Expression of Mutant Ubiquitin and Proteostasis Impairment in Kii Amyotrophic Lateral Sclerosis/Parkinsonism-Dementia Complex Brains.

abstract：:Adult male rats were housed in a colony environment for six months, with ad lib access to anise-flavored 10% ethanol in water. Animals were then removed from the colony, and their consumption of alcohol during a period in isolated housing was measured. Individual rats were scored as high, moderate, and low consumers. ...

journal_title：Journal of neuropathology and experimental neurology

authors： Goldstein B,Maxwell DS,Ellison G,Hammer RP

更新日期：1983-09-01 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：: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：: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：: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：:We studied differences in the number and morphology of parvalbumin-immunoreactive (PV-IR) interneurons in 43 hippocampal specimens from patients with classical Ammon's horn sclerosis (AHS) who underwent anterior temporal lobectomy, as compared with 14 autopsy and non-AHS surgical control specimens. PV-IR neuronal loss...

journal_title：Journal of neuropathology and experimental neurology

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

更新日期：1997-09-01 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：:The effects of regional heterogeneity on the accuracy of histological grading of gliomas are well known, but little has been reported about its implications for other diagnostic modalities. This study investigated the relationships of regional heterogeneity in tumor proliferative activity, measured by Ki-67 labeling i...

journal_title：Journal of neuropathology and experimental neurology

authors： Coons SW,Johnson PC

更新日期：1993-11-01 00:00:00

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：:The light and electron microscopic features of an unique retroperitoneal ganglioneuroblastoma in a four-year-old female are described. The unprecedented concurrence of Hirano, zebra, membranous cytoplasmic (MCB), and Pick bodies in the same population of neoplastic, sympathetic ganglion cells provides further evidence...

journal_title：Journal of neuropathology and experimental neurology

authors： Powers JM,Balentine JD,Wiśniewski HM,Terry RD

更新日期：1976-01-01 00:00:00

abstract：:Clinical and neuropathological staging of Alzheimer disease (AD) neurodegeneration and neuronal loss dynamics is the baseline for identification of treatment targets and timing. The aim of this study of 14 brain regions in 25 subjects diagnosed with AD and 13 age-matched control subjects was to establish the pattern o...

journal_title：Journal of neuropathology and experimental neurology

authors： Wegiel J,Flory M,Kuchna I,Nowicki K,Ma SY,Wegiel J,Badmaev E,Leon M,Wisniewski T,Reisberg B

更新日期：2021-01-01 00:00:00

abstract：:Neurofilamentous axonal swellings occur in a number of degenerative and toxic disorders of the nervous system. In one of these, experimental intoxication with beta, beta'-iminodiproprionitrile (IDPN), accumulation of neurofilaments has been shown to result from a defect in slow axonal transport. The consequence of thi...

journal_title：Journal of neuropathology and experimental neurology

authors： Clark AW,Griffin JW,Price DL

更新日期：1980-01-01 00:00:00

abstract：:We have reported that the ambient gyrus is the site with the greatest accumulation of argyrophilic grains (AGs) and that the degeneration of the ambient gyrus is responsible for dementia with grains. Here we analyzed 1,405 serial autopsy cases from 2 hospitals and detected AGs only in cases older than 56 years of age....

journal_title：Journal of neuropathology and experimental neurology

authors： Saito Y,Ruberu NN,Sawabe M,Arai T,Tanaka N,Kakuta Y,Yamanouchi H,Murayama S

更新日期：2004-09-01 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：:The roles of bone morphogenetic proteins (BMPs) and their receptors (BMPRs) in meningioma biology are not known. In this study, frozen tissues from 26 World Health Organization Grades I to III meningiomas were analyzed by Western blot for BMP-2/4, BMPR IA, and BMPR II, and activation of downstream p-Smad1, p38 mitogen...

journal_title：Journal of neuropathology and experimental neurology

authors： Johnson MD,O'Connell MJ,Vito F,Pilcher W

更新日期：2009-11-01 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：:Aberrant epidermal growth factor receptor (EGFR) expression promotes the pathogenesis of malignant peripheral nerve sheath tumors (MPNSTs), the most common malignancy associated with neurofibromatosis type 1, but the mechanisms by which EGFR expression promotes MPNST pathogenesis are poorly understood. We hypothesized...

