Sequential studies of a childhood myopathy: a clinical, histochemical and morphometric investigation.

abstract：:Semliki Forest virus infections in mice produce an encephalitis with demyelination. If before giving the virus the mice are treated with muramyl dipeptide in Freund's incomplete adjuvant, there is a significant increase in demyelination. If ovalbumin is added to the above and then followed after an interval by a secon...

journal_title：Neuropathology and applied neurobiology

authors： Illavia SJ,Webb HE,Colover J

更新日期：1988-11-01 00:00:00

abstract：:The four GPI-anchored cell adhesion molecules that exemplify the IgLON family are most highly expressed in the nervous system and associate to form up to six different heterodimeric 'Diglons' that can modify cell adhesion and inhibit axon migration. Recently, two members, OPCML and LSAMP, were identified as putative t...

journal_title：Neuropathology and applied neurobiology

authors： Reed JE,Dunn JR,du Plessis DG,Shaw EJ,Reeves P,Gee AL,Warnke PC,Sellar GC,Moss DJ,Walker C

更新日期：2007-02-01 00:00:00

abstract：:The avirulent demyelinating strain A7(74) of Semliki Forest virus after passage through mouse brain in vivo and mouse brain cell cultures has been shown to react immunologically with immune sera against galactocerebroside, glucocerebroside, total ganglioside and GT1b ganglioside but not against myelin or sulphatide . ...

journal_title：Neuropathology and applied neurobiology

authors： Webb HE,Mehta S,Gregson NA,Leibowitz S

更新日期：1984-03-01 00:00:00

abstract：:Ultrastructural studies of spongiform encephalopathy (SE) reveal no very early pathological changes in kuru where membrane lamellation has been reported. This observation is challenged. In the later stages of SE, two main theories are examined--the spiroplasma theory and the prion (6 nm filament) theory. Neither are s...

journal_title：Neuropathology and applied neurobiology

authors： Gray EG

更新日期：1986-03-01 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：: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：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：: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：:Defects of mitochondrial function have been proposed as a potential mechanism in the development and pathogenesis of Alzheimer's disease (AD) and neuronal apoptosis. Mitochondrial enzyme-deficient pyramidal neurones are found in greater quantities in the hippocampus of AD patients than in age-matched controls. The pre...

journal_title：Neuropathology and applied neurobiology

authors： Cottrell DA,Borthwick GM,Johnson MA,Ince PG,Turnbull DM

更新日期：2002-10-01 00:00:00

abstract：:To characterize the distribution of apoptotic neurons and their relationships with the stage of disease, a history of HIV-dementia, and the degree of productive HIV infection, microglial activation and axonal damage, we examined the brains of 40 patients. Samples of frontal and temporal cortex, basal ganglia and brain...

journal_title：Neuropathology and applied neurobiology

authors： Adle-Biassette H,Chrétien F,Wingertsmann L,Héry C,Ereau T,Scaravilli F,Tardieu M,Gray F

更新日期：1999-04-01 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：:The causes of most neurological disorders are not fully understood. Inflammation and blood-brain barrier dysfunction appear to play major roles in the pathology of these diseases. Inflammatory insults that occur during brain development may have widespread effects later in life for a spectrum of neurological disorders...

journal_title：Neuropathology and applied neurobiology

authors： Stolp HB,Dziegielewska KM

更新日期：2009-04-01 00:00:00

abstract：:CADASIL (Cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy) is a type of small-artery stroke and vascular dementia-inducing pathology of the brain. In order to explain the molecular mechanisms behind the alterations to the blood vessels in CADASIL subjects, we scrutinized the e...

journal_title：Neuropathology and applied neurobiology

authors： Brulin-Fardoux P,Godfrain C,Maurage CA,De Reuck J,Hauw JJ,Kaltner H,Bovin NV,Gabius HJ,Ruchoux MM,Kiss R,Camby I

更新日期：2003-08-01 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：AIMS:Clusterin is a topologically dynamic chaperone protein with the ability to participate in both intra- and extacellular proteostasis. Clusterin has been shown to be upregulated in the spinal cord of patients with amyotrophic lateral sclerosis (ALS) and has been shown to protect against TDP-43 protein misfolding in ...

