cases with abnormal growth of the central nervous system (36-98 postconceptional weeks). Morphometric analyses were performed on ...

journal_title：Journal of neuropathology and experimental neurology

authors： O'Kusky JR,Kozuki DE,Norman MG

更新日期：1995-07-01 00:00:00

abstract：:Canine smooth muscle cells (SMCs), cultured from amyloid-affected brain blood vessels accumulate Alzheimer amyloid-beta peptide (Abeta) intracellularly, either spontaneously or after treatment with apolipoprotein E (apoE). ApoE is codeposited with Abeta, which suggests that apoE participates in Abeta accumulation. We ...

journal_title：Journal of neuropathology and experimental neurology

authors： Mazur-Kolecka B,Frackowiak J,Krzeslowska J,Ramakrishna N,Haske T,Emmerling MR,Zhang W,Kim KS,Wisniewski HM

更新日期：1999-03-01 00:00:00

abstract：:CD44 is a glycoprotein present on the surface of some lymphocyte cell populations and other non-lymphoid cells, and is involved in many functions related to cell-cell and cell-matrix interactions. In this study, expression of CD44 antigen in primary neural cell cultures derived from fetal and adult human brains was in...

journal_title：Journal of neuropathology and experimental neurology

authors： Moretto G,Xu RY,Kim SU

更新日期：1993-07-01 00:00:00

abstract：:A retrospective analysis of 14 patients who presented with a progressively expanding mass lesion(s) shown at biopsy/autopsy to represent acute demyelination was carried out. The aims of this study were to determine the optimal neuropathological approach to diagnosis and to determine the clinical evolution of this cond...

journal_title：Journal of neuropathology and experimental neurology

authors： Annesley-Williams D,Farrell MA,Staunton H,Brett FM

更新日期：2000-06-01 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：:Microglial activation and behavioral abnormalities occur before neuronal loss in experimental murine prion disease; the behavioral changes coincide with a reduction in synaptic plasticity. Because synaptic plasticity depends on an intact perineuronal net (PN), a specialized extracellular matrix that surrounds parvalbu...

journal_title：Journal of neuropathology and experimental neurology

authors： Franklin SL,Love S,Greene JR,Betmouni S

更新日期：2008-03-01 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 understand the basis of oligodendrocyte (OL) susceptibility to oxidative injury, purified rat OL cultures at different stages of maturation were exposed to nitric oxide (NO) donors with fast or slow kinetics of release and to tert-butyl-hydroperoxide, a membrane-permeant organic hydroperoxide. OL precursors (pre-OL...

journal_title：Journal of neuropathology and experimental neurology

authors： Bernardo A,Greco A,Levi G,Minghetti L

更新日期：2003-05-01 00:00:00

abstract：:The evolving neuropathology of primarily undamaged cortical regions adjacent to the injured site has been studied in 36 infants who survived a variety of perinatally acquired encephalopathies (microgyrias, ulegyrias, multicystic encephalopathies, porencephalies, and hydranencephalies) and later died of unrelated cause...

journal_title：Journal of neuropathology and experimental neurology

authors： Marín-Padilla M

更新日期：1999-05-01 00:00:00

abstract：:We have encountered a series of 8 third ventricular neoplasms with a distinctive chordoid appearance that appear to represent a clinicopathologic entity. The tumors occurred in 7 females and 1 male, ranging in age from 31 to 70 years. In all cases, imaging studies showed a large well-circumscribed third ventricular ma...

journal_title：Journal of neuropathology and experimental neurology

authors： Brat DJ,Scheithauer BW,Staugaitis SM,Cortez SC,Brecher K,Burger PC

更新日期：1998-03-01 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