abstract：:Accumulation of the tau protein in fibrillar intracellular aggregates is a defining feature of multiple neurodegenerative diseases collectively referred to as tauopathies. Despite intensive study of tau, there is limited information on the formation and clearance dynamics of tau inclusions. Using rAAV vectors to media...

journal_title：Acta neuropathologica

authors： Croft CL,Goodwin MS,Ryu DH,Lessard CB,Tejeda G,Marrero M,Vause AR,Paterno G,Cruz PE,Lewis J,Giasson BI,Golde TE

更新日期：2021-01-26 00:00:00

abstract：:Granulovacuolar degeneration (GVD) is a common feature in Alzheimer's disease (AD). The occurrence of GVD is closely associated with that of neurofibrillary tangles (NFTs) and GVD is even considered to be a pre-NFT stage in the disease process of AD. Currently, the composition of GVD bodies, the mechanisms associated ...

journal_title：Acta neuropathologica

authors： Hondius DC,Koopmans F,Leistner C,Pita-Illobre D,Peferoen-Baert RM,Marbus F,Paliukhovich I,Li KW,Rozemuller AJM,Hoozemans JJM,Smit AB

更新日期：2021-01-25 00:00:00

abstract：:Mutations in the sarcomeric protein titin, encoded by TTN, are emerging as a common cause of myopathies. The diagnosis of a TTN-related myopathy is, however, often not straightforward due to clinico-pathological overlap with other myopathies and the prevalence of TTN variants in control populations. Here, we present a...

journal_title：Acta neuropathologica

authors： Rees M,Nikoopour R,Fukuzawa A,Kho AL,Fernandez-Garcia MA,Wraige E,Bodi I,Deshpande C,Özdemir Ö,Daimagüler HS,Pfuhl M,Holt M,Brandmeier B,Grover S,Fluss J,Longman C,Farrugia ME,Matthews E,Hanna M,Muntoni F,Sarkozy

更新日期：2021-01-15 00:00:00

abstract：:Alpha-synuclein (αSyn) preformed fibrils (PFF) induce endogenous αSyn aggregation leading to reduced synaptic transmission. Neuronal activity modulates release of αSyn; however, whether neuronal activity regulates the spreading of αSyn pathology remains elusive. Here, we established a hippocampal slice culture system ...

journal_title：Acta neuropathologica

authors： Wu Q,Shaikh MA,Meymand ES,Zhang B,Luk KC,Trojanowski JQ,Lee VM

更新日期：2020-12-01 00:00:00

abstract：:Mislocalization and abnormal deposition of TDP-43 into the cytoplasm (TDP-43 proteinopathy) is a hallmark in neurons of amyotrophic lateral sclerosis (ALS) and frontotemporal lobar degeneration (FTLD). However, the pathogenic mechanism of the diseases linked to TDP-43 is largely unknown. We hypothesized that the failu...

journal_title：Acta neuropathologica

authors： Nagano S,Jinno J,Abdelhamid RF,Jin Y,Shibata M,Watanabe S,Hirokawa S,Nishizawa M,Sakimura K,Onodera O,Okada H,Okada T,Saito Y,Takahashi-Fujigasaki J,Murayama S,Wakatsuki S,Mochizuki H,Araki T

更新日期：2020-11-01 00:00:00

abstract：:Multiple sclerosis (MS) is the most frequent demyelinating disease in young adults and despite significant advances in immunotherapy, disease progression still cannot be prevented. Promotion of remyelination, an endogenous repair mechanism resulting in the formation of new myelin sheaths around demyelinated axons, rep...

journal_title：Acta neuropathologica

authors： Starost L,Lindner M,Herold M,Xu YKT,Drexler HCA,Heß K,Ehrlich M,Ottoboni L,Ruffini F,Stehling M,Röpke A,Thomas C,Schöler HR,Antel J,Winkler J,Martino G,Klotz L,Kuhlmann T

更新日期：2020-11-01 00:00:00

abstract：:MicroRNAs are recognized as important regulators of many facets of physiological brain function while also being implicated in the pathogenesis of several neurological disorders. Dysregulation of miR155 is widely reported across a variety of neurodegenerative conditions, including Alzheimer's disease (AD), Parkinson's...

