The HSPB8-BAG3 chaperone complex is upregulated in astrocytes in the human brain affected by protein aggregation diseases.

abstract：:The critical role of the neurofibromatosis 1 (NF1) gene as a tumour suppressor has been clearly demonstrated for malignancies arising in NF1 patients. However, little is known about the more common benign tumours, such as the pilocytic astrocytoma. Most NF1-associated astrocytomas are benign and clinically non-progres...

journal_title：Neuropathology and applied neurobiology

authors： Gutmann DH,Donahoe J,Brown T,James CD,Perry A

更新日期：2000-08-01 00:00:00

abstract：AIM:Levels of ubiquitin carboxyl-terminal hydrolase L1 (UCHL1) are robustly increased in spinal muscular atrophy (SMA) patient fibroblasts and mouse models. We therefore wanted to establish whether changes in UCHL1 contribute directly to disease pathogenesis, and to assess whether pharmacological inhibition of UCHL1 re...

journal_title：Neuropathology and applied neurobiology

authors： Powis RA,Mutsaers CA,Wishart TM,Hunter G,Wirth B,Gillingwater TH

更新日期：2014-12-01 00:00:00

abstract：AIMS:Amyloid-β (Aβ) oligomers trigger synaptic degeneration that precedes plaque and tangle pathology. However, the signalling molecules that link Aβ oligomers to synaptic pathology remain unclear. Here, we addressed the potential role of RAPGEF2 as a novel signalling molecule in Aβ oligomer-induced synaptic and cognit...

journal_title：Neuropathology and applied neurobiology

authors： Jang YN,Jang H,Kim GH,Noh JE,Chang KA,Lee KJ

更新日期：2020-12-20 00:00:00

abstract：AIMS:The secondary dystroglycanopathies represent a heterogeneous group of congenital muscular dystrophies characterized by the defective glycosylation of alpha dystroglycan. These disorders are associated with mutations in at least 17 genes, including Fukutin-related protein (FKRP). At the severe end of the clinical s...

journal_title：Neuropathology and applied neurobiology

authors： Booler HS,Pagalday-Vergara V,Williams JL,Hopkinson M,Brown SC

更新日期：2017-06-01 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：:Chicks were fed a diet rich in oxidized oil and deficient in alpha-tocopherol. As soon as nutritional encephalopathy developed the animals were immediately put on to a normal diet, and kept alive for up to 166 days. Examination of the cerebellum showed multiple foci of healed encephalomalacia with EM appearance of bas...

journal_title：Neuropathology and applied neurobiology

authors： Sandbank U,Bubis JJ,Bartov I,Dror I,Budowski P,Wolman M

更新日期：1980-01-01 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：: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：:Inflammatory cells from the meninges of guinea-pigs with acute EAE have been isolated and quantitated. A mean of 7.5 x 10(6) cells were recovered from the brains of animals showing clinical signs of disease in contrast to only 1.5 x 10(6) cells in the controls. The immunological specificity of the meningeal inflammato...

journal_title：Neuropathology and applied neurobiology

authors： Allsopp G,Parker D,Hinrichs DJ,Turk JL

更新日期：1981-03-01 00:00:00

abstract：:A single dose (19 mg kg-1) of triethyllead given to weanling rats produces necrosis in a small number of hippocampal pyramidal (CA3) and hilar neurons with reversible changes in the remaining neurons of this region. The sequence of events has been studied by light and electron microscopy over a period from 12 h to 14 ...

journal_title：Neuropathology and applied neurobiology

authors： Nolan CC,Brown AW

更新日期：1989-09-01 00:00:00

abstract：:In babies of low birth weight dying in the first week of life, bilirubin encephalopathy involving the thalamus only or the thalamus and one or two other areas occurred in nineteen of 376 cases examined over a 10 year period. Although coexistent germinal layer haemorrhage was present in sixteen, this was not thought to...

journal_title：Neuropathology and applied neurobiology

authors： Sherwood AJ,Smith JF

更新日期：1983-07-01 00:00:00

abstract：:We present the clinical and neuropathological findings of a patient with early onset Alzheimer's dementia (AD), heterozygous carrier of the rare Apolipoprotein E Christchurch (APOEch) variant. The patient did not harbor any pathogenic mutation in known Mendelian genes related to AD or other neurodegenerative disorders...

journal_title：Neuropathology and applied neurobiology

authors： Hernandez I,Gelpi E,Molina-Porcel L,Bernal S,Rodríguez-Santiago B,Dols-Icardo O,Ruiz A,Alcolea D,Boada M,Lleó A,Clarimón J

更新日期：2020-10-23 00:00:00

abstract：:Pathologists have been establishing archives of human organs and tissue for research use for many years now. Controversy has arisen recently over these collections, particularly with regard to the right of patients or relatives to consent to removal and retention of tissue, genetic research using stored tissue samples...

journal_title：Neuropathology and applied neurobiology

authors： Ashcroft R

更新日期：2000-10-01 00:00:00

abstract：AIMS:Infection of the mouse central nervous system with wild type (WT) and vaccine strains of measles virus (MV) results in lack of clinical signs and limited antigen detection. It is considered that cell entry receptors for these viruses are not present on murine neural cells and infection is restricted at cell entry....

