Human mucopolysaccharidosis IIID: clinical, biochemical, morphological and immunohistochemical characteristics.

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：:Cutaneous and leptomeningeal vascular malformations are hallmarks of the Sturge-Weber Syndrome (SWS), resulting in chronic ischemic tissue damage. The mechanisms underlying the pathobiology of these progressive lesions are unknown. Aberrant expression of angiogenic factors has been implicated in the genesis and mainte...

journal_title：Journal of neuropathology and experimental neurology

authors： Comati A,Beck H,Halliday W,Snipes GJ,Plate KH,Acker T

更新日期：2007-01-01 00:00:00

abstract：:Wallerian degeneration of the CNS is accompanied by axonal dystrophy or swelling. To understand the mechanisms by which swellings arise, we studied their spatiotemporal dynamics, ultrastructure, composition, and the conditions that affect their formation in vivo and ex vivo. In contrast to peripheral nerve axons, lesi...

journal_title：Journal of neuropathology and experimental neurology

authors： Beirowski B,Nógrádi A,Babetto E,Garcia-Alias G,Coleman MP

更新日期：2010-05-01 00:00:00

abstract：:The presence of intermediate filaments (IF) (diameter 10 nm) is a characteristic electron microscopic finding in the cytoplasm of meningioma cells. To identify these IF, immunohistochemical staining for cytokeratins and vimentin and two-dimensional (2-D) gel electrophoresis followed by immunoblot analysis were applied...

journal_title：Journal of neuropathology and experimental neurology

authors： Halliday WC,Yeger H,Duwe GF,Phillips MJ

更新日期：1985-11-01 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：:Frontotemporal dementia with inclusion body myopathy and Paget disease of bone is a rare, autosomal-dominant disorder caused by mutations in the gene valosin-containing protein (VCP). The CNS pathology is characterized by a novel pattern of ubiquitin pathology distinct from sporadic and familial frontotemporal lobar d...

journal_title：Journal of neuropathology and experimental neurology

authors： Neumann M,Mackenzie IR,Cairns NJ,Boyer PJ,Markesbery WR,Smith CD,Taylor JP,Kretzschmar HA,Kimonis VE,Forman MS

更新日期：2007-02-01 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：:Glomeruloid bodies (GBs), tumor-associated vascular structures with a superficial resemblance to renal glomeruli, are important histopathological features of glioblastoma multiforme, but have also been described in other types of tumors and in cerebral metastases. The purpose of this study was to elucidate the pathoge...

journal_title：Journal of neuropathology and experimental neurology

authors： Döme B,Tímár J,Paku S

更新日期：2003-06-01 00:00:00

abstract：:Cerebellar ataxia is a prominent clinical symptom in patients with mitochondrial DNA (mtDNA) disease. This is often progressive with onset in young adulthood. We performed a detailed neuropathologic investigation of the olivary-cerebellum in 14 genetically and clinically well-defined patients with mtDNA disease. Quant...

journal_title：Journal of neuropathology and experimental neurology

authors： Lax NZ,Hepplewhite PD,Reeve AK,Nesbitt V,McFarland R,Jaros E,Taylor RW,Turnbull DM

更新日期：2012-02-01 00:00:00

abstract：:Side population (SP) cells identified using the Hoechst 33342 fluorescent dye efflux technique overlap with the tumor stem cell fraction that is responsible for tumorigenesis and recurrence in malignant gliomas. Because diffuse invasion of glioma cells represents the main obstacle to successful therapy and underlies r...

journal_title：Journal of neuropathology and experimental neurology

authors： Weber K,Paulus W,Senner V

更新日期：2010-06-01 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：: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：:Atypical teratoid/rhabdoid tumors are malignant embryonal tumors characterized by the presence of rhabdoid cells, genetic alterations affecting the SMARCB1 gene (hSNF5/INI1), and a poor prognosis. Whether INI1 plays a role in the pathogenesis of other central nervous system tumors is uncertain. We report on cases of 2...

journal_title：Journal of neuropathology and experimental neurology

authors： Hasselblatt M,Oyen F,Gesk S,Kordes U,Wrede B,Bergmann M,Schmid H,Frühwald MC,Schneppenheim R,Siebert R,Paulus W

更新日期：2009-12-01 00:00:00

abstract：:Prolonged deprivation of vitamin B12 in rhesus monkeys produced changes in the central nervous system that were indistinguishable topographically and histologically from those of human subacute combined degeneration. Ultrastructural studies of early lesions of the spinal cord disclosed a degeneration of myelin charact...

journal_title：Journal of neuropathology and experimental neurology

authors： Agamanolis DP,Victor M,Harris JW,Hines JD,Chester EM,Kark JA

更新日期：1978-05-01 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：: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：:Fas-apoptotic inhibitory molecule 2 (Faim2) is a neuron-specific membrane protein and a member of the evolutionary conserved lifeguard apoptosis regulatory gene family. Its neuroprotective effect in acute neurological diseases has been demonstrated in an in vivo model of focal cerebral ischemia. Here we show that Faim...

