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：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:Limb-girdle muscular dystrophy R9 (LGMDR9) is an autosomal recessive disorder caused by mutations in the fukutin-related protein gene (FKRP), encoding a glycosyltransferase involved in α-dystroglycan modification. Muscle atrophy, a significant feature of LGMDR9, occurs by a change in the normal balance between pro...

journal_title：Neuropathology and applied neurobiology

authors： Franekova V,Storjord HI,Leivseth G,Nilssen Ø

更新日期：2020-12-18 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: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：: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：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：AIM:To understand the progression of CLN1 disease and develop effective therapies we need to characterize early sites of pathology. Therefore, we performed a comprehensive evaluation of the nature and timing of early CLN1 disease pathology in the spinal cord, which appears especially vulnerable, and how this may affect...

journal_title：Neuropathology and applied neurobiology

authors： Nelvagal HR,Dearborn JT,Ostergaard JR,Sands MS,Cooper JD

更新日期：2020-08-25 00:00:00

abstract：AIMS:Brain mural cells (BMC), smooth muscle cells and pericytes, interact closely with endothelial cells and modulate numerous cerebrovascular functions. A loss of BMC function is suspected to play a role in the pathophysiology of Alzheimer's Disease (AD). METHODS:BMC markers, namely smooth muscle alpha actin (α-SMA) ...

journal_title：Neuropathology and applied neurobiology

authors： Bourassa P,Tremblay C,Schneider JA,Bennett DA,Calon F

更新日期：2020-08-01 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：:Paediatric diffuse high-grade gliomas (pHGG) are rare, but deadly tumours. The discovery of recurrent mutations in the tail of histone H3, changing lysine 27 to methionine, or glycine 34 to arginine or valine, has illuminated a critical role for epigenetic dysregulation in the aetiology of childhood gliomas and opened...

journal_title：Neuropathology and applied neurobiology

authors： Kasper LH,Baker SJ

更新日期：2020-02-01 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：AIMS:We observed a microvascular structure in the cerebral cortex that has not, to our knowledge, been previously described. We have termed the structure a 'raspberry', referring to its appearance under a bright-field microscope. We hypothesized that raspberries form through angiogenesis due to some form of brain ischa...

journal_title：Neuropathology and applied neurobiology

authors： Ek Olofsson H,Englund E

更新日期：2019-10-01 00:00:00

abstract：AIMS:Alzheimer's disease (AD) is characterized by degeneration of cholinergic basal forebrain (CBF) neurons in the nucleus basalis of Meynert (nbM), which provides the major cholinergic input to the cortical mantle and is related to cognitive decline in patients with AD. Cortical histone deacetylase (HDAC) dysregulatio...

journal_title：Neuropathology and applied neurobiology

authors： Mahady L,Nadeem M,Malek-Ahmadi M,Chen K,Perez SE,Mufson EJ

更新日期：2019-06-01 00:00:00

abstract：AIM:The insular cortex consists of a heterogenous cytoarchitecture and diverse connections and is thought to integrate autonomic, cognitive, emotional and interoceptive functions to guide behaviour. In Parkinson's disease (PD) and dementia with Lewy bodies (DLB), it reveals α-synuclein pathology in advanced stages. The...

journal_title：Neuropathology and applied neurobiology

authors： Fathy YY,Jonker AJ,Oudejans E,de Jong FJJ,van Dam AW,Rozemuller AJM,van de Berg WDJ

更新日期：2019-04-01 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：AIMS:Paediatric low-grade gliomas (pLGGs) are a heterogeneous group of brain tumours associated with a high overall survival: however, they are prone to recur and supratentorial lesions are difficult to resect, being associated with high percentage of disease recurrence. Our aim was to shed light on the biology of pLGG...

journal_title：Neuropathology and applied neurobiology

authors： Catanzaro G,Besharat ZM,Miele E,Chiacchiarini M,Po A,Carai A,Marras CE,Antonelli M,Badiali M,Raso A,Mascelli S,Schrimpf D,Stichel D,Tartaglia M,Capper D,von Deimling A,Giangaspero F,Mastronuzzi A,Locatelli F,Ferrett

