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Acta Neuropathologica Communications

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https://www.readbyqxmd.com/read/29747701/astrocyte-remodeling-without-gliosis-precedes-optic-nerve-axonopathy
#1
Melissa L Cooper, John W Collyer, David J Calkins
Astroyctes serve myriad functions but are especially critical in white matter tracts, where energy-demanding axons propagate action potentials great distances between neurons. Axonal dependence on astrocytes for even normal function accentuates the critical role astrocytes serve during disease. In glaucoma, the most common optic neuropathy, sensitivity to intraocular pressure (IOP) challenges RGC axons early, including degradation of anterograde transport to the superior colliculus (SC). Astrocyte remodeling presages overt axon degeneration in glaucoma and thus may present a therapeutic opportunity...
May 10, 2018: Acta Neuropathologica Communications
https://www.readbyqxmd.com/read/29747690/a53t-%C3%AE-synuclein-overexpression-in-murine-locus-coeruleus-induces-parkinson-s-disease-like-pathology-in-neurons-and-glia
#2
Martin Timo Henrich, Fanni Fruzsina Geibl, Bolam Lee, Wei-Hua Chiu, James Benjamin Koprich, Jonathan Michael Brotchie, Lars Timmermann, Niels Decher, Lina Anita Matschke, Wolfgang Hermann Oertel
Degeneration of noradrenergic locus coeruleus neurons occurs during the prodromal phase of Parkinson's disease and contributes to a variety of non-motor symptoms, e.g. depression, anxiety and REM sleep behavior disorder. This study was designed to establish the first locus coeruleus α-synucleinopathy mouse model, which should provide sufficient information about the time-course of noradrenergic neurodegeneration, replicate cardinal histopathological features of the human Parkinson's disease neuropathology and finally lead to robust histological markers, which are sufficient to assess the pathological changes in a quantitative and qualitative way...
May 10, 2018: Acta Neuropathologica Communications
https://www.readbyqxmd.com/read/29730992/a-new-tao-kinase-inhibitor-reduces-tau-phosphorylation-at-sites-associated-with-neurodegeneration-in-human-tauopathies
#3
Caterina Giacomini, Chuay-Yeng Koo, Natalia Yankova, Ignatius A Tavares, Selina Wray, Wendy Noble, Diane P Hanger, Jonathan D H Morris
In Alzheimer's disease (AD) and related tauopathies, the microtubule-associated protein tau is highly phosphorylated and aggregates to form neurofibrillary tangles that are characteristic of these neurodegenerative diseases. Our previous work has demonstrated that the thousand-and-one amino acid kinases (TAOKs) 1 and 2 phosphorylate tau on more than 40 residues in vitro. Here we show that TAOKs are phosphorylated and active in AD brain sections displaying mild (Braak stage II), intermediate (Braak stage IV) and advanced (Braak stage VI) tau pathology and that active TAOKs co-localise with both pre-tangle and tangle structures...
May 7, 2018: Acta Neuropathologica Communications
https://www.readbyqxmd.com/read/29724241/systemic-inhibition-of-the-membrane-attack-complex-impedes-neuroinflammation-in-chronic-relapsing-experimental-autoimmune-encephalomyelitis
#4
Iliana Michailidou, Aldo Jongejan, Jeroen P Vreijling, Theodosia Georgakopoulou, Marit B de Wissel, Ruud A Wolterman, Patrick Ruizendaal, Ngaisah Klar-Mohamad, Anita E Grootemaat, Daisy I Picavet, Vinod Kumar, Cees van Kooten, Trent M Woodruff, B Paul Morgan, Nicole N van der Wel, Valeria Ramaglia, Kees Fluiter, Frank Baas
The complement system is a key driver of neuroinflammation. Activation of complement by all pathways, results in the formation of the anaphylatoxin C5a and the membrane attack complex (MAC). Both initiate pro-inflammatory responses which can contribute to neurological disease. In this study, we delineate the specific roles of C5a receptor signaling and MAC formation during the progression of experimental autoimmune encephalomyelitis (EAE)-mediated neuroinflammation. MAC inhibition was achieved by subcutaneous administration of an antisense oligonucleotide specifically targeting murine C6 mRNA (5 mg/kg)...
