Abnormalities in neural progenitor cells in a dog model of lysosomal storage disease.

abstract：:The balance between excitatory and inhibitory synaptic inputs is critical for the physiological control of motoneurons. The maintenance of a low-intracellular chloride concentration by the potassium chloride cotransporter 2 (KCC2) is essential for the efficacy of fast synaptic inhibition of mature motoneurons in respo...

journal_title：Journal of neuropathology and experimental neurology

authors： Fuchs A,Ringer C,Bilkei-Gorzo A,Weihe E,Roeper J,Schütz B

更新日期：2010-10-01 00:00:00

abstract：:The role of regenerating axons in the restructuring of the endoneurium into minifascicles after peripheral nerve injury was investigated. Endoneurial changes were studied in the control distal stumps of crushed rat sciatic nerves and in the distal nerve stumps in which, in addition to other cellular elements, the orig...

journal_title：Journal of neuropathology and experimental neurology

authors： Popović M,Bresjanac M,Sketelj J

更新日期：1994-11-01 00:00:00

abstract：:The main hallmarks of human hippocampal sclerosis are neuronal loss and gliosis; reductions in microvasculature labeling in the cornu Ammonis 1 in this condition have been detected using alkaline phosphatase histochemistry. To determine whether the reduction in alkaline phosphatase activity is coupled with a loss of b...

journal_title：Journal of neuropathology and experimental neurology

authors： Kastanauskaite A,Alonso-Nanclares L,Blazquez-Llorca L,Pastor J,Sola RG,DeFelipe J

更新日期：2009-08-01 00:00:00

abstract：:This is a report of an unusual, densely cellular, midcerebellar neoplasm in a seven-year-old boy. Although clinically consistent with a medulloblastoma, immunohistochemistry and electron microscopy demonstrated glial and rhabdomyoblastic differentiation in the tumor. We discuss the differential diagnosis of this tumor...

journal_title：Journal of neuropathology and experimental neurology

authors： Dickson DW,Hart MN,Menezes A,Cancilla PA

更新日期：1983-11-01 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：: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 Niemann-Pick Type C (NPC) disease, the concentration of cholesterol increases with age in every tissue except the brain. This study investigates whether accumulation of cholesterol might also occur within the cells of the central nervous system (CNS), but be obscured by the simultaneous loss of sterol from myelin a...

journal_title：Journal of neuropathology and experimental neurology

authors： Xie C,Burns DK,Turley SD,Dietschy JM

更新日期：2000-12-01 00:00:00

abstract：:Sequential immunocytochemical double labeling of glial fibrillary acidic protein (GFAP) and transferrin (Tf) was used for the simultaneous identification of astrocytes and oligodendrocytes in paraffin-embedded sections of the rat brain. Paraformaldehyde or formalin-alcohol-acetic acid fixation followed by protease tre...

journal_title：Journal of neuropathology and experimental neurology

authors： Martin SM,Landel HB,Lansing AJ,Vijayan VK

更新日期：1991-03-01 00:00:00

abstract：:Paramyxoviruses such as measles virus or canine distemper virus are etiological agents for acute and chronic encephalitis (measles inclusion body encephalitis, subacute sclerosing panencephalitis and chronic distemper encephalitis or old dog encephalitis). The mechanisms by which viral injury leads to neurological dis...

journal_title：Journal of neuropathology and experimental neurology

authors： Bernard A,Fevre-Montange M,Bencsik A,Giraudon P,Wild TF,Confavreux C,Belin MF

更新日期：1993-09-01 00:00:00

abstract：:Oligodendrogliomas are characterized by frequent loss of heterozygosity (LOH) on chromosomes 1p and 19q, but additional genetic alterations are likely to be involved. In this study, we screened 28 oligodendrogliomas (WHO grade II) and 20 anaplastic oligodendrogliomas (WHO grade III) for alterations in the RB1/CDK4/p16...

journal_title：Journal of neuropathology and experimental neurology

authors： Watanabe T,Yokoo H,Yokoo M,Yonekawa Y,Kleihues P,Ohgaki H

更新日期：2001-12-01 00:00:00

abstract：:Increased numbers of corpora amylacea have been observed in the resected mesial temporal lobe of many patients with complex partial seizures (CPS) and Ammon's horn sclerosis (AHS). Several heat shock proteins (HSPs) are induced by seizures and have been suggested as an etiologic factor in the formation corpora amylace...

journal_title：Journal of neuropathology and experimental neurology

authors： Erdamar S,Zhu ZQ,Hamilton WJ,Armstrong DL,Grossman RG

更新日期：2000-08-01 00:00:00

abstract：:Kii amyotrophic lateral sclerosis/parkinsonism-dementia complex (ALS/PDC) is a progressive neurodegenerative disorder that is endemic to the Kii peninsula of Japan. The disorder is clinically characterized by a variable combination of parkinsonism, dementia, and motor neuron symptoms. Despite extensive investigations,...

journal_title：Journal of neuropathology and experimental neurology

authors： Verheijen BM,Morimoto S,Sasaki R,Oyanagi K,Kokubo Y,Kuzuhara S,van Leeuwen FW

更新日期：2020-08-01 00:00:00

abstract：:In 63 patients with malabsorption syndromes, 16 with congenital biliary atresia (BA) and 47 with cystic fibrosis (CF), axonal dystrophy in the gracile nucleus (ADG) was studied. Of the 16 patients with BA, ADG of considerable severity was observed in all 10 over one year of age. Of the 47 patients with CF, it was obse...

journal_title：Journal of neuropathology and experimental neurology

authors： Sung JH,Park SH,Mastri AR,Warwick WJ

更新日期：1980-09-01 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：:The role of axonal transport in the pathogenesis of the axonal swellings which develop at the severed ends of transected axons was studied by electron microscopic (EM) autoradiography. Proteins carried by fast anterograde transport in rat sciatic nerves were labeled with [3H]-leucine or [3H]-fucose; [3H]-leucine, [3H]...

