Mutational inactivation of the nijmegen breakage syndrome gene (NBS1) in glioblastomas is associated with multiple TP53 mutations.

abstract：:Glioblastoma (GBM), representing WHO grade IV astrocytoma, is a relatively common primary brain tumor in adults with an exceptionally dismal prognosis. With an incidence rate of over 10 000 cases in the United States annually, the median survival rate ranges from 10-15 months in IDH1/2-wildtype tumors and 24-31 months...

journal_title：Journal of neuropathology and experimental neurology

authors： Richardson TE,Patel S,Serrano J,Sathe AA,Daoud EV,Oliver D,Maher EA,Madrigales A,Mickey BE,Taxter T,Jour G,White CL,Raisanen JM,Xing C,Snuderl M,Hatanpaa KJ

更新日期：2019-06-01 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：:Natalizumab, a monoclonal antibody directed against α4 integrins, has, to date, been associated with 399 cases of progressive multifocal leukoencephalopathy (PML) worldwide in patients receiving treatment for multiple sclerosis (MS). Because of the limited number of histologic studies, the possible interplay between M...

journal_title：Journal of neuropathology and experimental neurology

authors： Wüthrich C,Popescu BF,Gheuens S,Marvi M,Ziman R,Denq SP,Tham M,Norton E,Parisi JE,Dang X,Lucchinetti CF,Koralnik IJ

更新日期：2013-11-01 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：: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：:Despite the key role of γ-aminobutyric acid (GABA) neurons in the modulation of cerebral cortical output, little is known about their development in the human cortex. We analyzed several GABAergic parameters in standardized regions of the cerebral cortex and white matter in a total of 38 human fetuses and infants from...

journal_title：Journal of neuropathology and experimental neurology

authors： Xu G,Broadbelt KG,Haynes RL,Folkerth RD,Borenstein NS,Belliveau RA,Trachtenberg FL,Volpe JJ,Kinney HC

更新日期：2011-10-01 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：:The Daumas-Duport grading scheme (DDGS) is a commonly used method for determining the grade of a tumor. It scores 4 histologic features and is used as a prognostic tool in adult astroglial tumors. This system of assigning children to prognostically homogeneous groups has not been evaluated. The Childhood Brain Tumor C...

journal_title：Journal of neuropathology and experimental neurology

authors： Brown WD,Gilles FH,Tavaré CJ,Rorke LB,Davis RL,Adelman L,Hedley-Whyte ET,Leviton A

更新日期：1998-11-01 00:00:00

abstract：:Selenoprotein P (Sepp1) is involved in selenium homeostasis. Mice with a deletion of Sepp1, replacement of it by the shortened form Sepp1(Delta240-361), or deletion of its receptor apolipoprotein E receptor 2 develop severe neurologic dysfunction when fed low-selenium diet. Because the brainstems of Sepp1(-/-) mice ha...

journal_title：Journal of neuropathology and experimental neurology

authors： Valentine WM,Abel TW,Hill KE,Austin LM,Burk RF

更新日期：2008-01-01 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：:Hereditary diffuse leukoencephalopathy with spheroids (HDLS) is a familial neurodegenerative disease clinically characterized by progressive cognitive and motor dysfunction. Mutations in the colony-stimulating factor 1 receptor (CSF1R) gene have recently been identified in HDLS patients. The presence of diffuse axonal...

journal_title：Journal of neuropathology and experimental neurology

authors： Riku Y,Ando T,Goto Y,Mano K,Iwasaki Y,Sobue G,Yoshida M

更新日期：2014-12-01 00:00:00

abstract：:The content of RNA and the volume of motor neurons isolated from the lateral portion of the cervical swelling were examined in six control and six amyotrophic lateral sclerosis (ALS) cases obtained at autopsy. The mean volume of motor neurons in the ALS group did not differ significantly from the values obtained in th...

journal_title：Journal of neuropathology and experimental neurology

authors： Davidson TJ,Hartmann HA,Johnson PC

更新日期：1981-01-01 00:00:00

abstract：:The effect of experimental obstructive jaundice on the oxidative status of brain tissues in rats was examined. Twenty-four male Wistar rats were divided into 4 groups: Group I was the control, group II was the sham operated, and groups III and IV were bile duct ligated and killed on the 5th and the 10th day, respectiv...

journal_title：Journal of neuropathology and experimental neurology

authors： Chroni E,Patsoukis N,Karageorgos N,Konstantinou D,Georgiou C

更新日期：2006-02-01 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：:An A and L system of neutral amino acid transport has been demonstrated previously in cerebral microvessels in vivo and in isolated microvessels in vitro. This report describes the neutral amino acid transport properties of cultured cerebral endothelial cells and investigates the influence of astroglia on the transpor...

journal_title：Journal of neuropathology and experimental neurology

authors： Cancilla PA,DeBault LE

更新日期：1983-03-01 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：:Cerebral microbleeds (CMB) are a common MRI finding, representing underlying cerebral microhemorrhages (CMH). The etiology of CMB and microhemorrhages is obscure. We conducted a pathological investigation of CMH, combining standard and immunohistological analyses of postmortem human brains. We analyzed 5 brain regions...

journal_title：Journal of neuropathology and experimental neurology

authors： Wadi LC,Grigoryan MM,Kim RC,Fang C,Kim J,Corrada MM,Paganini-Hill A,Fisher MJ

