journal
MENU ▼
Read by QxMD icon Read
search

Acta Neuropathologica Communications

journal
https://www.readbyqxmd.com/read/29310723/iatrogenic-creutzfeldt-jakob-disease-with-amyloid-%C3%AE-pathology-an-international-study
#1
Ignazio Cali, Mark L Cohen, Stéphane Haїk, Piero Parchi, Giorgio Giaccone, Steven J Collins, Diane Kofskey, Han Wang, Catriona A McLean, Jean-Philippe Brandel, Nicolas Privat, Véronique Sazdovitch, Charles Duyckaerts, Tetsuyuki Kitamoto, Ermias D Belay, Ryan A Maddox, Fabrizio Tagliavini, Maurizio Pocchiari, Ellen Leschek, Brian S Appleby, Jiri G Safar, Lawrence B Schonberger, Pierluigi Gambetti
The presence of pathology related to the deposition of amyloid-β (Aβ) has been recently reported in iatrogenic Creutzfeldt-Jakob disease (iCJD) acquired from inoculation of growth hormone (GH) extracted from human cadaveric pituitary gland or use of cadaveric dura mater (DM) grafts.To investigate this phenomenon further, a cohort of 27 iCJD cases - 21 with adequate number of histopathological sections - originating from Australia, France, Italy, and the Unites States, were examined by immunohistochemistry, amyloid staining, and Western blot analysis of the scrapie prion protein (PrPSc), and compared with age-group matched cases of sporadic CJD (sCJD), Alzheimer disease (AD) or free of neurodegenerative diseases (non-ND)...
January 8, 2018: Acta Neuropathologica Communications
https://www.readbyqxmd.com/read/29301568/surfen-a-proteoglycan-binding-agent-reduces-inflammation-but-inhibits-remyelination-in-murine-models-of-multiple-sclerosis
#2
Jordan R Warford, Anna-Claire Lamport, Derek R Clements, Alicia Malone, Barry E Kennedy, Youra Kim, Shashi A Gujar, David W Hoskin, Alexander S Easton
Proteoglycans are promising therapeutic targets in Multiple Sclerosis (MS), because they regulate many aspects of the immune response. This was studied using surfen, an agent that binds both heparan sulphate proteoglycans (HSPGs) and chondroitin sulphate proteoglycans (CSPGs). Initial cell culture work on bone marrow derived macrophages (BMDMs) found that surfen reduced concentrations of the chemokines CCL2, CCL4 and CCL5, with reduced messenger (m)RNA expression for Tumor Necrosis Factor, IL-6, IL-1β and inducible nitric oxide synthase...
January 4, 2018: Acta Neuropathologica Communications
https://www.readbyqxmd.com/read/29298733/progressive-striatonigral-degeneration%C3%A2-in-a-transgenic-mouse-model-of-multiple-system-atrophy-translational-implications-for-interventional-therapies
#3
Violetta Refolo, Francesco Bez, Alexia Polissidis, Daniela Kuzdas-Wood, Edith Sturm, Martina Kamaratou, Werner Poewe, Leonidas Stefanis, M Angela Cenci, Marina Romero-Ramos, Gregor K Wenning, Nadia Stefanova
Multiple system atrophy (MSA) is a rapidly progressive neurodegenerative disorder characterized by widespread oligodendroglial cytoplasmic inclusions of filamentous α-synuclein, and neuronal loss in autonomic centres, basal ganglia and cerebellar circuits. It has been suggested that primary oligodendroglial α-synucleinopathy may represent a trigger in the pathogenesis of MSA, but the mechanisms underlying selective vulnerability and disease progression are unclear. The post-mortem analysis of MSA brains provides a static final picture of the disease neuropathology, but gives no clear indication on the sequence of pathogenic events in MSA...