May 3, 2018: Acta Neuropathologica Communications
https://www.readbyqxmd.com/read/29716656/large-inter-and-intra-case-variability-of-first-generation-tau-pet-ligand-binding-in-neurodegenerative-dementias
#5
Melissa C Wren, Tammaryn Lashley, Erik Årstad, Kerstin Sander
Imaging of pathological tau with positron emission tomography (PET) has the potential to allow early diagnosis of the dementias and monitoring of disease progression, including assessment of therapeutic interventions, in vivo. The first generation of tau PET tracers, including the carbazole flortaucipir and the 2-arylquinolines of the THK series, are now used in clinical research; however, concerns have been raised about off-target binding and low sensitivity.With the aim to determine the nature of tau pathology depicted by structurally distinct tau ligands we carried out a microscopic neuropathological evaluation in post-mortem human brain tissue of cases with primary and secondary tauopathies...
May 1, 2018: Acta Neuropathologica Communications
https://www.readbyqxmd.com/read/29716652/%C3%AE-synuclein-fibril-induced-paradoxical-structural-and-functional-defects-in-hippocampal-neurons
#6
Jessica M Froula, Benjamin W Henderson, Jose Carlos Gonzalez, Jada H Vaden, John W Mclean, Yumei Wu, Gokulakrishna Banumurthy, Linda Overstreet-Wadiche, Jeremy H Herskowitz, Laura A Volpicelli-Daley
Neuronal inclusions composed of α-synuclein (α-syn) characterize Parkinson's Disease (PD) and Dementia with Lewy bodies (DLB). Cognitive dysfunction defines DLB, and up to 80% of PD patients develop dementia. α-Syn inclusions are abundant in the hippocampus, yet functional consequences are unclear. To determine if pathologic α-syn causes neuronal defects, we induced endogenous α-syn to form inclusions resembling those found in diseased brains by treating hippocampal neurons with α-syn fibrils. At seven days after adding fibrils, α-syn inclusions are abundant in axons, but there is no cell death at this time point, allowing us to assess for potential alterations in neuronal function that are not caused by neuron death...
May 1, 2018: Acta Neuropathologica Communications
https://www.readbyqxmd.com/read/29716643/tdp-43-pathology-in-anterior-temporal-pole-cortex-in-aging-and-alzheimer-s-disease
#7
Sukriti Nag, Lei Yu, Patricia A Boyle, Sue E Leurgans, David A Bennett, Julie A Schneider
TDP-43 pathology was investigated in the anterior temporal pole cortex (ATPC) and orbital frontal cortex (OFC), regions often degenerated in frontotemporal lobar degenerations (FTLD), in aging and Alzheimer's disease (AD). Diagnosis of dementia in the 1160 autopsied participants from 3 studies of community-dwelling elders was based on clinical evaluation and cognitive performance tests which were used to create summary measures of the five cognitive domains. Neuronal and glial TDP-43 cytoplasmic inclusions were quantitated in 8 brain regions by immunohistochemistry, and used in ANOVA and regression analyses...
May 1, 2018: Acta Neuropathologica Communications
https://www.readbyqxmd.com/read/29703245/d409h-gba1-mutation-accelerates-the-progression-of-pathology-in-a53t-%C3%AE-synuclein-transgenic-mouse-model
#8
Donghoon Kim, Heehong Hwang, Seulah Choi, Sang Ho Kwon, Suhyun Lee, Jae Hong Park, SangMin Kim, Han Seok Ko
Heterozygous mutations in glucocerebrosidase 1 (GBA1) are a major genetic risk factor for Parkinson's disease and Dementia with Lewy bodies. Mutations in GBA1 leads to GBA1 enzyme deficiency, and GBA1-associated parkinsonism has an earlier age of onset and more progressive parkinsonism. To investigate a potential influence of GBA1 deficiency caused by mutations in GBA1 on the disease progression of PD, GBA1 mice carrying D409H knock-in mutation were crossbred with the human A53T (hA53T) α-synuclein transgenic mice...