journal_title：Acta neuropathologica

authors： Readhead B,Haure-Mirande JV,Mastroeni D,Audrain M,Fanutza T,Kim SH,Blitzer RD,Gandy S,Dudley JT,Ehrlich ME

更新日期：2020-09-01 00:00:00

abstract：:Sporadic Creutzfeldt-Jakob disease (sCJD) is a transmissible brain proteinopathy. Five main clinicopathological subtypes (sCJD-MM(V)1, -MM(V)2C, -MV2K, -VV1, and -VV2) are currently distinguished. Histopathological evidence suggests that the localisation of prion aggregates and spongiform lesions varies among subtypes...

journal_title：Acta neuropathologica

authors： Pascuzzo R,Oxtoby NP,Young AL,Blevins J,Castelli G,Garbarino S,Cohen ML,Schonberger LB,Gambetti P,Appleby BS,Alexander DC,Bizzi A

更新日期：2020-08-01 00:00:00

abstract：:Progressive supranuclear palsy (PSP) is a 4R-tauopathy predominated by subcortical pathology in neurons, astrocytes, and oligodendroglia associated with various clinical phenotypes. In the present international study, we addressed the question of whether or not sequential distribution patterns can be recognized for PS...

journal_title：Acta neuropathologica

authors： Kovacs GG,Lukic MJ,Irwin DJ,Arzberger T,Respondek G,Lee EB,Coughlin D,Giese A,Grossman M,Kurz C,McMillan CT,Gelpi E,Compta Y,van Swieten JC,Laat LD,Troakes C,Al-Sarraj S,Robinson JL,Roeber S,Xie SX,Lee VM,Trojan

更新日期：2020-08-01 00:00:00

abstract：:Brain tumors are the most common solid tumors of childhood, and the genetic drivers and optimal therapeutic strategies for many of the different subtypes remain unknown. Here, we identify that bithalamic gliomas harbor frequent mutations in the EGFR oncogene, only rare histone H3 mutation (in contrast to their unilate...

journal_title：Acta neuropathologica

authors： Mondal G,Lee JC,Ravindranathan A,Villanueva-Meyer JE,Tran QT,Allen SJ,Barreto J,Gupta R,Doo P,Van Ziffle J,Onodera C,Devine P,Grenert JP,Samuel D,Li R,Metrock LK,Jin LW,Antony R,Alashari M,Cheshier S,Whipple NS,

更新日期：2020-06-01 00:00:00

abstract：:Synucleinopathies, such as Parkinson's disease (PD), multiple system atrophy (MSA), and dementia with Lewy bodies (DLB), are defined by the presence of α-synuclein (αSYN) aggregates throughout the nervous system but diverge from one another with regard to their clinical and pathological phenotype. The recent generatio...

journal_title：Acta neuropathologica

authors： Van der Perren A,Gelders G,Fenyi A,Bousset L,Brito F,Peelaerts W,Van den Haute C,Gentleman S,Melki R,Baekelandt V

更新日期：2020-06-01 00:00:00

abstract：:In amyotrophic lateral sclerosis (ALS) and spinal muscular atrophy (SMA), spinal and lower brainstem motor neurons degenerate, but some motor neuron subtypes are spared, including oculomotor neurons (OMNs). The mechanisms responsible for this selective degeneration are largely unknown, but the molecular signatures of ...

journal_title：Acta neuropathologica

authors： Nizzardo M,Taiana M,Rizzo F,Aguila Benitez J,Nijssen J,Allodi I,Melzi V,Bresolin N,Comi GP,Hedlund E,Corti S

更新日期：2020-05-01 00:00:00

abstract：:DICER1 syndrome is a rare tumor predisposition syndrome with manifestations that predominantly affect children and young adults. The syndrome is typically caused by heterozygous germline loss-of-function DICER1 alterations accompanied on the other allele by somatic missense mutations occurring at one of a few mutation...