journal_title：Journal of neuropathology and experimental neurology

authors： Byer SJ,Brossier NM,Peavler LT,Eckert JM,Watkins S,Roth KA,Carroll SL

更新日期：2013-03-01 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：: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：:We investigated whether antibody production to antigens arising in the subarachnoid space is depressed acutely after spinal cord injury (SCI), and whether such depression is due to abnormal catecholamine levels. To assess antibody responses, ovalbumin (OVA) was injected into the spinal subarachnoid space (i.t.) of rat...

journal_title：Journal of neuropathology and experimental neurology

authors： Vega JL,Ganea D,Jonakait GM

更新日期：2003-08-01 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：: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：:A working group supported by the Office of Rare Diseases of the National Institutes of Health formulated neuropathologic criteria for corticobasal degeneration (CBD) that were subsequently validated by an independent group of neuropathologists. The criteria do not require a specific clinical phenotype, since CBD can h...

journal_title：Journal of neuropathology and experimental neurology

authors： Dickson DW,Bergeron C,Chin SS,Duyckaerts C,Horoupian D,Ikeda K,Jellinger K,Lantos PL,Lippa CF,Mirra SS,Tabaton M,Vonsattel JP,Wakabayashi K,Litvan I,Office of Rare Diseases of the National Institutes of Health.

更新日期：2002-11-01 00:00:00

abstract：:The changes of nitric oxide synthase (NOS) activity and expression in experimental diabetic neuropathy have not been examined. Increases in ganglia NOS might be similar to those that follow axotomy, whereas declines in endothelial NOS (eNOS) and immunological NOS (iNOS) might explain dysfunction of microvessels or mac...

journal_title：Journal of neuropathology and experimental neurology

authors： Zochodne DW,Verge VM,Cheng C,Höke A,Jolley C,Thomsen K,Rubin I,Lauritzen M

更新日期：2000-09-01 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 aim of this study was to clarify the relationship between craniopharyngiomas (CP) and the third ventricle floor by analyzing the membranes between them. Eight fetal specimens were first examined by hematoxylin and eosin and immunofluorescence staining to determine optimal markers for identifying membrane structure...

journal_title：Journal of neuropathology and experimental neurology

authors： Qi S,Liu Y,Wang C,Fan J,Pan J,Zhang X,Lu Y

更新日期：2020-09-01 00:00:00

abstract：:Experimental cerebral ischemia was produced in gerbils by occlusion of the right common carotid artery in the neck. The evolution of the ischemic lesions was followed from five minutes to six hours by using the immunohistochemical techniques for tubulin and creatine kinase BB-isoenzyme. The earliest lesion was found i...

journal_title：Journal of neuropathology and experimental neurology

authors： Yanagihara T,Yoshimine T,Morimoto K,Yamamoto K,Homburger HA

更新日期：1985-03-01 00:00:00

abstract：:Although stroke is among the most common causes of death and chronic disability worldwide, the proteome of the ischemic human brain remains unknown. Only a few studies have investigated the ischemic brain proteome in rodent stroke models. We performed a proteomic study of the human brain after ischemic stroke using a ...

journal_title：Journal of neuropathology and experimental neurology

authors： Cuadrado E,Rosell A,Colomé N,Hernández-Guillamon M,García-Berrocoso T,Ribo M,Alcazar A,Ortega-Aznar A,Salinas M,Canals F,Montaner J

更新日期：2010-11-01 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：:The association of Charcot-Marie-Tooth (CMT) disease with renal dysfunction is uncommon but has long been recognized in several families. Recently, mutations in the INF2 gene, which encodes inverted formin-2, were identified in patients with focal segmental glomerulosclerosis and a dominant intermediate form of CMT (C...

journal_title：Journal of neuropathology and experimental neurology

authors： Mathis S,Funalot B,Boyer O,Lacroix C,Marcorelles P,Magy L,Richard L,Antignac C,Vallat JM

更新日期：2014-03-01 00:00:00