April 27, 2018: Acta Neuropathologica Communications
https://www.readbyqxmd.com/read/29699580/genetic-deletion-of-muscle-rank-or-selective-inhibition-of-rankl-is-not-as-effective-as-full-length-opg-fc-in-mitigating-muscular-dystrophy
#9
Sébastien S Dufresne, Antoine Boulanger-Piette, Sabrina Bossé, Anteneh Argaw, Dounia Hamoudi, Laetitia Marcadet, Daniel Gamu, Val A Fajardo, Hideo Yagita, Josef M Penninger, A Russell Tupling, Jérôme Frenette
Although there is a strong association between osteoporosis and skeletal muscle atrophy/dysfunction, the functional relevance of a particular biological pathway that regulates synchronously bone and skeletal muscle physiopathology is still elusive. Receptor-activator of nuclear factor κB (RANK), its ligand RANKL and the soluble decoy receptor osteoprotegerin (OPG) are the key regulators of osteoclast differentiation and bone remodelling. We thus hypothesized that RANK/RANKL/OPG, which is a key pathway for bone regulation, is involved in Duchenne muscular dystrophy (DMD) physiopathology...
April 24, 2018: Acta Neuropathologica Communications
https://www.readbyqxmd.com/read/29699569/prion-infectivity-is-encoded-exclusively-within-the-structure-of-proteinase-k-resistant-fragments-of-synthetically-generated-recombinant-prp-sc
#10
Fei Wang, Xinhe Wang, Romany Abskharon, Jiyan Ma
Transmissible spongiform encephalopathies, also known as prion diseases, are a group of fatal neurodegenerative disorders affecting both humans and animals. The central pathogenic event in prion disease is the misfolding of normal prion protein (PrPC ) into the pathogenic conformer, PrPSc , which self-replicates by converting PrPC to more of itself. The biochemical hallmark of PrPSc is its C-terminal resistance to proteinase K (PK) digestion, which has been historically used to define PrPSc and is still the most widely used characteristic for prion detection...
April 24, 2018: Acta Neuropathologica Communications
https://www.readbyqxmd.com/read/29669601/potent-prion-like-behaviors-of-pathogenic-%C3%AE-synuclein-and-evaluation-of-inactivation-methods
#11
Airi Tarutani, Tetsuaki Arai, Shigeo Murayama, Shin-Ichi Hisanaga, Masato Hasegawa
The concept that abnormal protein aggregates show prion-like propagation between cells has been considered to explain the onset and progression of many neurodegenerative diseases. Indeed, both synthetic amyloid-like fibrils and pathogenic proteins extracted from patients' brains induce self-templated amplification and cell-to-cell transmission in vitro and in vivo. However, it is unclear whether exposure to exogenous prion-like proteins can potentially cause these diseases in humans. Here, we investigated in detail the prion-like seeding activities of several kinds of pathogenic α-synuclein (α-syn), including synthetic fibrils and detergent-insoluble fractions extracted from brains of patients with α-synucleinopathies...
April 18, 2018: Acta Neuropathologica Communications
https://www.readbyqxmd.com/read/29653597/phosphorylated-tdp-43-ptdp-43-aggregates-in-the-axial-skeletal-muscle-of-patients-with-sporadic-and-familial-amyotrophic-lateral-sclerosis
#12
Matthew D Cykowski, Suzanne Z Powell, Joan W Appel, Anithachristy S Arumanayagam, Andreana L Rivera, Stanley H Appel
Muscle atrophy with weakness is a core feature of amyotrophic lateral sclerosis (ALS) that has long been attributed to motor neuron loss alone. However, several studies in ALS patients, and more so in animal models, have challenged this assumption with the latter providing direct evidence that muscle can play an active role in the disease. Here, we examined the possible role of cell autonomous pathology in 148 skeletal muscle samples from 57 ALS patients, identifying phosphorylated TAR DNA-binding protein (pTDP-43) inclusions in the muscle fibers of 19 patients (33...