更新日期：2018-12-01 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：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：:Over the last decade, there has been increasing evidence that hyperactivation of the mechanistic target of rapamycin (mTOR) pathway is a hallmark of malformations of cortical development such as focal cortical dysplasia (FCD) or hemimegalencephaly. The mTOR pathway governs protein and lipid synthesis, cell growth and ...

journal_title：Neuropathology and applied neurobiology

authors： Marsan E,Baulac S

更新日期：2018-02-01 00:00:00

abstract：AIMS:Glioblastomas are highly aggressive and treatment resistant. Increasing evidence suggests that tumour-associated macrophages/microglia (TAMs) facilitate tumour progression by acquiring a M2-like phenotype. Our objective was to investigate the prognostic value of TAMs in gliomas using automated quantitative double ...

journal_title：Neuropathology and applied neurobiology

authors： Sørensen MD,Dahlrot RH,Boldt HB,Hansen S,Kristensen BW

更新日期：2018-02-01 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：AIMS:Normal neurovascular coupling, mediated by the fine interplay and communication of cells within the neurovascular unit, is critical for maintaining normal brain activity and cognitive function. This study investigated whether, with advancing age there is disruption of neurovascular coupling and specific cellular c...

journal_title：Neuropathology and applied neurobiology

authors： Duncombe J,Lennen RJ,Jansen MA,Marshall I,Wardlaw JM,Horsburgh K

更新日期：2017-10-01 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：AIMS:Cytoplasmic accumulation of the nuclear protein transactive response DNA-binding protein 43 (TDP-43) is an early determinant of motor neuron degeneration in most amyotrophic lateral sclerosis (ALS) cases. We previously disclosed this accumulation in circulating lymphomonocytes (CLM) of ALS patients with mutant TAR...

journal_title：Neuropathology and applied neurobiology

authors： De Marco G,Lomartire A,Calvo A,Risso A,De Luca E,Mostert M,Mandrioli J,Caponnetto C,Borghero G,Manera U,Canosa A,Moglia C,Restagno G,Fini N,Tarella C,Giordana MT,Rinaudo MT,Chiò A

更新日期：2017-02-01 00:00:00

abstract：:Congenital myopathies are clinically and genetically a heterogeneous group of early onset neuromuscular disorders, characterized by hypotonia and muscle weakness. Clinical severity and age of onset are variable. Many patients are severely affected at birth while others have a milder, moderately progressive or nonprogr...

journal_title：Neuropathology and applied neurobiology

authors： Sewry CA,Wallgren-Pettersson C

更新日期：2017-02-01 00:00:00

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：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：AIM:Pilocytic astrocytomas represent the most common paediatric tumours of the central nervous system. Dissemination through the ventricular system occurs rarely in patients with pilocytic astrocytomas; however, it is more common in infants with diencephalic tumours, and is associated with a poor outcome. Despite histo...

journal_title：Neuropathology and applied neurobiology

authors： Gessi M,Engels AC,Lambert S,Rothämel T,von Hornstein S,Collins VP,Denkhaus D,Gnekow A,Pietsch T

更新日期：2016-04-01 00:00:00

abstract：AIMS:Oxidative damage and an associated DNA damage response (DDR) are evident in mild cognitive impairment and early Alzheimer's disease, suggesting that neuronal dysfunction resulting from oxidative DNA damage may account for some of the cognitive impairment not fully explained by Alzheimer-type pathology. METHODS:Fr...

journal_title：Neuropathology and applied neurobiology

authors： Simpson JE,Ince PG,Minett T,Matthews FE,Heath PR,Shaw PJ,Goodall E,Garwood CJ,Ratcliffe LE,Brayne C,Rattray M,Wharton SB,MRC Cognitive Function and Ageing Neuropathology Study Group.