journal_title：Journal of neuropathology and experimental neurology

authors： Tauber SC,Harms K,Falkenburger B,Weis J,Sellhaus B,Nau R,Schulz JB,Reich A

更新日期：2014-01-01 00:00:00

abstract：:The phenotypic identities and characterization of neural networks disrupted after neonatal hypoxia-ischemia (HI) in the preterm brain remain to be elucidated. Interruption of the central serotonergic (5-hydroxytryptamine [5-HT]) system can lead to numerous functional deficits, many of which match those in human preter...

journal_title：Journal of neuropathology and experimental neurology

authors： Wixey JA,Reinebrant HE,Buller KM

更新日期：2011-01-01 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：:To understand neuroinflammation-related gene regulation during normal aging and in sporadic Alzheimer disease (sAD), we performed functional genomics analysis and analyzed messenger RNA (mRNA) expression by quantitative reverse transcription-polymerase chain reaction of 22 genes involved in neuroinflammation-like resp...

journal_title：Journal of neuropathology and experimental neurology

authors： López-González I,Schlüter A,Aso E,Garcia-Esparcia P,Ansoleaga B,LLorens F,Carmona M,Moreno J,Fuso A,Portero-Otin M,Pamplona R,Pujol A,Ferrer I

更新日期：2015-04-01 00:00:00

abstract：:1,25-dihydroxyvitamin D3 (1,25-D3) is already known to prevent clinical signs of experimental allergic encephalomyelitis when animals are treated during the immunization phase. In the present work we have evaluated the ability of 1,25-D3 to inhibit chronic relapsing experimental allergic encephalomylitis (EAE) of the ...

journal_title：Journal of neuropathology and experimental neurology

authors： Nataf S,Garcion E,Darcy F,Chabannes D,Muller JY,Brachet P

更新日期：1996-08-01 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：: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：:Bergmann fibers and the distribution of Golgi epithelial cells were significantly altered in staggerer, reeler and double mutant (affected by both staggerer and reeler conditions). However, the Bergmann fiber anomalies in double mutant were not identical to those of either staggerer or reeler. A comparison of these an...

journal_title：Journal of neuropathology and experimental neurology

authors： Yoon CH

更新日期：1977-05-01 00:00:00

abstract：:Growing evidence indicates that alterations within the peripheral nervous system (PNS) are involved at an early stage in the amyotrophic lateral sclerosis (ALS) pathogenetic cascade. In this study, magnetic resonance imaging (MRI), neurophysiologic analyses, and histologic analyses were used to monitor the extent of P...

journal_title：Journal of neuropathology and experimental neurology

authors： Riva N,Chaabane L,Peviani M,Ungaro D,Domi T,Dina G,Bianchi F,Spano G,Cerri F,Podini P,Corbo M,Carro UD,Comi G,Bendotti C,Quattrini A

更新日期：2014-07-01 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：:Mitochondrial Zn2+ accumulation, particularly in CA1 neurons, occurs after ischemia and likely contributes to mitochondrial dysfunction and subsequent neurodegeneration. However, the relationship between mitochondrial Zn2+ accumulation and their disruption has not been examined at the ultrastructural level in vivo. We...

journal_title：Journal of neuropathology and experimental neurology

authors： Yin HZ,Wang HL,Ji SG,Medvedeva YV,Tian G,Bazrafkan AK,Maki NZ,Akbari Y,Weiss JH

更新日期：2019-07-01 00:00:00

abstract：:Genome-wide association studies have established BIN1 (Bridging Integrator 1) as the most significant late-onset Alzheimer disease (AD) susceptibility locus after APOE We analyzed BIN1 protein expression using automated immunohistochemistry on the hippocampal CA1 region in 19 patients with either no, mild, or moderate...

journal_title：Journal of neuropathology and experimental neurology

authors： Adams SL,Tilton K,Kozubek JA,Seshadri S,Delalle I

更新日期：2016-08-01 00:00:00

abstract：:Neuroaxonal dystrophy, a distinctive axonopathy characterized by marked enlargement of distal axons, is the hallmark pathologic alteration in aged and diabetic human prevertebral sympathetic ganglia and in corresponding rodent models. Neuroaxonal dystrophy is thought to represent the abnormal outcome of cycles of syna...

journal_title：Journal of neuropathology and experimental neurology

authors： Schmidt RE,Parvin CA,Green KG

更新日期：2008-12-01 00:00:00

abstract：:The development and differentiation of oligodendrocytes (OC) in developing mouse spinal cord (MSC) were investigated by correlative analysis of light and electron microscopy (EM), and immunoperoxidase studies for glial fibrillary acidic protein (GFAP) and myelin basic protein (MBP). The sequential development of glial...

journal_title：Journal of neuropathology and experimental neurology

authors： Choi BH

更新日期：1986-09-01 00:00:00