Mice deficient in all forms of lysosomal beta-hexosaminidase show mucopolysaccharidosis-like pathology.

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：:Xeroderma pigmentosum group A (XPA) and Cockayne syndrome (CS) are hereditary DNA repair disorders complicated by progressive neurodegeneration. Here we immunohistochemically examine the in situ expression of materials that are produced by oxidative stress and glutamate transporters (which can contribute to prevention...

journal_title：Journal of neuropathology and experimental neurology

authors： Hayashi M,Itoh M,Araki S,Kumada S,Shioda K,Tamagawa K,Mizutani T,Morimatsu Y,Minagawa M,Oda M

更新日期：2001-04-01 00:00:00

abstract：:Interferon-stimulated gene 15 (ISG15), an antagonist of the ubiquitin pathway, is elevated in cells and brain tissues obtained from ataxia telangiectasia (A-T) patients. Previous studies reveal that an elevated ISG15 pathway inhibits ubiquitin-dependent protein degradation, leading to activation of basal autophagy as ...

journal_title：Journal of neuropathology and experimental neurology

authors： Kim CD,Reed RE,Juncker MA,Fang Z,Desai SD

更新日期：2017-07-01 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：: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：:The relevance of cerebral amyloid angiopathy (CAA) to the pathogenesis of Alzheimer disease (AD) and dementia in general emphasizes the importance of developing novel targeting approaches for detecting and treating cerebrovascular amyloid (CVA) deposits. We developed a nanoparticle-based technology that uses a monoclo...

journal_title：Journal of neuropathology and experimental neurology

authors： Poduslo JF,Hultman KL,Curran GL,Preboske GM,Chamberlain R,Marjańska M,Garwood M,Jack CR Jr,Wengenack TM

更新日期：2011-08-01 00:00:00

abstract：:Peripheral polyneuropathies are common and their diagnosis may be challenging. We compared the results from sural-nerve and skin biopsies in 33 patients with a polyneuropathy and neuropathic pain examined in our hospital over a 6-year period. The biopsies were all from the same lower limb of each patient. Intraepiderm...

journal_title：Journal of neuropathology and experimental neurology

authors： Duchesne M,Magy L,Richard L,Ingrand P,Neau JP,Mathis S,Vallat JM

更新日期：2016-01-01 00:00:00

abstract：:Neurofibrillary tangles (NFTs) represent products of insoluble tau protein in the brains of patients with Alzheimer disease (AD). The cerebrospinal fluid (CSF) tau level is a biomarker in AD diagnosis. The soluble portion of tau protein in brain parenchyma is presumably the source for CSF tau but this has not previous...

journal_title：Journal of neuropathology and experimental neurology

authors： Han P,Serrano G,Beach TG,Caselli RJ,Yin J,Zhuang N,Shi J

更新日期：2017-01-01 00:00:00

abstract：:Immunohistochemical identification of myelin basic protein (MBP) is a sensitive method for assessing myelination in the human fetal central nervous system (CNS). However, the temporospatial relationship of expression of two other major myelin proteins, proteolipid protein (PLP) and myelin-associated glycoprotein (MAG)...

journal_title：Journal of neuropathology and experimental neurology

authors： Weidenheim KM,Bodhireddy SR,Rashbaum WK,Lyman WD

更新日期：1996-06-01 00:00:00

abstract：:In adult cerebral astrocytomas, the more anaplasia that is present, the more malignancy that occurs. Cell proliferation antigen staining (MIB-1) and DNA labeling methods for apoptosis using paraffin sections from 39 cases were compared with histopathological grading systems for predicting patient survival. Cases were ...

journal_title：Journal of neuropathology and experimental neurology

authors： Rhodes RH

更新日期：1998-08-01 00:00:00

abstract：:The concept of the presence of mucopolysaccharides forming an interstitial ground substance in central nervous tissues is revived. The biochemical reports of both neutral and acid mucopolysaccharides in both gray and white matter of the normal brain are reviewed. The earlier histochemical reports and the histochemical...

journal_title：Journal of neuropathology and experimental neurology

authors： Feigin I

更新日期：1980-01-01 00:00:00

abstract：:Fibrosis is the main cause of irreversible nerve damage in leprosy. Phenotypic changes in Mycobacterium leprae (ML)-infected Schwann cells (SCs) have been suggested to mediate this process. We found that SC line cultures stimulated with ML upregulated transforming growth factor-β1 (TGF-β1), and that TGF-β1 or ML induc...

journal_title：Journal of neuropathology and experimental neurology

authors： Petito RB,Amadeu TP,Pascarelli BM,Jardim MR,Vital RT,Antunes SL,Sarno EN

更新日期：2013-04-01 00:00:00

abstract：:Mutations in the parkin gene are the most common cause of early-onset autosomal recessive Parkinson disease (PD). The pathogenic mechanisms of how parkin mutations lead to the development of PD are not fully understood. Studies of cell cultures and of Drosophila have suggested a dominant negative effect for the clinic...

journal_title：Journal of neuropathology and experimental neurology

authors： Van Rompuy AS,Lobbestael E,Van der Perren A,Van den Haute C,Baekelandt V

更新日期：2014-02-01 00:00:00

abstract：:Cortical lesions (CLs) are an important component of multiple sclerosis (MS) pathology; they correlate better with physical disability and cognitive impairment than white matter lesions (WMLs). Because remyelination can be extensive in CLs, we quantified remyelination in gray matter (GM) and white matter (WM), address...

