Review: Prion-like mechanisms of transactive response DNA binding protein of 43 kDa (TDP-43) in amyotrophic lateral sclerosis (ALS).

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:Wnt activation in medulloblastomas is associated with good outcome. Upfront testing and risk-adapted stratification of patients will be done in future clinical studies. In a cohort of 186 paediatric medulloblastomas our aim was to identify the optimal methods in standard clinical practice to detect this subgroup. ...

journal_title：Neuropathology and applied neurobiology

authors： Goschzik T,Zur Mühlen A,Kristiansen G,Haberler C,Stefanits H,Friedrich C,von Hoff K,Rutkowski S,Pfister SM,Pietsch T

更新日期：2015-02-01 00:00:00

abstract：:Cyclins are important regulators of the cell cycle; there is increasing evidence that some cyclins are positively involved in carcinogenesis. Amplification and translocation of the cyclin genes and overexpression of their mRNAs and proteins have been observed in a variety of tumours. We studied cyclin A protein in ast...

journal_title：Neuropathology and applied neurobiology

authors： Chakrabarty A,Bridges LR

更新日期：1998-06-01 00:00:00

abstract：:In a previous study, we described the inhibitory effects of the growth factor-antagonist, trapidil, on spontaneously occurring oligodendrocyte remyelination in the rat spinal cord following lysolecithin-induced demyelination [30]. The objective of the present study was to further investigate the mechanisms of trapidil...

journal_title：Neuropathology and applied neurobiology

authors： McKay JS,Blakemore WF,Franklin RJ

更新日期：1998-12-01 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：:Scrapie is a prion disease or transmissible spongiform encephalopathy (TSE) of sheep, goats and moufflon. As with its human counterparts, pathology consists of vacuolation, gliosis and accumulations of abnormal forms of a host prion protein (PrPd) in the brain of affected individuals. Immunohistochemical methods can b...

journal_title：Neuropathology and applied neurobiology

authors： Jeffrey M,González L

更新日期：2007-08-01 00:00:00

abstract：:Accumulating evidence indicates an important pathophysiological role of brain inflammation in epilepsy. In this review, we will provide an update of specific inflammatory pathways that have been proposed to be crucial in the underlying molecular mechanisms of epilepsy, including the interleukin-1 receptor/toll-like re...

journal_title：Neuropathology and applied neurobiology

authors： van Vliet EA,Aronica E,Vezzani A,Ravizza T

更新日期：2018-02-01 00:00:00

abstract：:In this study we describe the effects of trapidil, a putative platelet-derived growth factor-antagonist, on spontaneously occurring remyelination in rat spinal cord. Demyelination was created in the dorsal funiculus of 6- and 11-week-old female rats by the direct injection of 1.0 microliter of 1% lysolecithin. The ani...

journal_title：Neuropathology and applied neurobiology

authors： McKay JS,Blakemore WF,Franklin RJ

更新日期：1997-02-01 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：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：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：:Mitochondrial abnormalities, in particular the accumulation of mitochondrial DNA mutations, have been proposed as a potential cause of normal ageing. One group of patients with mtDNA disorders have a nuclear DNA defect which accelerates the chronological accumulation of mitochondrial DNA mutations. These patients prov...

journal_title：Neuropathology and applied neurobiology

authors： Cottrell DA,Ince PG,Wardell TM,Turnbull DM,Johnson MA

更新日期：2001-06-01 00:00:00

abstract：AIMS:Resident and peripherally derived glioma associated microglia/macrophages (GAMM) play a key role in driving tumour progression, angiogenesis, invasion and attenuating host immune responses. Differentiating these cells' origins is challenging and current preclinical models such as irradiation-based adoptive transfe...

journal_title：Neuropathology and applied neurobiology

authors： Yu K,Youshani AS,Wilkinson FL,O'Leary C,Cook P,Laaniste L,Liao A,Mosses D,Waugh C,Shorrock H,Pathmanaban O,Macdonald A,Kamaly-Asl I,Roncaroli F,Bigger BW