journal_title：Neuropathology and applied neurobiology

authors： Gregory JM,Elliott E,McDade K,Bak T,Pal S,Chandran S,Abrahams S,Smith C

更新日期：2020-04-01 00:00:00

abstract：:Transferase dUTP nick-end labelling (TUNEL) analysis was used to compare the occurrence of cell death in the cerebral wall of cocaine-exposed and drug-naïve monkey fetuses. The rhesus monkeys providing the drug-exposed fetuses received 10 mg/kg of cocaine orally (in fruit treats) in the morning and in the evening betw...

journal_title：Neuropathology and applied neurobiology

authors： He N,Song ZM,Lidow MS

更新日期：1999-12-01 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：: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：: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：AIMS:Although telomere length (TL) and telomere maintenance proteins (shelterins) are markers of cellular senescence and peripheral blood biomarkers of Alzheimer's disease (AD), little information is available on telomeric alterations during the prodromal stage (MCI) of AD. We investigated TL in the default mode networ...

journal_title：Neuropathology and applied neurobiology

authors： Mahady LJ,He B,Malek-Ahmadi M,Mufson EJ

更新日期：2020-10-26 00:00:00

abstract：:Sandhoff disease is a heritable lysosomal storage disease resulting from impaired degradation of GM2 ganglioside and related substrates. A mouse model of Sandhoff disease created by gene targeting displays progressive neurological manifestations, similar to patients with the disease. In the present in vivo and in vitr...

journal_title：Neuropathology and applied neurobiology

authors： Sango K,Yamanaka S,Ajiki K,Tokashiki A,Watabe K

更新日期：2002-02-01 00:00:00

abstract：:Cerebrospinal fluid (CSF) was taken from strain 13 guinea pigs in various stages of chronic relapsing experimental allergic encephalomyelitis, the spinal cords removed for histological examination and meningeal stretch preparations made. CSF cells were counted and characterized by morphological studies, anti-IgG and a...

journal_title：Neuropathology and applied neurobiology

authors： Suckling AJ,Wilson NR,Kirby JA,Rumsby MG

更新日期：1983-05-01 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：:Viliuisk encephalomyelitis (VE) is an unique neurological disease occurring in the Iakut (Sakha) people of Siberia. Evolution of the disease follows one of three broad clinical forms: subacute, slowly progressive or chronic. Death occurs within 3 to 6 months in subacute cases and within 6 years in the slowly progressi...

journal_title：Neuropathology and applied neurobiology

authors： McLean CA,Masters CL,Vladimirtsev VA,Prokhorova IA,Goldfarb LG,Asher DM,Vladimirtsev AI,Alekseev VP,Gajdusek DC

更新日期：1997-06-01 00:00:00

abstract：:Rats were given 2,5-hexanediol in their water for more than 5 weeks. The changes in the optic pathways were studied both qualitatively and quantitatively. Increase in 10-nm filaments within axons was noticeable from 10 days onwards in the superior colliculus and in the brachium of the superior colliculus. From then on...

journal_title：Neuropathology and applied neurobiology

authors： Jones HB,Cavanagh JB

更新日期：1982-07-01 00:00:00

abstract：:Studies in vitro have shown that phosphorylated translation initiation factor 2 alpha (TIF 2 alpha) may have several functions, including regulation of protein synthesis, control of cell death and procurement of resistance to oxidative stress in nerve cells. These properties may have implications in certain human neur...

journal_title：Neuropathology and applied neurobiology

authors： Ferrer I

更新日期：2002-12-01 00:00:00

abstract：:In many experimental models of skeletal muscle damage and in human muscle disease, empty basement membrane tubes remain following the destruction of muscle fibres. In the present study we test the hypothesis that the empty basement membrane tubes play an essential role in the orientation of regenerating muscle fibres....

journal_title：Neuropathology and applied neurobiology

authors： Caldwell CJ,Mattey DL,Weller RO

更新日期：1990-06-01 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：:Adhalin and alpha-dystroglycan are two components of a complex of proteins that, in conjunction with dystrophin, provide a link between the subsarcolemmal cytoskeleton and the basal lamina of the extracellular matrix of skeletal muscle. In the absence of dystrophin, in Duchenne muscular dystrophy (DMD) and the mdx mou...

journal_title：Neuropathology and applied neurobiology

authors： Cullen MJ,Walsh J,Roberds SL,Campbell KP

更新日期：1996-02-01 00:00:00