The mucopolysaccharides of the ground substance of the human brain.

abstract：:Spinal muscular atrophy (SMA) is characterized by loss of motor neurons in the spinal cord that results in muscle denervation and profound weakness in affected patients. We sought evidence for primary muscle involvement in the disease during human development by analyzing the expression of several muscle cytoskeletal ...

journal_title：Journal of neuropathology and experimental neurology

authors： Martínez-Hernández R,Bernal S,Alias L,Tizzano EF

更新日期：2014-06-01 00:00:00

abstract：:The accumulation of misfolded or unfolded proteins in the endoplasmic reticulum (ER) lumen causes a cellular stress response termed the unfolded protein response. Although ER stress has been implicated in various neurodegenerative diseases, the morphological features of aggregated proteins in ER lumina that may cause ...

journal_title：Journal of neuropathology and experimental neurology

authors： Sasaki S

更新日期：2010-04-01 00:00:00

abstract：:The expression of preproenkephalin messenger RNA was studied in the brain of Parkinson disease (PD) patients using in situ hybridization. All these patients were treated with levodopa (LD) and the development of motor complications was recorded. Eleven normal controls and 14 PD patients were used, of which 4 developed...

journal_title：Journal of neuropathology and experimental neurology

authors： Calon F,Birdi S,Rajput AH,Hornykiewicz O,Bédard PJ,Di Paolo T

更新日期：2002-02-01 00:00:00

abstract：:Vanishing white matter disease (VWM) is a progressive cavitating disease of central white matter due to a deficiency of the translation initiation factor eIF2B. Oligodendrocytes appear to be numerically increased in some white matter areas, while decreased in others. We compared oligodendrocytes of cerebral, cerebella...

journal_title：Journal of neuropathology and experimental neurology

authors： Van Haren K,van der Voorn JP,Peterson DR,van der Knaap MS,Powers JM

更新日期：2004-06-01 00:00:00

abstract：:Brain aging is accompanied by increased oxidative stress and what has been termed "neuroinflammation," which might contribute to age-related neurodegenerative diseases. We analyzed expression in the transcription of innate inflammatory response genes in eleven representative regions including frontal, parietal, inferi...

journal_title：Journal of neuropathology and experimental neurology

authors： López-González I,Tebé Cordomí C,Ferrer I

更新日期：2017-02-01 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

abstract：:Neuron-derived molecules are potent regulators of oligodendrocyte differentiation and myelination during brain development and upon demyelination. Their analysis will thus contribute to understanding remyelination failure in demyelinating diseases, such as multiple sclerosis (MS). Previously, we have identified neuron...

journal_title：Journal of neuropathology and experimental neurology

authors： de Jong CGHM,Stancic M,Pinxterhuis TH,van Horssen J,van Dam AM,Gabius HJ,Baron W

更新日期：2018-11-01 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：: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：:Essential tremor (ET) patients develop more cognitive impairment and dementia than controls, although there are surprisingly few data on the neuropathological basis for cognitive changes in ET. In this postmortem study, we assessed tau and other pathologies in 26 ET cases and 73 controls (non-ET) (1:3 matching). The m...

journal_title：Journal of neuropathology and experimental neurology

authors： Farrell K,Cosentino S,Iida MA,Chapman S,Bennett DA,Faust PL,Louis ED,Crary JF

更新日期：2019-01-01 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：:Brain atrophy is a common finding in patients with AIDS, but the relationship of atrophy to HIV-associated dementia is unclear. We used unbiased, stereological methods on postmortem brain specimens to estimate volumes of different brain regions in patients prospectively diagnosed with and without HIV-associated dement...

journal_title：Journal of neuropathology and experimental neurology

authors： Subbiah P,Mouton P,Fedor H,McArthur JC,Glass JD

更新日期：1996-10-01 00:00:00

abstract：:Versican is a large extracellular proteoglycan and is expressed in a variety of tissues including the central nervous system. A malignant astrocytoma cell line U87 with high motility expressed a higher level of versican than another malignant astrocytoma cell line U343 with lower motility. We observed that the U87 cel...

journal_title：Journal of neuropathology and experimental neurology

authors： Ang LC,Zhang Y,Cao L,Yang BL,Young B,Kiani C,Lee V,Allan K,Yang BB

更新日期：1999-06-01 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：:Well myelinated cultures of newborn mouse cerebellum, exposed to varying concentrations of sodium diethyldithiocarbamate (DDC), a heavy metal chelating agent, were examined by light and electron microscopy. DDC treatment of cultures for 24-48 hours produced swellings of axons and presynaptic endings, the morphological...