更新日期：2019-02-01 00:00:00

abstract：:Two cases of Aspergillus infection of the nervous system are reported. In the first case it occurred in a healthy individual and presented as a space occupying lesion mimicking a tuberculous granuloma. In the second case it was an opportunistic infection in a patient with predisposing conditions and manifested as chro...

journal_title：Neuropathology and applied neurobiology

authors： Diengdoh JV,Barnard RO,Thomas DG

更新日期：1983-11-01 00:00:00

abstract：AIMS:The deposition of amyloid peptides (A beta) in the cortex and hippocampus is the primary trigger of Alzheimer's disease (AD). Recent studies also indicated that the M2 subtype of muscarinic acetylcholine receptors (M2mAChR) may be a key molecule involved in cognitive dysfunction. Thus, the purpose of this study wa...

journal_title：Neuropathology and applied neurobiology

authors： González I,Arévalo-Serrano J,Pérez JL,Gonzalo P,Gonzalo-Ruiz A

更新日期：2008-10-01 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: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：: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：:Amyotrophic lateral sclerosis (ALS) is a fatal neurodegenerative disease characterized by loss of upper and lower motor neurones leading to muscle weakness and paralysis. Despite recent advances in the genetics of ALS, the mechanisms underlying motor neurone degeneration are not fully understood. Mitochondria are know...

journal_title：Neuropathology and applied neurobiology

authors： Duffy LM,Chapman AL,Shaw PJ,Grierson AJ

更新日期：2011-06-01 00:00:00

abstract：OBJECTIVE:To perform a systematic review and meta-analysis on the prevalence of transactive response DNA-binding protein 43 (TDP-43) proteinopathy in cognitively normal older adults. METHODS:We systematically reviewed and performed a meta-analysis on the prevalence of TDP-43 proteinopathy in older adults with normal c...

journal_title：Neuropathology and applied neurobiology

authors： Nascimento C,Di Lorenzo Alho AT,Bazan Conceição Amaral C,Leite REP,Nitrini R,Jacob-Filho W,Pasqualucci CA,Hokkanen SRK,Hunter S,Keage H,Kovacs GG,Grinberg LT,Suemoto CK

更新日期：2018-04-01 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：:The development of granule cells and the formation of synapses with mossy fibres were investigated in cerebella from jaundiced (j/j) Gunn rats with hereditary hyperbilirubinemia. The external granular layer was thinner than that in cerebella from heterozygous (+/j) Gunn rats without hyperbilirubinemia on days 12 and 1...

journal_title：Neuropathology and applied neurobiology

authors： Takagishi Y,Yamamura H

更新日期：1993-08-01 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：:Flow through the vasculature of an experimental rat glioma has been investigated during in situ perfusion of the brain, via the ascending aorta, with a simple saline solution. Using such a system, it has been shown previously that the blood-brain barrier will remain intact with an adequate cerebral perfusate flow rate...

journal_title：Neuropathology and applied neurobiology

authors： Luthert PJ,Greenwood J

更新日期：1994-10-01 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：: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：AIMS:Granulovacuolar degeneration involves the accumulation of large, double membrane-bound bodies within certain neurones during the course of Alzheimer's disease (AD) and other adult-onset dementias. Because of the two-layer membrane morphology, it has been proposed that the bodies are related to autophagic organelle...

journal_title：Neuropathology and applied neurobiology

authors： Funk KE,Mrak RE,Kuret J

更新日期：2011-04-01 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：: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：:The pathological findings in Alzheimer's disease (AD) are partly attributed to alterations in calcium-binding protein (CBP) functions. We showed previously that immunoreactivity of secretagogin, a recently cloned CBP, in the human hippocampus is restricted to pyramidal neurones and that the amount of immunoreactive ne...

journal_title：Neuropathology and applied neurobiology

authors： Attems J,Preusser M,Grosinger-Quass M,Wagner L,Lintner F,Jellinger K

更新日期：2008-02-01 00:00:00