journal_title：Acta neuropathologica

authors： de Kock L,Priest JR,Foulkes WD,Alexandrescu S

更新日期：2020-04-01 00:00:00

abstract：:Parkinson's disease (PD) is the most common neurodegenerative movement disorder and is characterized by the progressive loss of dopaminergic (DA) neurons in the substantia nigra pars compacta (SNc) and the gradual appearance of α-synuclein (α-syn)-containing neuronal protein aggregates. Although the exact mechanism of...

journal_title：Acta neuropathologica

authors： Tan Y,Sgobio C,Arzberger T,Machleid F,Tang Q,Findeis E,Tost J,Chakroun T,Gao P,Höllerhage M,Bötzel K,Herms J,Höglinger G,Koeglsperger T

更新日期：2020-02-01 00:00:00

abstract：:The original version of this article unfortunately contained a typesetting error in Fig 3c. The corrected Fig. 3 is given in the following page. ...

journal_title：Acta neuropathologica

authors： Liu APY,Gudenas B,Lin T,Orr BA,Klimo P Jr,Kumar R,Bouffet E,Gururangan S,Crawford JR,Kellie SJ,Chintagumpala M,Fisher MJ,Bowers DC,Hassall T,Indelicato DJ,Onar-Thomas A,Ellison DW,Boop FA,Merchant TE,Robinson GW,N

更新日期：2020-02-01 00:00:00

abstract：:In search of novel genes associated with glioma pathogenesis, we have previously shown frequent deletions of the KIAA1797/FOCAD gene in malignant gliomas, and a tumor suppressor function of the encoded focadhesin impacting proliferation and migration of glioma cells in vitro and in vivo. Here, we examined an associati...

journal_title：Acta neuropathologica

authors： Brand F,Förster A,Christians A,Bucher M,Thomé CM,Raab MS,Westphal M,Pietsch T,von Deimling A,Reifenberger G,Claus P,Hentschel B,Weller M,Weber RG

更新日期：2020-01-01 00:00:00

abstract：:The original version of this article unfortunately contained a mistake. The Panel A in the published figure 5 is incorrect. The corrected Figure 5 is placed in the following page. ...

journal_title：Acta neuropathologica

authors： Saijo E,Metrick MA 2nd,Koga S,Parchi P,Litvan I,Spina S,Boxer A,Rojas JC,Galasko D,Kraus A,Rossi M,Newell K,Zanusso G,Grinberg LT,Seeley WW,Ghetti B,Dickson DW,Caughey B

更新日期：2020-01-01 00:00:00

abstract：:Extracellular deposition of amyloid β-protein (Aβ) in amyloid plaques and intracellular accumulation of abnormally phosphorylated τ-protein (p-τ) in neurofibrillary tangles (NFTs) represent pathological hallmark lesions of Alzheimer's disease (AD). Both lesions develop in parallel in the human brain throughout the pre...

journal_title：Acta neuropathologica

authors： Gomes LA,Hipp SA,Rijal Upadhaya A,Balakrishnan K,Ospitalieri S,Koper MJ,Largo-Barrientos P,Uytterhoeven V,Reichwald J,Rabe S,Vandenberghe R,von Arnim CAF,Tousseyn T,Feederle R,Giudici C,Willem M,Staufenbiel M,Thal DR

更新日期：2019-12-01 00:00:00

abstract：:Multiple system atrophy (MSA) is characterized by the presence of distinctive glial cytoplasmic inclusions (GCIs) within oligodendrocytes that contain the neuronal protein alpha-synuclein (aSyn) and the oligodendroglia-specific phosphoprotein TPPP/p25α. However, the role of oligodendroglial aSyn and p25α in the format...

journal_title：Acta neuropathologica

authors： Mavroeidi P,Arvanitaki F,Karakitsou AK,Vetsi M,Kloukina I,Zweckstetter M,Giller K,Becker S,Sorrentino ZA,Giasson BI,Jensen PH,Stefanis L,Xilouri M

更新日期：2019-09-01 00:00:00

abstract：:In 2011, genome-wide association studies implicated a polymorphism near CD33 as a genetic risk factor for Alzheimer's disease. This finding sparked interest in this member of the sialic acid-binding immunoglobulin-type lectin family which is linked to innate immunity. Subsequent studies found that CD33 is expressed in...

