Spinal manifestations of CLN1 disease start during the early postnatal period.

abstract：AIMS:14,15-Epoxyeicosatrienoic acid (14,15-EET) is abundantly expressed in brain and exerts protective effects against ischaemia. 14,15-EET is hydrolysed by soluble epoxide hydrolase (sEH). sEH-/- mice show a higher level of 14,15-EET in the brain. Astrocytes play a pivotal role in neuronal survival under ischaemic con...

journal_title：Neuropathology and applied neurobiology

authors： Yuan L,Liu J,Dong R,Zhu J,Tao C,Zheng R,Zhu S

更新日期：2016-12-01 00:00:00

abstract：:The effects of exposure to a cytotoxic agent, methylazoxymethanol acetate (MAM Ac), on the distribution, density and quantitative morphology of microglia in the rat forebrain have been examined with the aid of a peroxidase-conjugated lectin derived from Griffonia simplicifolia. Following exposure to MAM Ac (25 mg/kg m...

journal_title：Neuropathology and applied neurobiology

authors： Ashwell KW

更新日期：1992-12-01 00:00:00

abstract：:Dysfunction of the ubiquitin-dependent proteolytic pathway contributes to progressive accumulation of ubiquitinated protein inclusions in neurodegenerative disorders, such as Parkinson's disease (PD). Ubiquitin C-terminal hydrolase-L1 (UCH-L1), alternatively designated protein gene product 9.5 (PGP9.5), is a neural de...

journal_title：Neuropathology and applied neurobiology

authors： Satoh JI,Kuroda Y

更新日期：2001-04-01 00:00:00

abstract：:An unusual inherited progressive distal myopathy of early childhood onset is described in two sisters from a consanguineous Asian family. Motor milestones were normal but gait deteriorated slowly thereafter with development of generalized hypotonia and muscle weakness particularly in the wrist extensors and hand muscl...

journal_title：Neuropathology and applied neurobiology

authors： Mahon M,Kristmundsdottir F,Cumming WJ,Noronha MJ

更新日期：1989-01-01 00:00:00

abstract：BACKGROUND:In definite Creutzfeldt-Jakob disease (CJD), morphological and immunohistochemical patterns are useful to identify molecular subtypes. Severe cerebellar pathology and hippocampal involvement helps to identify VV subtypes. The rare VV1 variant (<1%), more frequent in young individuals, is additionally charact...

journal_title：Neuropathology and applied neurobiology

authors： Martinez-Saez E,Gelpi E,Rey MJ,Ferrer I,Ribalta T,Botta-Orfila T,Nos C,Yagüe J,Sanchez-Valle R

更新日期：2012-04-01 00:00:00

abstract：:Alzheimer's and Parkinson's diseases (AD, PD) are among the most common neurodegenerative disorders in adults. Both AD and PD have a complex aetiology, and it is widely considered that genetic factors, acting independently or in concert with other genetic and/or environmental factors, modify the risk of developing the...

journal_title：Neuropathology and applied neurobiology

authors： Egensperger R,Kösel S,Schnopp NM,Mehraein P,Graeber MB

更新日期：1997-08-01 00:00:00

abstract：AIMS:The aim of this study was to describe the regional profiles of microglial activation in sporadic Creutzfeldt-Jakob disease (sCJD) subtypes and analyse the influence of prion strain, disease duration and codon 129 genotype. METHODS:We studied the amount/severity and distribution of activated microglia, protease-re...

journal_title：Neuropathology and applied neurobiology

authors： Franceschini A,Strammiello R,Capellari S,Giese A,Parchi P

更新日期：2018-10-01 00:00:00

abstract：:Transparent Golgi preparations counterstained for Alzheimer's neurofibrillary changes rendered possible the demonstration of neuropil threads in defined cellular processes. Only dendrites of tangle-bearing cortical nerve cells were found to contain neuropil threads. Processes of glial cells as well as axons present in...

journal_title：Neuropathology and applied neurobiology

authors： Braak H,Braak E

更新日期：1988-01-01 00:00:00

abstract：:The topography and cellular events in the experimental lesions caused by chlorosugars, 6-aminonicotinamide, dinitrobenzene and tribromoimidazole in animals are considered in relation to those features in human acute thiamine deficiency (Wernicke's) encephalopathy and for comparison in Leigh's disease. The topography a...

journal_title：Neuropathology and applied neurobiology

authors： Cavanagh JB

更新日期：1993-12-01 00:00:00

abstract：AIMS:Brain oedema is a major contributing factor to the morbidity and mortality of a variety of brain disorders. Although there has been considerable progress in our understanding of pathophysiological and molecular mechanisms associated with brain oedema so far, more effective treatment is required and is still awaite...

journal_title：Neuropathology and applied neurobiology

authors： Karmacharya MB,Kim KH,Kim SY,Chung J,Min BH,Park SR,Choi BH

更新日期：2015-06-01 00:00:00

abstract：AIMS:Nakajo-Nishimura syndrome (NNS) is an autosomal recessive disease caused by biallelic mutations in the PSMB8 gene that encodes the immunoproteasome subunit β5i. There have been only a limited number of reports on the clinicopathological features of the disease in genetically confirmed cases. METHODS:We studied cl...