journal_title：Journal of neuropathology and experimental neurology

authors： Kim SU,Rizzuto N

更新日期：1975-11-01 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：: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：:Molecular pathology has identified 2 distinct forms of neuronal inclusion body in Amyotrophic Lateral Sclerosis (ALS). ALS-type inclusions are skeins or small dense filamentous aggregates which can only be demonstrated by ubiquitin immunocytochemistry (ICC). In contrast hyaline conglomerates (HC) are large multifocal ...

journal_title：Journal of neuropathology and experimental neurology

authors： Ince PG,Tomkins J,Slade JY,Thatcher NM,Shaw PJ

更新日期：1998-10-01 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：:In many degenerative and metabolic muscle diseases cells die without a marked inflammatory response. A potential mechanism how this could be accomplished is apoptotic cell death. Since there are no data available about apoptosis in cells of myogenic origin, we examined rat skeletal myoblasts for their potential to und...

journal_title：Journal of neuropathology and experimental neurology

authors： Stangel M,Zettl UK,Mix E,Zielasek J,Toyka KV,Hartung HP,Gold R

更新日期：1996-01-01 00:00:00

abstract：:In Niemann-Pick Type C (NPC) disease, the concentration of cholesterol increases with age in every tissue except the brain. This study investigates whether accumulation of cholesterol might also occur within the cells of the central nervous system (CNS), but be obscured by the simultaneous loss of sterol from myelin a...

journal_title：Journal of neuropathology and experimental neurology

authors： Xie C,Burns DK,Turley SD,Dietschy JM

更新日期：2000-12-01 00:00:00

abstract：:The idiopathic inflammatory myopathies (IIMs) dermatomyositis, polymyositis, and inclusion body myositis are characterized by myofiber degeneration and inflammation. The triggering factors of muscle autoaggression in these disorders are unknown, but infiltrating T cells may be activated locally and proliferate in situ...

journal_title：Journal of neuropathology and experimental neurology

authors： Bernasconi P,Cappelletti C,Navone F,Nessi V,Baggi F,Vernos I,Romaggi S,Confalonieri P,Mora M,Morandi L,Mantegazza R

更新日期：2008-06-01 00:00:00

abstract：:Injury to neonatal nerves, unlike adult nerves, results in poor regeneration and extensive motoneuron death. We examined whether exposure to a more mature nerve environment could rescue axotomized motoneurons following neonatal injury. The sciatic nerve in 1 hindlimb of 3-day-old (P3) rats was transected and the cut e...

journal_title：Journal of neuropathology and experimental neurology

authors： Low HL,Nogradi A,Vrbová G,Greensmith L

更新日期：2003-01-01 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：:Cerebral accumulation of amyloid-beta (Abeta) is characteristic of Alzheimer disease and of amyloid precursor protein (APP) transgenic mice. Here, we assessed the efficacy of CI-1011, an inhibitor of acyl-coenzyme A:cholesterol acyltransferase, which is suitable for clinical use, in reducing amyloid pathology in both ...

journal_title：Journal of neuropathology and experimental neurology

authors： Huttunen HJ,Havas D,Peach C,Barren C,Duller S,Xia W,Frosch MP,Hutter-Paier B,Windisch M,Kovacs DM

更新日期：2010-08-01 00:00:00

abstract：:A series of 43 human gliomas, consisting of 30 glioblastomas, 7 anaplastic astrocytomas, 3 low grade astrocytomas, 2 ependymomas, and 1 oligodendroglioma, was studied for amplification of the epidermal growth factor receptor (EGFR) and mouse double minute 2 (MDM2) genes. DNA extracted from formalin-fixed, paraffin-emb...

journal_title：Journal of neuropathology and experimental neurology

authors： Hunter SB,Abbott K,Varma VA,Olson JJ,Barnett DW,James CD

更新日期：1995-01-01 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：:To develop improved therapies for medulloblastoma, we studied the ability of a neuroattenuated HSV-1 ICP34.5 mutant (variant-1716) to replicate within and destory an authentic medulloblastoma cell line known as Med 283 (D283) using immunohistochemistry, in situ hybridization, and viral titrations. In vitro studies sho...

journal_title：Journal of neuropathology and experimental neurology

authors： Lasner TM,Kesari S,Brown SM,Lee VM,Fraser NW,Trojanowski JQ

更新日期：1996-12-01 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：:The interaction of phosphatidylserine (PS), exposed on the surface of apoptotic cells and with its specific receptor (PtdSerR) expressed by microglia, is a crucial event in the recognition and clearance of apoptotic neurons. Here, we extend our previous studies in which PS-liposomes mimicking apoptotic cells were used...

journal_title：Journal of neuropathology and experimental neurology

authors： De Simone R,Ajmone-Cat MA,Tirassa P,Minghetti L

更新日期：2003-02-01 00:00:00