January 3, 2018: Acta Neuropathologica Communications
https://www.readbyqxmd.com/read/29298724/brainstem-tau-pathology-in-alzheimer-s-disease-is-characterized-by-increase-of-three-repeat-tau-and-independent-of-amyloid%C3%A2-%C3%AE
#4
Miho Uematsu, Ayako Nakamura, Momoko Ebashi, Katsuiku Hirokawa, Ryosuke Takahashi, Toshiki Uchihara
INTRODUCTION: Alzheimer-type neuropil threads (NTs) and neurofibrillary tangles (NFTs) are comprised of either 4 repeat (4R)-tau, 3 repeat (3R)-tau, or a mixture of both. In the hippocampus, the number of NFTs, and the proportion of 3R tau progressively increases. If this preferential accumulation of 3R tau also occurs in the brainstem, it may be fundamentally related to progression of Alzheimer pathology. METHODS: Midbrain and pontine sections of brainstems from 23 cases (Braak-NFT stages I/II: 8, III/IV: 8, and V/VI: 7) were double immunofluorolabeled for 4R and 3R tau...
January 3, 2018: Acta Neuropathologica Communications
https://www.readbyqxmd.com/read/29298722/pyroglutamate-and-isoaspartate-modified-amyloid-beta-in-ageing-and-alzheimer-s-disease
#5
Maria Luisa Moro, Andrew Stephen Phillips, Katie Gaimster, Christian Paul, Amritpal Mudher, James A R Nicoll, Delphine Boche
Alzheimer's disease (AD) is the most common cause of dementia among older adults. Accumulation of amyloid-β (Aβ) in the brain is considered central in AD pathogenesis and its understanding crucial for developing new diagnostic and therapeutic approaches. Recent literature suggests that ageing may induce post translational modifications in Aβ, in the form of spontaneous amino acid modifications, which enhance its pathogenic properties, contributing to its aggregation.In this study, we have investigated whether the isoaspartate (IsoD-Aβ) and pyroglutamate (pE3-Aβ) modified forms of Aβ are significantly associated with AD pathology or represent markers of ageing...
January 3, 2018: Acta Neuropathologica Communications
https://www.readbyqxmd.com/read/29258615/what-is-the-evidence-that-tau-pathology-spreads-through-prion-like-propagation
#6
REVIEW
Amrit Mudher, Morvane Colin, Simon Dujardin, Miguel Medina, Ilse Dewachter, Seyedeh Maryam Alavi Naini, Eva-Maria Mandelkow, Eckhard Mandelkow, Luc Buée, Michel Goedert, Jean-Pierre Brion
Emerging experimental evidence suggests that the spread of tau pathology in the brain in Tauopathies reflects the propagation of abnormal tau species along neuroanatomically connected brain areas. This propagation could occur through a "prion-like" mechanism involving transfer of abnormal tau seeds from a "donor cell" to a "recipient cell" and recruitment of normal tau in the latter to generate new tau seeds. This review critically appraises the evidence that the spread of tau pathology occurs via such a "prion-like" mechanism and proposes a number of recommendations for directing future research...
December 19, 2017: Acta Neuropathologica Communications
https://www.readbyqxmd.com/read/29246238/absence-of-mgmt-promoter-methylation-in-diffuse-midline-glioma-h3-k27m-mutant
#7
LETTER
Rouzbeh Banan, Arne Christians, Stephan Bartels, Ulrich Lehmann, Christian Hartmann
No abstract text is available yet for this article.
December 15, 2017: Acta Neuropathologica Communications
https://www.readbyqxmd.com/read/29237481/retraction-note-transgenic-mice-overexpressing-the-als-linked-protein-matrin-3-develop-a-profound-muscle-phenotype
#8
Christina Moloney, Sruti Rayaprolu, John Howard, Susan Fromholt, Hilda Brown, Matt Collins, Mariela Cabrera, Colin Duffy, Zoe Siemienski, Dave Miller, Maurice S Swanson, Lucia Notterpek, David R Borchelt, Jada Lewis
The authors are retracting this article. The article describes mice expressing wild-type human MATR3. However, since publication the authors have become aware that all of the lines of mice described express human MATR3 containing the F115C mutation. Transgenic mice expressing wild-type and mutant Matrin were created simultaneously in their laboratory and, at a crucial stage of generating the DNA for embryo injection, as confirmed by an investigation by the University of Florida, the DNA preparations were accidentally mislabelled...