更新日期：2016-02-01 00:00:00

abstract：AIMS:To explore alterations in the expression of genes encoding enzymes involved in purine metabolism in Parkinson's disease (PD) brains as purines are the core of the DNA, RNA, nucleosides and nucleotides which participate in a wide variety of crucial metabolic pathways. METHODS:Analysis of mRNA using real-time quant...

journal_title：Neuropathology and applied neurobiology

authors： Garcia-Esparcia P,Hernández-Ortega K,Ansoleaga B,Carmona M,Ferrer I

更新日期：2015-12-01 00:00:00

abstract：:Amyotrophic lateral sclerosis (ALS) is a fatal devastating neurodegenerative disorder which predominantly affects the motor neurons in the brain and spinal cord. The death of the motor neurons in ALS causes subsequent muscle atrophy, paralysis and eventual death. Clinical and biological evidence now demonstrates that ...

journal_title：Neuropathology and applied neurobiology

authors： Smethurst P,Sidle KC,Hardy J

更新日期：2015-08-01 00:00:00

abstract：AIMS:Frontotemporal lobar degeneration (FTLD) and motor neurone disease are linked by the possession of a hexanucleotide repeat expansion in C9ORF72, and both show neuronal cytoplasmic inclusions within cerebellar and hippocampal neurones which are TDP-43 negative but immunoreactive for p62 and dipeptide repeat protein...

journal_title：Neuropathology and applied neurobiology

authors： Baborie A,Griffiths TD,Jaros E,Perry R,McKeith IG,Burn DJ,Masuda-Suzukake M,Hasegawa M,Rollinson S,Pickering-Brown S,Robinson AC,Davidson YS,Mann DM

更新日期：2015-08-01 00:00:00

abstract：AIMS:Population-based studies have shown that approximately 20% of the ageing population (aged 65 years and over) with dementia have little or no classical Alzheimer-type neuropathology. Cumulative DNA damage and a reduced capacity of DNA repair may result in neuronal dysfunction and contribute to cognitive impairment ...

journal_title：Neuropathology and applied neurobiology

authors： Simpson JE,Ince PG,Matthews FE,Shaw PJ,Heath PR,Brayne C,Garwood C,Higginbottom A,Wharton SB,MRC Cognitive Function and Ageing Neuropathology Study Group.

更新日期：2015-06-01 00:00:00

abstract：AIMS:The serotonin 2A receptor (HTR2A) is widely expressed in the brain and involved in the modulation of fear, mood, anxiety and other symptoms. HTR2A and HTR2A gene variations are implicated in depression, schizophrenia, anxiety and obsessive-compulsive disorder. To understand HTR2A signalling changes in psychiatric ...

journal_title：Neuropathology and applied neurobiology

authors： Marinova Z,Monoranu CM,Fetz S,Walitza S,Grünblatt E

更新日期：2015-06-01 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：INTRODUCTION:Cerebral ischaemia is the defining pathophysiological abnormality in most forms of vascular dementia (VAD), but the pathogenesis of the dementia remains poorly understood. In Alzheimer's disease (AD), there is early loss of synaptic proteins, but these have been little studied in VAD. MATERIALS AND METHOD...

journal_title：Neuropathology and applied neurobiology

authors： Sinclair LI,Tayler HM,Love S

更新日期：2015-06-01 00:00:00

abstract：AIMS:Much evidence demonstrates that Toll-like receptor-4 (TLR4)-mediated microglial activation is an important contributor to the inflammatory injury in intracerebral haemorrhage (ICH). However, the exact mechanism of TLR4-mediated microglial activation induced by ICH is not clear. In addition, microglial autophagy is...

journal_title：Neuropathology and applied neurobiology

authors： Yang Z,Liu B,Zhong L,Shen H,Lin C,Lin L,Zhang N,Yuan B

更新日期：2015-06-01 00:00:00

abstract：AIMS:Insufficient oligodendroglial differentiation of oligodendroglial progenitor cells (OPCs) is suggested to be responsible for remyelination failure and astroglial scar formation in Theiler's murine encephalomyelitis (TME). The aim of the present study is to identify molecular key regulators of OPC differentiation i...

journal_title：Neuropathology and applied neurobiology

authors： Sun Y,Lehmbecker A,Kalkuhl A,Deschl U,Sun W,Rohn K,Tzvetanova ID,Nave KA,Baumgärtner W,Ulrich R

更新日期：2015-04-01 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