journal_title：Journal of neuropathology and experimental neurology

authors： Strijbis EMM,Kooi EJ,van der Valk P,Geurts JJG

更新日期：2017-05-01 00:00:00

abstract：:We report a novel case of a World Health Organization grade 3 anaplastic meningioma arising from the olfactory groove in an 83-year-old woman. Molecular and methylation profiling confirm this lesion to be an NF2 subtype, methylation class intermediate type B meningioma. As most meningiomas in this location are indolen...

journal_title：Journal of neuropathology and experimental neurology

authors： Foo ASC,Tan DTM,Tan CL,Sahm F,von Deimling A,Yeo TT

更新日期：2017-12-01 00:00:00

abstract：:Rates of remyelination decline with age and this has been attributed to slower recruitment of oligodendrocyte progenitor cells (OPCs) into areas of demyelination and slower differentiation of OPCs into remyelinating oligodendrocytes. When considering causes for reduced recruitment rates, intrinsic causes (alterations ...

journal_title：Journal of neuropathology and experimental neurology

authors： Chari DM,Crang AJ,Blakemore WF

更新日期：2003-09-01 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 favorable response of oligodendrogliomas correlates well with characteristic chromosomal losses, of which loss of the short arm of chromosome 1 is most predictive. Oligodendrogliomas are histopathologically heterogeneous tumors and, in addition to the classic honeycomb histology, fields of nonclassic histology are...

journal_title：Journal of neuropathology and experimental neurology

authors： Kros JM,van der Weiden M,Zheng PP,Hop WC,van den Bent MJ,Kouwenhoven MC

更新日期：2007-12-01 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 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：:This investigation tested the hypothesis that the growth inhibiting effects of human beta-interferon on cultured human glioma cells involves changes in the ganglioside composition of these cells. Four cell lines derived from human malignant gliomas (12-18, U-251 MG, I29-A, 7-24) and two lines from human fetal brain (C...

journal_title：Journal of neuropathology and experimental neurology

authors： Yates AJ,Markowitz DL,Stephens RE,Pearl DK,Whisler RL

更新日期：1988-03-01 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：: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：:To test the hypothesis that Alzheimer disease (AD) is a clinical and pathologic continuum between normal aging and end-stage dementia, we selected a convenience sample of subjects from the National Alzheimer Coordinating Center 2005 to 2012 autopsy cohort (n = 2,083) with the last clinical evaluation within 2 years be...

journal_title：Journal of neuropathology and experimental neurology

authors： Serrano-Pozo A,Qian J,Monsell SE,Frosch MP,Betensky RA,Hyman BT

更新日期：2013-12-01 00:00:00

abstract：:The age-related change in the neuronal RNA content, volume, and the RNA concentration of 2,160 single cell bodies was examined from the prefrontal cortex. Human brains from 15 normal and 3 demented patients of ages ranging from 8 months to 94 years were obtained at post-mortem examination. The neuronal RNA showed an a...

journal_title：Journal of neuropathology and experimental neurology

authors： Uemura E,Hartmann HA

更新日期：1978-09-01 00:00:00

abstract：:Progressive multifocal leukoencephalopathy (PML) occurs most often in immunosuppressed individuals. The lesions of PML result from astrocyte and oligodendrocyte infection by the polyomavirus JC (JCV); JCV has also been shown to infect and destroy cerebellar granule cell neurons (GCNs) in 2 human immunodeficiency virus...

journal_title：Journal of neuropathology and experimental neurology

authors： Wüthrich C,Cheng YM,Joseph JT,Kesari S,Beckwith C,Stopa E,Bell JE,Koralnik IJ

更新日期：2009-01-01 00:00:00

abstract：:Deletions on the short arm of chromosome 1 (1p) and the long arm of chromosome 19 (19q) are molecular hallmark lesions of oligodendroglial tumors. Ever since oligodendroglial tumors with 1p and 19q deletions were shown to respond to chemotherapy, neuropathologists have been facing increasing requests for such molecula...

journal_title：Journal of neuropathology and experimental neurology

authors： Hartmann C,Mueller W,Lass U,Kamel-Reid S,von Deimling A

更新日期：2005-01-01 00:00:00

abstract：:In the present study, we investigated the expression of protease-activated receptors (PARs), receptors for thrombin, in substantia nigra pars compacta (SNpc) of Parkinson disease (PD) brains and cultures of human neurons, astrocytes, oligodendrocytes, and microglia as determined by immunocytochemistry and reverse tran...

journal_title：Journal of neuropathology and experimental neurology

authors： Ishida Y,Nagai A,Kobayashi S,Kim SU

更新日期：2006-01-01 00:00:00

abstract：:Reactive astrogliosis is associated with many pathologic processes in the central nervous system, including gliomas. The glycoprotein podoplanin (PDPN) is upregulated in malignant gliomas. Using a syngeneic intracranial glioma mouse model, we show that PDPN is highly expressed in a subset of glial fibrillary acidic pr...

journal_title：Journal of neuropathology and experimental neurology

authors： Kolar K,Freitas-Andrade M,Bechberger JF,Krishnan H,Goldberg GS,Naus CC,Sin WC

更新日期：2015-01-01 00:00:00

abstract：:Mutations in the leucine-rich repeat kinase 2 gene (LRRK2) have been recently identified in families with autosomal-dominant late-onset Parkinson disease. We report that by reverse transcriptase-polymerase chain reaction, the mRNA of LRRK2 is expressed in soluble extracts of human brain, liver, and heart and in cultur...

journal_title：Journal of neuropathology and experimental neurology

authors： Miklossy J,Arai T,Guo JP,Klegeris A,Yu S,McGeer EG,McGeer PL

更新日期：2006-10-01 00:00:00