December 13, 2017: Acta Neuropathologica Communications
https://www.readbyqxmd.com/read/29216908/clinical-and-neuropathological-features-of-als-ftd-with-tia1-mutations
#9
Veronica Hirsch-Reinshagen, Cyril Pottier, Alexandra M Nicholson, Matt Baker, Ging-Yuek R Hsiung, Charles Krieger, Pheth Sengdy, Kevin B Boylan, Dennis W Dickson, Marsel Mesulam, Sandra Weintraub, Eileen Bigio, Lorne Zinman, Julia Keith, Ekaterina Rogaeva, Sasha A Zivkovic, David Lacomis, J Paul Taylor, Rosa Rademakers, Ian R A Mackenzie
Mutations in the stress granule protein T-cell restricted intracellular antigen 1 (TIA1) were recently shown to cause amyotrophic lateral sclerosis (ALS) with or without frontotemporal dementia (FTD). Here, we provide detailed clinical and neuropathological descriptions of nine cases with TIA1 mutations, together with comparisons to sporadic ALS (sALS) and ALS due to repeat expansions in C9orf72 (C9orf72+). All nine patients with confirmed mutations in TIA1 were female. The clinical phenotype was heterogeneous with a range in the age at onset from late twenties to the eighth decade (mean = 60 years) and disease duration from one to 6 years (mean = 3 years)...
December 7, 2017: Acta Neuropathologica Communications
https://www.readbyqxmd.com/read/29208041/metabolomics-reveals-distinct-antibody-independent-molecular-signatures-of-ms-aqp4-antibody-and-mog-antibody-disease
#10
Maciej Jurynczyk, Fay Probert, Tianrong Yeo, George Tackley, Tim D W Claridge, Ana Cavey, Mark R Woodhall, Siddharth Arora, Torsten Winkler, Eric Schiffer, Angela Vincent, Gabriele DeLuca, Nicola R Sibson, M Isabel Leite, Patrick Waters, Daniel C Anthony, Jacqueline Palace
The overlapping clinical features of relapsing remitting multiple sclerosis (RRMS), aquaporin-4 (AQP4)-antibody (Ab) neuromyelitis optica spectrum disorder (NMOSD), and myelin oligodendrocyte glycoprotein (MOG)-Ab disease mean that detection of disease specific serum antibodies is the gold standard in diagnostics. However, antibody levels are not prognostic and may become undetectable after treatment or during remission. Therefore, there is still a need to discover antibody-independent biomarkers. We sought to discover whether plasma metabolic profiling could provide biomarkers of these three diseases and explore if the metabolic differences are independent of antibody titre...
December 6, 2017: Acta Neuropathologica Communications
https://www.readbyqxmd.com/read/29195512/blood-brain-barrier-hyperpermeability-precedes-demyelination-in-the-cuprizone-model
#11
Stefan A Berghoff, Tim Düking, Lena Spieth, Jan Winchenbach, Sina K Stumpf, Nina Gerndt, Kathrin Kusch, Torben Ruhwedel, Wiebke Möbius, Gesine Saher
In neuroinflammatory disorders such as multiple sclerosis, the physiological function of the blood-brain barrier (BBB) is perturbed, particularly in demyelinating lesions and supposedly secondary to acute demyelinating pathology. Using the toxic non-inflammatory cuprizone model of demyelination, we demonstrate, however, that the onset of persistent BBB impairment precedes demyelination. In addition to a direct effect of cuprizone on endothelial cells, a plethora of inflammatory mediators, which are mainly of astroglial origin during the initial disease phase, likely contribute to the destabilization of endothelial barrier function in vivo...