journal_title：Acta neuropathologica

authors： Estus S,Shaw BC,Devanney N,Katsumata Y,Press EE,Fardo DW

更新日期：2019-08-01 00:00:00

abstract：:Amyotrophic lateral sclerosis (ALS) is a lethal disease characterized by motor neuron degeneration and associated with aggregation of nuclear RNA-binding proteins (RBPs), including FUS. How FUS aggregation and neurodegeneration are prevented in healthy motor neurons remain critically unanswered questions. Here, we use...

journal_title：Acta neuropathologica

authors： Marrone L,Drexler HCA,Wang J,Tripathi P,Distler T,Heisterkamp P,Anderson EN,Kour S,Moraiti A,Maharana S,Bhatnagar R,Belgard TG,Tripathy V,Kalmbach N,Hosseinzadeh Z,Crippa V,Abo-Rady M,Wegner F,Poletti A,Troost D,A

更新日期：2019-07-01 00:00:00

abstract：:Pelizaeus-Merzbacher disease (PMD) is an untreatable and fatal leukodystrophy. In a model of PMD with perturbed blood-brain barrier integrity, cholesterol supplementation promotes myelin membrane growth. Here, we show that in contrast to the mouse model, dietary cholesterol in two PMD patients did not lead to a major ...

journal_title：Acta neuropathologica

authors： Stumpf SK,Berghoff SA,Trevisiol A,Spieth L,Düking T,Schneider LV,Schlaphoff L,Dreha-Kulaczewski S,Bley A,Burfeind D,Kusch K,Mitkovski M,Ruhwedel T,Guder P,Röhse H,Denecke J,Gärtner J,Möbius W,Nave KA,Saher G

更新日期：2019-07-01 00:00:00

abstract：:Virtually all phases of spinal cord injury pathogenesis, including inflammation, cell proliferation and differentiation, as well as tissue remodeling, are mediated in part by infiltrating monocyte-derived macrophages. It is now clear that these infiltrating macrophages have distinct functions from resident microglia a...

journal_title：Acta neuropathologica

authors： Milich LM,Ryan CB,Lee JK

更新日期：2019-05-01 00:00:00

abstract：:It is well established that the binding of pathogenic aquaporin-4 (AQP4)-specific autoantibodies to astrocytes may initiate a cascade of events culminating in the destruction of these cells and in the formation of large tissue-destructive lesions typical for patients with neuromyelitis optica spectrum disorders (NMOSD...

journal_title：Acta neuropathologica

authors： Hillebrand S,Schanda K,Nigritinou M,Tsymala I,Böhm D,Peschl P,Takai Y,Fujihara K,Nakashima I,Misu T,Reindl M,Lassmann H,Bradl M

更新日期：2019-03-01 00:00:00

abstract：:In Alzheimer's disease (AD) and other tauopathies, the cytosolic protein Tau misfolds and forms intracellular aggregates which accumulate within the brain leading to neurodegeneration. Clinical progression is tightly linked to the progressive spread of Tau pathology throughout the brain, and several lines of evidence ...

journal_title：Acta neuropathologica

authors： Courade JP,Angers R,Mairet-Coello G,Pacico N,Tyson K,Lightwood D,Munro R,McMillan D,Griffin R,Baker T,Starkie D,Nan R,Westwood M,Mushikiwabo ML,Jung S,Odede G,Sweeney B,Popplewell A,Burgess G,Downey P,Citron M

更新日期：2018-11-01 00:00:00

abstract：:Nodding syndrome is an epidemic neurologic disorder of unknown cause that affects children in the subsistence-farming communities of East Africa. We report the neuropathologic findings in five fatal cases (13-18 years of age at death) of nodding syndrome from the Acholi people in northern Uganda. Neuropathologic exami...

journal_title：Acta neuropathologica

authors： Pollanen MS,Onzivua S,Robertson J,McKeever PM,Olawa F,Kitara DL,Fong A

更新日期：2018-11-01 00:00:00

abstract：:α-Synuclein (αSyn) histopathology defines several neurodegenerative disorders, including Parkinson's disease, Lewy body dementia, and Alzheimer's disease (AD). However, the functional link between soluble αSyn and disease etiology remains elusive, especially in AD. We, therefore, genetically targeted αSyn in APP trans...