更新日期：2020-10-01 00:00:00

abstract：:To determine whether arterial wall degeneration, in combination with hemodynamic insult, causes cerebral artery aneurysms in a dog model, we simulated the geometry and hemodynamics of a human artery by surgical reconstruction of both common carotid arteries in 12 dogs. The dogs were then randomly assigned to one of th...

journal_title：Journal of neuropathology and experimental neurology

authors： Zhu YQ,Li MH,Yan L,Tan HQ,Cheng YS

更新日期：2014-09-01 00:00:00

abstract：:Studies dealing with transport of proteins from the oligodendrocyte cell body to the myelin sheath reveal the presence of different transport pathways. Proteolipid protein (PLP) is synthesized at the rough endoplasmic reticulum (ER) and then processed through the Golgi apparatus and transported to the myelin membranes...

journal_title：Journal of neuropathology and experimental neurology

authors： Bauer J,Bradl M,Klein M,Leisser M,Deckwerth TL,Wekerle H,Lassmann H

更新日期：2002-01-01 00:00:00

abstract：:Cerebral palsy is a common birth disorder that frequently involves ischemic-type injury to developing white matter (WM). Dead glial cells are a common feature of this injury and here we describe a novel form of acute ischemic cell death in developing WM astrocytes. Ischemia, modeled by the withdrawal of oxygen and glu...

journal_title：Journal of neuropathology and experimental neurology

authors： Thomas R,Salter MG,Wilke S,Husen A,Allcock N,Nivison M,Nnoli AN,Fern R

更新日期：2004-08-01 00:00:00

abstract：:Sibling concurrence of pathologically confirmed prion disease has only been reported in association with pathogenic mutation of the prion protein gene (PRNP). Here, we report 2 siblings with classic neuropathologic features of sporadic Creutzfeldt-Jakob disease unexplained by PRNP mutation or known risk factors for ia...

journal_title：Journal of neuropathology and experimental neurology

authors： Webb TE,Pal S,Siddique D,Heaney DC,Linehan JM,Wadsworth JD,Joiner S,Beck J,Wroe SJ,Stevenson V,Brandner S,Mead S,Collinge J

更新日期：2008-09-01 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：:Much of the morbidity after traumatic brain injury (TBI) is associated with traumatic axonal injury (TAI). Although most TAI studies focus on corpus callosum white matter, the visual system has received increased interest. To assess visual system TAI, we developed a mouse model of optic nerve TAI. It is unknown, howev...

journal_title：Journal of neuropathology and experimental neurology

authors： Wang J,Fox MA,Povlishock JT

更新日期：2013-08-01 00:00:00

abstract：:After the finding that anti-prion antibodies stain sensory and sympathetic ganglia in variant Creutzfeldt-Jakob disease (vCJD), it was suggested that this localization supported the oral route of entry. However, prion accumulation subsequently also appeared in the peripheral nervous system (PNS) in sporadic cases. Thi...

journal_title：Journal of neuropathology and experimental neurology

authors： Lee CC,Kuo LT,Wang CH,Scaravilli F,An SF

更新日期：2005-08-01 00:00:00

abstract：:Acetylcholinesterase (AChE) activity has been studied in normal, control and denervated muscle of rabbits by electron microscopic-cytochemistry and radiometric assay. A small amount of butyrylcholinesterase (BuChE) activity is also found in biochemical assay of unfixed muscle, but it is not demonstrable cytochemically...

journal_title：Journal of neuropathology and experimental neurology

authors： Tennyson VM,Kremzner LT,Brzin M

更新日期：1977-03-01 00:00:00

abstract：:Glial cytoplasmic inclusions (GCIs) are a pathologic hallmark of multiple system atrophy (MSA), but their pathogenetic roles need to be clarified. To determine possible roles of GCIs in individual cells, serial optical sections obtained by confocal microscopy were reconstructed to yield 3-dimensional (3D) images of th...

journal_title：Journal of neuropathology and experimental neurology

authors： Uyama N,Uchihara T,Mochizuki Y,Nakamura A,Takahashi R,Mizutani T

更新日期：2009-10-01 00:00:00

abstract：:Localized hypertrophic neuropathy is a rare Schwann cell proliferation that usually affects single nerves from the extremities, and it is of unclear etiology in its pure form. RASopathies are a defined group of genetic diseases with overlapping clinical features, usually secondary to germline mutations in genes encodi...

journal_title：Journal of neuropathology and experimental neurology

authors： Vizcaino MA,Belzberg A,Ahlawat S,Belakhoua S,Chen L,Staedtke V,Rodriguez FJ

更新日期：2020-06-01 00:00:00

abstract：:The tau deposits found in neurodegenerative diseases are classified based on their isoforms, that is, 3-repeat (3R) tau and 4-repeat (4R) tau. These isoforms are distinguishable using the antibodies RD3 and RD4, respectively, and Gallyas (Gal) and Campbell-Switzer (CS) silver staining methods, respectively. Tau is als...

journal_title：Journal of neuropathology and experimental neurology

authors： Ichihara K,Uchihara T,Nakamura A,Suzuki Y,Mizutani T

更新日期：2009-09-01 00:00:00

abstract：:β-Amyloid (Aβ) plaques can trigger chronic inflammation in the cellular environment that recruits infiltrating macrophages during the course of Alzheimer disease (AD). Activated macrophages release pro-inflammatory cytokines that increase neurotoxicity associated with AD. A major impediment to investigating neuroinfla...

journal_title：Journal of neuropathology and experimental neurology

authors： Kozyrev N,Albers S,Yang J,Prado VF,Prado MAM,Fonseca GJ,Rylett RJ,Dekaban GA

更新日期：2020-11-01 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