April 13, 2018: Acta Neuropathologica Communications
https://www.readbyqxmd.com/read/29642926/zidovudine-ameliorates-pathology-in-the-mouse-model-of-duchenne-muscular-dystrophy-via-p2rx7-purinoceptor-antagonism
#13
Rasha Al-Khalidi, Chiara Panicucci, Paul Cox, Natalia Chira, Justyna Róg, Christopher N J Young, Rhiannon E McGeehan, Kameshwari Ambati, Jayakrishna Ambati, Krzysztof Zabłocki, Elisabetta Gazzerro, Stephen Arkle, Claudio Bruno, Dariusz C Górecki
Duchenne muscular dystrophy (DMD) is the most common inherited muscle disorder that causes severe disability and death of young men. This disease is characterized by progressive muscle degeneration aggravated by sterile inflammation and is also associated with cognitive impairment and low bone density. Given that no current treatment can improve the long-term outcome, approaches with a strong translational potential are urgently needed. Duchenne muscular dystrophy (DMD) alters P2RX7 signaling in both muscle and inflammatory cells and inhibition of this receptor resulted in a significant attenuation of muscle and non-muscle symptoms in DMDmdx mouse model...
April 11, 2018: Acta Neuropathologica Communications
https://www.readbyqxmd.com/read/29615132/neuronal-sphingosine-kinase-2-subcellular-localization-is-altered-in-alzheimer-s-disease-brain
#14
Gaëlle Dominguez, Marie-Lise Maddelein, Mélanie Pucelle, Yvan Nicaise, Claude-Alain Maurage, Charles Duyckaerts, Olivier Cuvillier, Marie-Bernadette Delisle
BACKGROUND: Alzheimer's disease (AD) is characterized by the accumulation of β-amyloid (Aβ) peptides and hyperphosphorylated tau protein accompanied by neuronal loss. Aβ accumulation has been associated with an impaired sphingosine 1-phosphate (S1P) metabolism. S1P is generated by sphingosine kinases (SphKs), of which there are two isoenzymes SphK1 and SphK2, and degraded by the sphingosine 1-phosphate lyase (SPL). We previously reported, that both a decrease in SphK1 expression and an increase in SPL expression, correlated with amyloid deposits in the entorhinal cortex of AD brains, suggesting a global loss of pro-survival S1P in AD neurons...
April 3, 2018: Acta Neuropathologica Communications
https://www.readbyqxmd.com/read/29615128/prion-like-propagation-of-%C3%AE-amyloid-aggregates-in-the-absence-of-app-overexpression
#15
Alejandro Ruiz-Riquelme, Heather H C Lau, Erica Stuart, Adrienn N Goczi, Zhilan Wang, Gerold Schmitt-Ulms, Joel C Watts
The amyloid cascade hypothesis posits that the initiating event in Alzheimer's disease (AD) is the aggregation and deposition of the β-amyloid (Aβ) peptide, which is a proteolytic cleavage product of the amyloid precursor protein (APP). Mounting evidence suggests that the formation and spread of prion-like Aβ aggregates during AD may contribute to disease progression. Inoculation of transgenic mice that overexpress APP with pre-formed Aβ aggregates results in the prion-like induction of cerebral Aβ deposition...
April 3, 2018: Acta Neuropathologica Communications
https://www.readbyqxmd.com/read/29544532/braf-activating-mutations-involving-the-%C3%AE-3-%C3%AE-c-loop-in-v600e-negative-anaplastic-pleomorphic-xanthoastrocytoma
#16
LETTER
Drew Pratt, Sandra Camelo-Piragua, Kathryn McFadden, Denise Leung, Rajen Mody, Arul Chinnaiyan, Carl Koschmann, Sriram Venneti
No abstract text is available yet for this article.