journal_title：Acta neuropathologica

authors： Khan SS,LaCroix M,Boyle G,Sherman MA,Brown JL,Amar F,Aldaco J,Lee MK,Bloom GS,Lesné SE

更新日期：2018-10-01 00:00:00

abstract：:Neurotropic herpesviruses can establish lifelong infection in humans and contribute to severe diseases including encephalitis and neurodegeneration. However, the mechanisms through which the brain's immune system recognizes and controls viral infections propagating across synaptically linked neuronal circuits have rem...

journal_title：Acta neuropathologica

authors： Fekete R,Cserép C,Lénárt N,Tóth K,Orsolits B,Martinecz B,Méhes E,Szabó B,Németh V,Gönci B,Sperlágh B,Boldogkői Z,Kittel Á,Baranyi M,Ferenczi S,Kovács K,Szalay G,Rózsa B,Webb C,Kovacs GG,Hortobágyi T,West BL,Kö

更新日期：2018-09-01 00:00:00

abstract：:Recently, we described a machine learning approach for classification of central nervous system tumors based on the analysis of genome-wide DNA methylation patterns [6]. Here, we report on DNA methylation-based central nervous system (CNS) tumor diagnostics conducted in our institution between the years 2015 and 2018....

journal_title：Acta neuropathologica

authors： Capper D,Stichel D,Sahm F,Jones DTW,Schrimpf D,Sill M,Schmid S,Hovestadt V,Reuss DE,Koelsche C,Reinhardt A,Wefers AK,Huang K,Sievers P,Ebrahimi A,Schöler A,Teichmann D,Koch A,Hänggi D,Unterberg A,Platten M,Wick

更新日期：2018-08-01 00:00:00

abstract：:Cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL) and a phenotypically similar recessive condition (CARASIL) have emerged as important genetic model diseases for studying the molecular pathomechanisms of cerebral small vessel disease (SVD). CADASIL, the most frequent ...

journal_title：Acta neuropathologica

authors： Zellner A,Scharrer E,Arzberger T,Oka C,Domenga-Denier V,Joutel A,Lichtenthaler SF,Müller SA,Dichgans M,Haffner C

更新日期：2018-07-01 00:00:00

abstract：:The peripheral immune system plays a critical role in aging and in the response to brain injury. Emerging data suggest inflammatory responses are exacerbated in older animals following ischemic stroke; however, our understanding of these age-related changes is poor. In this work, we demonstrate marked differences in t...

journal_title：Acta neuropathologica

authors： Ritzel RM,Lai YJ,Crapser JD,Patel AR,Schrecengost A,Grenier JM,Mancini NS,Patrizz A,Jellison ER,Morales-Scheihing D,Venna VR,Kofler JK,Liu F,Verma R,McCullough LD

更新日期：2018-07-01 00:00:00

abstract：:The accumulation of misfolded α-synuclein (aSyn) and neuron loss define several neurodegenerative disorders including Parkinson's disease (PD) and dementia with Lewy bodies (DLB). However, the precise relationship between pathology and neurotoxicity and why these processes disproportionately affect certain neuron subp...

journal_title：Acta neuropathologica

authors： Luna E,Decker SC,Riddle DM,Caputo A,Zhang B,Cole T,Caswell C,Xie SX,Lee VMY,Luk KC

更新日期：2018-06-01 00:00:00

abstract：:Amyotrophic lateral sclerosis (ALS) is a devastating neurodegenerative disease with no effective treatments. Numerous RNA-binding proteins (RBPs) have been shown to be altered in ALS, with mutations in 11 RBPs causing familial forms of the disease, and 6 more RBPs showing abnormal expression/distribution in ALS albeit...

journal_title：Acta neuropathologica

authors： Bakkar N,Kovalik T,Lorenzini I,Spangler S,Lacoste A,Sponaugle K,Ferrante P,Argentinis E,Sattler R,Bowser R