journal_title：Journal of neuropathology and experimental neurology

authors： Griffin JW,Price DL,Engel WK,Drachman DB

更新日期：1977-03-01 00:00:00

abstract：:A recent analysis of a group of patients with central pontine myelinolysis (CPM) disclosed that a rapid rise in serum sodium from a hyponatremic baseline preceded the clinical onset of the disorder. To test the view that electrolyte derangements may be crucial in the pathogenesis of CPM, rats were given hypertonic sal...

journal_title：Journal of neuropathology and experimental neurology

authors： Kleinschmidt-DeMasters BK,Norenberg MD

更新日期：1982-01-01 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 ascending reticular activating system (ARAS) mediates arousal, an essential component of human consciousness. Lesions of the ARAS cause coma, the most severe disorder of consciousness. Because of current methodological limitations, including of postmortem tissue analysis, the neuroanatomic connectivity of the huma...

journal_title：Journal of neuropathology and experimental neurology

authors： Edlow BL,Takahashi E,Wu O,Benner T,Dai G,Bu L,Grant PE,Greer DM,Greenberg SM,Kinney HC,Folkerth RD

更新日期：2012-06-01 00:00:00

abstract：:Brainstem Lewy bodies (LB) are neuronal inclusions that are closely related to Parkinson's disease (PD). The filamentous component of LB from patients with PD contains biochemically altered neurofilaments (NF). Herein we have tested the hypothesis that the oxidized products of catechols may covalently crosslink NF. Ne...

journal_title：Journal of neuropathology and experimental neurology

authors： Montine TJ,Farris DB,Graham DG

更新日期：1995-05-01 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：:Pilocytic astrocytoma (PA) is the most common glioma of childhood. Despite their relatively high incidence, the molecular mechanisms responsible for tumorigenesis and growth of PA are poorly understood. Previous in vitro studies in our laboratory showed that despite the absence of ErbB1, PA was sensitive to ErbB1 tyro...

journal_title：Journal of neuropathology and experimental neurology

authors： Addo-Yobo SO,Straessle J,Anwar A,Donson AM,Kleinschmidt-Demasters BK,Foreman NK

更新日期：2006-08-01 00:00:00

abstract：:Early inflammatory events may inhibit functional recovery after injury in both the peripheral and central nervous systems. We investigated the role of the inflammatory tumor necrosis factor/nuclear factor-kappaB (NF-kappaB) axis on events subsequent to sciatic nerve crush injury in adult rats. Electrophoretic mobility...

journal_title：Journal of neuropathology and experimental neurology

authors： Smith D,Tweed C,Fernyhough P,Glazner GW

更新日期：2009-06-01 00:00:00

abstract：:Spinal muscular atrophy (SMA) is characterized by loss of motor neurons in the spinal cord that results in muscle denervation and profound weakness in affected patients. We sought evidence for primary muscle involvement in the disease during human development by analyzing the expression of several muscle cytoskeletal ...

journal_title：Journal of neuropathology and experimental neurology

authors： Martínez-Hernández R,Bernal S,Alias L,Tizzano EF

更新日期：2014-06-01 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：:Smooth muscle cells cultured from leptomeningeal vessels from old dogs with amyloid-angiopathy accumulate intracellular deposits that are immunoreactive for amyloid-beta peptide (A beta). We used this cellular model in the present study to examine the influence of sera and cerebrospinal fluid on intracellular accumula...

journal_title：Journal of neuropathology and experimental neurology

authors： Mazur-Kolecka B,Frackowiak J,Carroll RT,Wisniewski HM

更新日期：1997-03-01 00:00:00

abstract：:How to assess the substantial neuronal loss in a neurodegenerative disease such as Alzheimer disease is still being debated. Recently, stereological procedures have been proposed that claim improved accuracy and statistical power, but the results of some of these investigations have been controversial. In this study w...

journal_title：Journal of neuropathology and experimental neurology

authors： Everall IP,DeTeresa R,Terry R,Masliah E

更新日期：1997-11-01 00:00:00

abstract：:The content of RNA and volume of individual neurons isolated from the nucleus dorsalis and from the ventrolateral portion of the lumbar swelling were determined in eight cases of amyotrophic lateral sclerosis (ALS) and eight controls whose spinal cords were obtained at autopsy. The mean content of RNA in the lumbar mo...

journal_title：Journal of neuropathology and experimental neurology

authors： Davidson TJ,Hartmann HA

更新日期：1981-03-01 00:00:00

abstract：:Alpha-synuclein (alpha-syn) is an important component of neuronal and glial inclusions in brains of patients with several neurodegenerative disorders. Sporadic inclusion-body myositis (s-IBM) is the most common progressive muscle disease of older patients. Its muscle phenotype shows several similarities with Alzheimer...

journal_title：Journal of neuropathology and experimental neurology

authors： Askanas V,Engel WK,Alvarez RB,McFerrin J,Broccolini A

更新日期：2000-07-01 00:00:00

abstract：:Chorea-Acanthocytosis (CHAC) is an autosomal recessive disease characterized by neurodegeneration and acanthocytosis. Enhanced creatine kinase concentration is a constant feature of the condition. The mechanism underlying CHAC is unknown. However, acanthocytosis and enhanced creatine kinase suggest a protein defect th...

journal_title：Journal of neuropathology and experimental neurology

authors： Melone MA,Di Fede G,Peluso G,Lus G,Di Iorio G,Sampaolo S,Capasso A,Gentile V,Cotrufo R

更新日期：2002-10-01 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