March 15, 2018: Acta Neuropathologica Communications
https://www.readbyqxmd.com/read/29510748/preventing-mutant-huntingtin-proteolysis-and-intermittent-fasting-promote-autophagy-in-models-of-huntington-disease
#17
Dagmar E Ehrnhoefer, Dale D O Martin, Mandi E Schmidt, Xiaofan Qiu, Safia Ladha, Nicholas S Caron, Niels H Skotte, Yen T N Nguyen, Kuljeet Vaid, Amber L Southwell, Sabine Engemann, Sonia Franciosi, Michael R Hayden
Huntington disease (HD) is caused by the expression of mutant huntingtin (mHTT) bearing a polyglutamine expansion. In HD, mHTT accumulation is accompanied by a dysfunction in basal autophagy, which manifests as specific defects in cargo loading during selective autophagy. Here we show that the expression of mHTT resistant to proteolysis at the caspase cleavage site D586 (C6R mHTT) increases autophagy, which may be due to its increased binding to the autophagy adapter p62. This is accompanied by faster degradation of C6R mHTT in vitro and a lack of mHTT accumulation the C6R mouse model with age...
March 6, 2018: Acta Neuropathologica Communications
https://www.readbyqxmd.com/read/29506560/intravenous-injection-of-beta-amyloid-seeds-promotes-cerebral-amyloid-angiopathy-caa
#18
Michael Burwinkel, Manuel Lutzenberger, Frank L Heppner, Walter Schulz-Schaeffer, Michael Baier
Seeding and spread of beta-amyloid (Aβ) pathologies have been considered to be based on prion-like mechanisms. However, limited transmissibility of Aβ seeding activity upon peripheral exposure would represent a key difference to prions, not only in terms of pathogenesis but also in terms of potential transmission of disease. We partially characterized the seeded Aβ amyloidosis after intracerebral injection of various brain homogenates in APP/PS1 mice. One particularly seed-laden homogenate was selected to investigate the development of Aβ pathologies after intravenous exposure...
March 5, 2018: Acta Neuropathologica Communications
https://www.readbyqxmd.com/read/29499767/white-matter-changes-in-alzheimer-s-disease-a-focus-on-myelin-and-oligodendrocytes
#19
REVIEW
Sara E Nasrabady, Batool Rizvi, James E Goldman, Adam M Brickman
Alzheimer's disease (AD) is conceptualized as a progressive consequence of two hallmark pathological changes in grey matter: extracellular amyloid plaques and neurofibrillary tangles. However, over the past several years, neuroimaging studies have implicated micro- and macrostructural abnormalities in white matter in the risk and progression of AD, suggesting that in addition to the neuronal pathology characteristic of the disease, white matter degeneration and demyelination may be also important pathophysiological features...
March 2, 2018: Acta Neuropathologica Communications
https://www.readbyqxmd.com/read/29499756/rapid-detection-of-2-hydroxyglutarate-in-frozen-sections-of-idh-mutant-tumors-by-maldi-tof-mass-spectrometry
#20
Rémi Longuespée, Annika K Wefers, Elena De Vita, Aubry K Miller, David E Reuss, Wolfgang Wick, Christel Herold-Mende, Mark Kriegsmann, Peter Schirmacher, Andreas von Deimling, Stefan Pusch
All isocitrate dehydrogenase (IDH) mutant solid neoplasms exhibit highly elevated levels of D-2-hydroxyglutarate (D-2HG). Detection of 2HG in tumor tissues currently is performed by gas or liquid chromatography-mass spectrometry (GC- or LC-MS) or biochemical detection. While these methods are highly accurate, a considerable amount of time for tissue preparation and a relatively high amount of tissue is required for testing. We here present a rapid approach to detect 2HG in brain tumor tissue based on matrix-assisted laser desorption ionization - time of flight mass spectrometry (MALDI-TOF)...
March 2, 2018: Acta Neuropathologica Communications
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