journal_title：Neuropathology and applied neurobiology

authors： Ayaki T,Murata K,Kanazawa N,Uruha A,Ohmura K,Sugie K,Kasagi S,Li F,Mori M,Nakajima R,Sasai T,Nishino I,Ueno S,Urushitani M,Furukawa F,Ito H,Takahashi R

更新日期：2020-10-01 00:00:00

abstract：:We have previously demonstrated that amyloid beta (Abeta) peptide is acutely toxic to retinal neurones in vivo and that this toxicity is mediated by an indirect mechanism. We have now extended these studies to look at the chronic effect of intravitreal injection of Abeta peptides on retinal ganglion cells (RGC), the p...

journal_title：Neuropathology and applied neurobiology

authors： Walsh DT,Bresciani L,Saunders D,Manca MF,Jen A,Gentleman SM,Jen LS

更新日期：2005-10-01 00:00:00

abstract：:Creutzfeldt-Jakob disease (CJD) is characterized by a loss of neurons accompanied by astrogliosis and spongiform changes in the neuropil. It has been recognized that reactive microglia occur in CJD but little is known about the regional distribution and extent of the microglial activation. We have, therefore, examined...

journal_title：Neuropathology and applied neurobiology

authors： Mühleisen H,Gehrmann J,Meyermann R

更新日期：1995-12-01 00:00:00

abstract：:Professional boxers and other contact sport athletes are exposed to repetitive brain trauma that may affect motor functions, cognitive performance, emotional regulation and social awareness. The term of chronic traumatic encephalopathy (CTE) was recently introduced to regroup a wide spectrum of symptoms such as cerebe...

journal_title：Neuropathology and applied neurobiology

authors： Costanza A,Weber K,Gandy S,Bouras C,Hof PR,Giannakopoulos P,Canuto A

更新日期：2011-10-01 00:00:00

abstract：AIMS:Plectin, a universally expressed multi-functional cytolinker protein, is crucial for intermediate filament networking, including crosstalk with actomyosin and microtubules. In addition to its involvement in a number of diseases affecting skin, skeletal muscle, heart, and other stress-exposed tissues, indications f...

journal_title：Neuropathology and applied neurobiology

authors： Valencia RG,Mihailovska E,Winter L,Bauer K,Fischer I,Walko G,Jorgacevski J,Potokar M,Zorec R,Wiche G

更新日期：2021-02-01 00:00:00

abstract：:Medulloblastomas (MBs) are malignant primitive neuroectodermal tumours (PNETs) of the cerebellum occurring predominantly in childhood. The association of monosomy of chromosome 22 with MB is controversial. Atypical teratoid/rhabdoid tumours (AT/RTs) of the brain share clinical and histological features with MBs and su...

journal_title：Neuropathology and applied neurobiology

authors： Kraus JA,Oster C,Sörensen N,Berthold F,Schlegel U,Tonn JC,Wiestler OD,Pietsch T

更新日期：2002-04-01 00:00:00

abstract：:A 50-year-old female patient died of an untreatable glioma apoplecticum. At autopsy a strongly vascularized glial tumour was found. The criteria for malignancy according to the WHO classification were only partially fulfilled by this tumour which displayed morphological features of an astrocytoma but could not be furt...

journal_title：Neuropathology and applied neurobiology

authors： Radner H,Kleinert R,Vennigerholz F,Denk H

更新日期：1990-04-01 00:00:00

abstract：:Ubiquitin targets proteins for attack by certain proteolytic enzymes, but the ubiquitinated cytoplasmic inclusions seen in some chronic neurodegenerative diseases may indicate the occurrence of reparative rather than destructive metabolic events. We have examined the production of ubiquitin in motor neurons of the rat...

journal_title：Neuropathology and applied neurobiology

authors： Savedia S,Kiernan JA

更新日期：1994-12-01 00:00:00

abstract：AIMS:Frontotemporal lobar degeneration (FTLD) is clinically and pathologically heterogeneous. Although associated with variations in MAPT, GRN and C9ORF72, the pathogenesis of these, and of other nongenetic, forms of FTLD, remains unknown. Epigenetic factors such as histone regulation by histone deacetylases (HDAC) may...

journal_title：Neuropathology and applied neurobiology

authors： Whitehouse A,Doherty K,Yeh HH,Robinson AC,Rollinson S,Pickering-Brown S,Snowden J,Thompson JC,Davidson YS,Mann DM

更新日期：2015-02-01 00:00:00

abstract：:Axonal damage in multiple sclerosis (MS) is correlated to disease progression. Early axonal damage may be compensated for by regenerative processes. Growth-associated protein 43 (GAP-43) is a marker for axonal growth and synaptogenesis in various neurodegenerative diseases. We investigated the expression of GAP-43 in ...

journal_title：Neuropathology and applied neurobiology

authors： Teunissen CE,Dijkstra CD,Jasperse B,Barkhof F,Vanderstichele H,Vanmechelen E,Polman CH,Bö L