更新日期：2018-02-01 00:00:00

abstract：:In addition to motor neurone degeneration, up to 50% of amyotrophic lateral sclerosis (ALS) patients present with cognitive decline. Understanding the neurobiological changes underlying these cognitive deficits is critical, as cognitively impaired patients exhibit a shorter survival time from symptom onset. Given the ...

journal_title：Acta neuropathologica

authors： Henstridge CM,Sideris DI,Carroll E,Rotariu S,Salomonsson S,Tzioras M,McKenzie CA,Smith C,von Arnim CAF,Ludolph AC,Lulé D,Leighton D,Warner J,Cleary E,Newton J,Swingler R,Chandran S,Gillingwater TH,Abrahams S,Spires-

更新日期：2018-02-01 00:00:00

abstract：:The essential role of the cellular prion protein (PrPC) in prion disorders such as Creutzfeldt-Jakob disease is well documented. Moreover, evidence is accumulating that PrPC may act as a receptor for protein aggregates and transduce neurotoxic signals in more common neurodegenerative disorders, such as Alzheimer's dis...

journal_title：Acta neuropathologica

authors： Watts JC,Bourkas MEC,Arshad H

更新日期：2018-02-01 00:00:00

abstract：:Mutations of isocitrate dehydrogenase 1 (IDH1) gene are most common in glioma, arguably preceding all known genetic alterations during tumor development. IDH1 mutations nearly invariably target the enzymatic active site Arg132, giving rise to the predominant IDH1R132H. Cells harboring IDH1 R132H -heterozygous mutation...

journal_title：Acta neuropathologica

authors： Tiburcio PDB,Xiao B,Berg S,Asper S,Lyne S,Zhang Y,Zhu X,Yan H,Huang LE

更新日期：2018-02-01 00:00:00

abstract：:Conventional genetic approaches and computational strategies have converged on immune-inflammatory pathways as key events in the pathogenesis of late onset sporadic Alzheimer's disease (LOAD). Mutations and/or differential expression of microglial specific receptors such as TREM2, CD33, and CR3 have been associated wi...

journal_title：Acta neuropathologica

authors： Haure-Mirande JV,Audrain M,Fanutza T,Kim SH,Klein WL,Glabe C,Readhead B,Dudley JT,Blitzer RD,Wang M,Zhang B,Schadt EE,Gandy S,Ehrlich ME

更新日期：2017-11-01 00:00:00

abstract：:Frontotemporal lobar degeneration with TDP-43 inclusions (FTLD-TDP) can typically be categorized into one of four distinct histopathologic patterns of TDP-43 pathology, types A to D. The strength of this histopathologic classification lies in the association between FTLD-TDP subtypes and various clinical and genetic f...

journal_title：Acta neuropathologica

authors： Lee EB,Porta S,Michael Baer G,Xu Y,Suh E,Kwong LK,Elman L,Grossman M,Lee VM,Irwin DJ,Van Deerlin VM,Trojanowski JQ

更新日期：2017-07-01 00:00:00

abstract：:The differential diagnosis of Creutzfeldt-Jakob disease (CJD) from other, sometimes treatable, neurological disorders is challenging, owing to the wide phenotypic heterogeneity of the disease. Real-time quaking-induced prion conversion (RT-QuIC) is a novel ultrasensitive in vitro assay, which, at variance with surroga...

journal_title：Acta neuropathologica

authors： Lattanzio F,Abu-Rumeileh S,Franceschini A,Kai H,Amore G,Poggiolini I,Rossi M,Baiardi S,McGuire L,Ladogana A,Pocchiari M,Green A,Capellari S,Parchi P

更新日期：2017-04-01 00:00:00

abstract：:Chronic traumatic encephalopathy (CTE) is a neurodegenerative disease associated with repetitive mild impact traumatic brain injury from contact sports. Recently, a consensus panel defined the pathognomonic lesion for CTE as accumulations of abnormally hyperphosphorylated tau (p-tau) in neurons (neurofibrillary tangle...

journal_title：Acta neuropathologica

authors： Shively SB,Edgerton SL,Iacono D,Purohit DP,Qu BX,Haroutunian V,Davis KL,Diaz-Arrastia R,Perl DP

更新日期：2017-03-01 00:00:00