journal_title：Neuropathology and applied neurobiology

authors： Abdullah H,Earle JA,Gardiner TA,Tangy F,Cosby SL

更新日期：2009-10-01 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：: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：:Multiple system atrophy (MSA) is an unrelenting, sporadic, adult-onset, neurodegenerative disease of unknown aetiology. Its clinically progressive course is characterized by a variable combination of parkinsonism, cerebellar ataxia and/or autonomic dysfunction. Neuropathological examination often reveals gross abnorma...

journal_title：Neuropathology and applied neurobiology

authors： Ahmed Z,Asi YT,Sailer A,Lees AJ,Houlden H,Revesz T,Holton JL

更新日期：2012-02-01 00:00:00

abstract：:The mechanism of the neuroprotective effect of hyperbaric oxygenation remains unclear although its clinical benefits have been well recognized for human ischaemic neuronal disease. The preventive effect of hyperbaric oxygenation against delayed neuronal death was investigated in the gerbil following transient forebrai...

journal_title：Neuropathology and applied neurobiology

authors： Konda A,Baba S,Iwaki T,Harai H,Koga H,Kimura T,Takamatsu J

更新日期：1996-08-01 00:00:00

abstract：:Serial sections of the brains of two cases with Alzheimer's disease were stained with the standard Bodian, modified Bielschowsky (reformed Gros-Schultze's modification) and thioflavin S methods. The numbers of demonstrated Alzheimer's neurofibrillary tangles (NFTs) were different between the two silver stains: from 15...

journal_title：Neuropathology and applied neurobiology

authors： Yamamoto T,Hirano A

更新日期：1986-01-01 00:00:00

abstract：:Changes of 5'-nucleotidase (5'-N) activity during subcultivation of C6 clonal rat glioma cells were studied histochemically, cytochemically and biochemically. Two different cell populations were seeded with high or low cell density respectively. 5'-N activity in cultured cells increased continuously in both population...

journal_title：Neuropathology and applied neurobiology

authors： Amano S,Sasahara M,Yamada E,Kataoka H,Hazama F

更新日期：1985-05-01 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：AIMS:The purpose of this study was to determine the functional recovery and protein regulation by transplanted induced pluripotent stem cells in a rat model of Huntington's disease (HD). METHODS:In a quinolinic acid-induced rat model of striatal degeneration, induced pluripotent stem cells were transplanted into the i...

journal_title：Neuropathology and applied neurobiology

authors： Mu S,Han L,Zhou G,Mo C,Duan J,He Z,Wang Z,Ren L,Zhang J

更新日期：2016-10-01 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：:Clinical, neuropathological and molecular genetic studies in a 9 month old boy with Pelizaeus-Merzbacher disease are described. The principal clinical features were developmental delay, nystagmus, stridor and seizures. Both brain and spinal cord showed almost complete absence of stainable central myelin, while cranial...

journal_title：Neuropathology and applied neurobiology

authors： Harding B,Ellis D,Malcolm S

更新日期：1995-04-01 00:00:00

abstract：AIMS:Adult neurogenesis is well described in the subventricular zone of the lateral ventricle walls and in the subgranular zone of the hippocampal dentate gyrus. However, recent studies indicate that self-renewal of neural stem cells (NSCs) is not restricted to these niches, but that diverse areas of the adult brain ar...

journal_title：Neuropathology and applied neurobiology

authors： Sanin V,Heeß C,Kretzschmar HA,Schüller U

更新日期：2013-08-01 00:00:00

abstract：:Using a combined biochemical and histological approach certain conclusions can be drawn as to the origin of the increase in lysosomal enzymes in white matter from MS brains. Firstly, there is a gradient of lysosomal enzyme activity, plaque greater than periplaque greater than macroscopically normal white matter, which...

journal_title：Neuropathology and applied neurobiology

authors： McKeown SR,Allen IV

更新日期：1978-11-01 00:00:00

abstract：:Immunohistochemistry using antibodies to dystrophin is the pathological basis for the diagnosis of Duchenne and Becker muscular dystrophy (DMD and BMD). While the sarcolemma of DMD muscle is negative, BMD muscle generally shows variable labelling because of the translation of a partially functional dystrophin that is ...

journal_title：Neuropathology and applied neurobiology

authors： Torelli S,Brown SC,Jimenez-Mallebrera C,Feng L,Muntoni F,Sewry CA

更新日期：2004-10-01 00:00:00

abstract：:Sarcoplasmic masses contain disorganized myofibrillar material and are a striking feature of myotonic dystrophy. However their significance is still unclear. Using immunocytochemistry we studied the expression of cytoskeletal proteins (desmin and vimentin), dystrophin, markers of myogenic differentiation (foetal myosi...

journal_title：Neuropathology and applied neurobiology

authors： Vattemi G,Tomelleri G,Filosto M,Savio C,Rizzuto N,Tonin P

更新日期：2005-02-01 00:00:00

abstract：:The cellular distribution of the lysosomal proteinase cathepsin D was studied in a series of 76 neoplasms and 18 non-neoplastic tissues from the human central nervous system, using a well-characterized polyclonal antibody in a peroxidase-antiperoxidase technique. In the normal and developing brain, cathepsin D is conf...

journal_title：Neuropathology and applied neurobiology

authors： Robson DK,Ironside JW,Reid WA,Bogue PR

更新日期：1990-02-01 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