Multiple sulfatase deficiency: clinical, neuropathological, ultrastructural and biochemical studies.

abstract：:The occurrence of neuroinflammation after spinal cord injury (SCI) is well established, but its function is debated, with both beneficial and detrimental consequences ascribed. A discriminate of the role of neuroinflammation may be the time period after SCI, and there is evidence to favor early neuroinflammation being...

journal_title：Journal of neuropathology and experimental neurology

authors： Rice T,Larsen J,Rivest S,Yong VW

更新日期：2007-03-01 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：: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：:Fragmentation of genomic DNA, a major biochemical feature of programmed cell death (apoptosis), is easily detected when apoptosis is prevalent. In brain tissue apoptotic cells are usually scarce and detection requires more sensitive techniques. We describe a highly sensitive method to quantify apoptosis in frozen huma...

journal_title：Journal of neuropathology and experimental neurology

authors： Olano JP,Wolf D,Keherly M,Gelman BB

更新日期：1996-11-01 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：: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：: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：:An overlapping population of adult primary sensory neurons that innervate the skin express the glial cell-derived neurotrophic factor coreceptor α1 (GFRα1), the lectin IB4, and the "regenerative brake" phosphatase and tensin homolog deleted on chromosome 10. Using an adapted turning and growth assay, we analyzed the g...

journal_title：Journal of neuropathology and experimental neurology

authors： Guo G,Singh V,Zochodne DW

更新日期：2014-09-01 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：:Tissue hypoxia and necrosis represent pathophysiologic and histologic hallmarks of glioblastoma (GBM). Although hypoxia inducible factor 1α (HIF-1α) plays crucial roles in the malignant phenotypes of GBM, developing HIF-1α-targeted agents has been hampered by the lack of a suitable preclinical model that recapitulates...

journal_title：Journal of neuropathology and experimental neurology

authors： Nigim F,Cavanaugh J,Patel AP,Curry WT Jr,Esaki S,Kasper EM,Chi AS,Louis DN,Martuza RL,Rabkin SD,Wakimoto H

更新日期：2015-07-01 00:00:00

abstract：:An experimental neuropathy in rats produced by tying loosely constrictive ligatures around one sciatic nerve has recently been shown to produce pain-related behavior that follows a reproducible time course. In the present study, we assessed the degree of thermal hyperesthesia and examined the sciatic nerves by light a...

journal_title：Journal of neuropathology and experimental neurology

authors： Sommer C,Galbraith JA,Heckman HM,Myers RR

更新日期：1993-05-01 00:00:00

abstract：:Numerous studies have established the key role of the Golgi apparatus (GA) in post-translational processing, transport and targeting of proteins destined for secretion, lysosomes and plasma membranes. Moreover, several studies performed in our laboratories have shown that the size of the immunocytochemically detected ...

journal_title：Journal of neuropathology and experimental neurology

authors： Salehi A,Ravid R,Gonatas NK,Swaab DF

更新日期：1995-09-01 00:00:00

abstract：:Rupture of a saccular intracranial aneurysm (sIA) is often fatal. Thus, early detection of rupture-prone sIAs is vital. Myeloperoxidase (MPO), derived mainly from neutrophils, associates with sIA rupture, and therefore its role in sIA pathogenesis warrants further studies. We analyzed MPO and its association with othe...

journal_title：Journal of neuropathology and experimental neurology

authors： Ollikainen E,Tulamo R,Lehti S,Hernesniemi J,Niemelä M,Kovanen PT,Frösen J

更新日期：2018-06-01 00:00:00

abstract：:Primary central nervous system lymphomas (PCNSLs) are more resistant to radiotherapy and chemotherapy in AIDS (A-PCNSLs) than in non-AIDS patients (NA-PCNSLs). We investigated 23 A-PCNSLs and 24 NA-PCNSLs. Lymphoma cell kinetics (i.e. proliferation [mitotic index, MIB-1 and PCNA labeling indices], apoptosis and turnov...

journal_title：Journal of neuropathology and experimental neurology

authors： Christov C,Adle-Biassette H,Lechapt E,Poron F,Gray F,Gaulard P,Gherardi RK

更新日期：1999-10-01 00:00:00

abstract：:Truncations of tau protein at aspartic acid421 (D421) and glutamic acid391 (E391) residues are associated with neurofibrillary tangles (NFTs) in the brains of Alzheimer disease (AD) patients. Using immunohistochemistry with antibodies to D421- and E391-truncated tau (Tau-C3 and MN423, respectively), we correlated the ...

journal_title：Journal of neuropathology and experimental neurology

authors： Basurto-Islas G,Luna-Muñoz J,Guillozet-Bongaarts AL,Binder LI,Mena R,García-Sierra F

更新日期：2008-05-01 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：:During the Zika epidemic in Brazil, a baby was born at term with microcephaly and arthrogryposis. The mother had Zika symptoms at 10 weeks of gestation. At 17 weeks, ultrasound showed cerebral malformation and ventriculomegaly. At 24 weeks, the amniotic fluid contained ZIKV RNA and at birth, placenta and maternal bloo...