更新日期：2006-06-01 00:00:00

abstract：AIMS:Neurogenesis in adult humans occurs in at least two areas of the brain, the subventricular zone of the telencephalon and the subgranular layer of the dentate gyrus in the hippocampal formation. We studied dentate gyrus subgranular layer neurogenesis in patients subjected to tailored antero-mesial temporal resectio...

journal_title：Neuropathology and applied neurobiology

authors： Paradisi M,Fernández M,Del Vecchio G,Lizzo G,Marucci G,Giulioni M,Pozzati E,Antonelli T,Lanzoni G,Bagnara GP,Giardino L,Calzà L

更新日期：2010-10-01 00:00:00

abstract：:Recent studies have described silver- and tau-positive glia and threads in the degenerating lesions of progressive supranuclear palsy. In this study, Gallyas-Braak silver impregnation and several immunohistochemical techniques were employed to examine the distribution of tangles, abnormal glia and threads in the cereb...

journal_title：Neuropathology and applied neurobiology

authors： Hanihara T,Amano N,Takahashi T,Nagatomo H,Yagashita S

更新日期：1995-08-01 00:00:00

abstract：:Patients infected with the malaria parasite Plasmodium falciparum may develop a diffuse reversible encephalopathy, termed cerebral malaria. It is unclear how the intraerythrocytic parasite, which sequesters in the cerebral microvasculature but does not enter the brain parenchyma, induces this neurological syndrome. Ad...

journal_title：Neuropathology and applied neurobiology

authors： Brown H,Hien TT,Day N,Mai NT,Chuong LV,Chau TT,Loc PP,Phu NH,Bethell D,Farrar J,Gatter K,White N,Turner G

更新日期：1999-08-01 00:00:00

abstract：AIM:Peroxisomes play a key role in lipid metabolism, and peroxisome defects have been associated with neurodegenerative diseases such as X-adrenoleukodystrophy and Alzheimer's disease. This study aims to elucidate the contribution of peroxisomes in lipid alterations of area 8 of the frontal cortex in the spectrum of TD...

journal_title：Neuropathology and applied neurobiology

authors： Andrés-Benito P,Gelpi E,Jové M,Mota-Martorell N,Obis È,Portero-Otin M,Povedano M,Pujol A,Pamplona R,Ferrer I

更新日期：2020-12-17 00:00:00

abstract：:Over the past few years, the traditional view of brain tumorigenesis has been revolutionized by advances in genomic medicine, molecular biology, stem cell biology and genetically engineered small-animal modelling. We now appreciate that paediatric brain tumours arise following specific genetic mutations in specialized...

journal_title：Neuropathology and applied neurobiology

authors： Thangarajh M,Gutmann DH

更新日期：2012-06-01 00:00:00

abstract：:The mechanisms underlying secondary or delayed cell death following traumatic brain injury (TBI) are poorly understood. Recent evidence from experimental models of TBI suggest that diffuse and widespread neuronal damage and loss is progressive and prolonged for months to years after the initial insult in selectively v...

journal_title：Neuropathology and applied neurobiology

authors： McIntosh TK,Saatman KE,Raghupathi R,Graham DI,Smith DH,Lee VM,Trojanowski JQ

更新日期：1998-08-01 00:00:00

abstract：AIMS:Apolipoprotein A-I (apoA-I), the principal apolipoprotein associated with high-density lipoproteins in the periphery, is also found at high concentrations in the cerebrospinal fluid. Previous studies have reported either no impact or vascular-specific effects of apoA-I knockout (KO) on β-amyloid (Aβ) pathology. Ho...

journal_title：Neuropathology and applied neurobiology

authors： Contu L,Carare RO,Hawkes CA

更新日期：2019-12-01 00:00:00

abstract：:The role of Schwann cells (SC) in perineurial regeneration after nerve injury has not yet been resolved. It was hypothesized that SC alone are able to induce at least partial morphological restoration of the destroyed orthotopic perineureum (PN). To test the hypothesis, a permanently denervated segment of the rat scia...

journal_title：Neuropathology and applied neurobiology

authors： Popović M,Bresjanac M,Sketelj J

更新日期：2000-06-01 00:00:00

abstract：AIMS:EphB2 is a member of receptor tyrosine kinases (RTKs) family that is essential for the cell adhesion, neural crest migration, axon guidance and synaptogenesis in the nervous system. Recent studies show that preservation of EphB2 in a transgenic mouse model of Alzheimer's disease (AD) rescues the cognitive deficit,...

journal_title：Neuropathology and applied neurobiology

authors： Qu M,Jiang J,Liu XP,Tian Q,Chen LM,Yin G,Liu D,Wang JZ,Zhu LQ

更新日期：2013-10-01 00:00:00

abstract：:The brain is vulnerable to a number of acute insults, with traumatic brain injury being among the commonest. Neuroinflammation is a common response to acute injury and microglial activation is a key component of the inflammatory response. In the acute and subacute phase it is likely that this response is protective an...

journal_title：Neuropathology and applied neurobiology

authors： Smith C

更新日期：2013-02-01 00:00:00