journal_title：Journal of neuropathology and experimental neurology

authors： Chimelli L,Moura Pone S,Avvad-Portari E,Farias Meira Vasconcelos Z,Araújo Zin A,Prado Cunha D,Raposo Thompson N,Lopes Moreira ME,Wiley CA,da Silva Pone MV

更新日期：2018-03-01 00:00:00

abstract：:The aim of this study was to clarify pathological and anatomical relationships between adamantinomatous craniopharyngiomas (ACP) and their surrounding structures. We previously established a QST classification scheme based on the apparent anatomic origin of the tumors. According to this classification, 13 type Q tumor...

journal_title：Journal of neuropathology and experimental neurology

authors： Liu Y,Qi ST,Wang CH,Pan J,Fan J,Peng JX,Zhang X,Bao Y,Liu YW

更新日期：2018-11-01 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：:Retinal vasculopathy with cerebral leukodystrophy (RVCL) is an autosomal-dominant disorder involving the cerebral, retinal, renal, and other systemic microvessels due to frameshift mutations in the TREX1 gene. Under physiological conditions, the TREX1 protein is localized in the cellular cytoplasm and perinuclear area...

journal_title：Journal of neuropathology and experimental neurology

authors： Saito R,Nozaki H,Kato T,Toyoshima Y,Tanaka H,Tsubata Y,Morioka T,Horikawa Y,Oyanagi K,Morita T,Onodera O,Kakita A

更新日期：2019-02-01 00:00:00

abstract：:TAR DNA-binding protein 43 (TDP-43) is a major component of the inclusions in frontotemporal lobar degeneration with ubiquitinated inclusions (FTLD-U). We studied TDP-43 pathology in the hippocampus and frontal cortex of autopsy brains from patients with FTLD-U (n = 68), dementia lacking distinctive histopathology (n ...

journal_title：Journal of neuropathology and experimental neurology

authors： Hatanpaa KJ,Bigio EH,Cairns NJ,Womack KB,Weintraub S,Morris JC,Foong C,Xiao G,Hladik C,Mantanona TY,White CL 3rd

更新日期：2008-04-01 00:00:00

abstract：:Neuron loss, synaptic decline, and spongiform change are the hallmarks of sporadic Creutzfeldt-Jakob disease (sCJD), and may be related to deficiencies in mitochondria, energy metabolism, and protein synthesis. To investigate these relationships, we determined the expression levels of genes encoding subunits of the 5 ...

journal_title：Journal of neuropathology and experimental neurology

authors： Ansoleaga B,Garcia-Esparcia P,Llorens F,Hernández-Ortega K,Carmona Tech M,Antonio Del Rio J,Zerr I,Ferrer I

更新日期：2016-08-01 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：:Large complex membranous bodies are described in the cerebral cortex of adult rats. Although these bodies are not very prevalent, they appear with increasing frequency as the age of the animal increases from 12 to 28 months. The membranous bodies are composed of varying amounts of membranes, cytoplasm and empty vacuol...

journal_title：Journal of neuropathology and experimental neurology

authors： Vaughan DW

更新日期：1976-03-01 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：:Myxopapillary ependymoma (MPE) is a relatively common neoplasm arising primarily in the filum terminale/lumbosacral region of the spinal cord. It is designated as a grade I tumor in the most recent WHO Classification of Tumours of the CNS, although aggressive clinical behavior can be observed, especially in cases aris...

journal_title：Journal of neuropathology and experimental neurology

authors： Gitto L,Serinelli S,Galbraith K,Williams M,Mirchia K,Galgano MA,Krishnamurthy S,de la Roza G,Viapiano MS,Walker JM,Jour G,Serrano J,DeLorenzo M,Snuderl M,Richardson TE

更新日期：2020-10-01 00:00:00

abstract：:This is the fourth recorded patient with extension of syringomyelia into the brain rostral to the mesencephalon verified at autopsy. A syrinx was demonstrated from the low thoracic segments of the spinal cord to the cervico-medullary junction, where a fibrovascular malformation and dural-arachnoid adhesions deformed t...

journal_title：Journal of neuropathology and experimental neurology

authors： Berry RG,Chambers RA,Lublin FD

更新日期：1981-11-01 00:00:00

abstract：:Lymphocytic choriomeningitis virus-induced central nervous system disease is characterized by death during a seizure approximately seven days after intracerebral inoculation. This process is mediated by thymus dependent lymphocytes, sensitized against viral antigens. Various forms of immunosuppressive treatment preven...

journal_title：Journal of neuropathology and experimental neurology

authors： Camenga DL,Walker DH,Murphy FA

更新日期：1977-01-01 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：:The genetic abnormality most frequently identified in glioblastomas is loss of alleles on chromosome 10. We have performed a comprehensive study of the PTEN tumor suppressor gene on 10q23, including loss of heterozygosity (LOH) analysis, multiplex PCR, mutation analysis, and reverse transcription PCR (RT-PCR). In tota...

journal_title：Journal of neuropathology and experimental neurology

authors： Schmidt EE,Ichimura K,Goike HM,Moshref A,Liu L,Collins VP

更新日期：1999-11-01 00:00:00