December 1, 2017: Acta Neuropathologica Communications
https://www.readbyqxmd.com/read/29195510/cerebrovascular-pathology-in-down-syndrome-and-alzheimer-disease
#12
Elizabeth Head, Michael J Phelan, Eric Doran, Ronald C Kim, Wayne W Poon, Frederick A Schmitt, Ira T Lott
People with Down syndrome (DS) are at high risk for developing Alzheimer disease (AD) with age. Typically, by age 40 years, most people with DS have sufficient neuropathology for an AD diagnosis. Interestingly, atherosclerosis and hypertension are atypical in DS with age, suggesting the lack of these vascular risk factors may be associated with reduced cerebrovascular pathology. However, because the extra copy of APP leads to increased beta-amyloid peptide (Aβ) accumulation in DS, we hypothesized that there would be more extensive and widespread cerebral amyloid angiopathy (CAA) with age in DS relative to sporadic AD...
December 1, 2017: Acta Neuropathologica Communications
https://www.readbyqxmd.com/read/29191246/munc18-1-gene-abnormalities-are-involved-in-neurodevelopmental-disorders-through-defective-cortical-architecture-during-brain-development
#13
Nanako Hamada, Ikuko Iwamoto, Hidenori Tabata, Koh-Ichi Nagata
While Munc18-1 interacts with Syntaxin1 and controls the formation of soluble N-ethylmaleimide-sensitive factor attachment protein receptors (SNARE) complex to regulate presynaptic vesicle fusion in developed neurons, this molecule is likely to be involved in brain development since its gene abnormalities cause early infantile epileptic encephalopathy with suppression-burst (Ohtahara syndrome), neonatal epileptic encephalopathy and other neurodevelopmental disorders. We thus analyzed physiological significance of Munc18-1 during cortical development...
November 30, 2017: Acta Neuropathologica Communications
https://www.readbyqxmd.com/read/29187256/astrocytes-in-mouse-models-of-tauopathies-acquire-early-deficits-and-lose-neurosupportive-functions
#14
Marta Sidoryk-Wegrzynowicz, Yannick N Gerber, Miriam Ries, Magdalena Sastre, Aviva M Tolkovsky, Maria Grazia Spillantini
Microtubule-associated protein tau aggregates constitute the characteristic neuropathological features of several neurodegenerative diseases grouped under the name of tauopathies. It is now clear that the process of tau aggregation is associated with neurodegeneration. Several transgenic tau mouse models have been developed where tau progressively aggregates, causing neuronal death. Previously we have shown that transplantation of astrocytes in P301S tau transgenic mice rescues cortical neuron death, implying that the endogenous astrocytes are deficient in survival support...
November 29, 2017: Acta Neuropathologica Communications
https://www.readbyqxmd.com/read/29187252/atypical-non-standard-functions-of-the-microtubule-associated-tau-protein
#15
REVIEW
Ioannis Sotiropoulos, Marie-Christine Galas, Joana M Silva, Efthimios Skoulakis, Susanne Wegmann, Mahmoud Bukar Maina, David Blum, Carmen Laura Sayas, Eva-Maria Mandelkow, Eckhard Mandelkow, Maria Grazia Spillantini, Nuno Sousa, Jesus Avila, Miguel Medina, Amrit Mudher, Luc Buee
Since the discovery of the microtubule-associated protein Tau (MAPT) over 40 years ago, most studies have focused on Tau's role in microtubule stability and regulation, as well as on the neuropathological consequences of Tau hyperphosphorylation and aggregation in Alzheimer's disease (AD) brains. In recent years, however, research efforts identified new interaction partners and different sub-cellular localizations for Tau suggesting additional roles beyond its standard function as microtubule regulating protein...
November 29, 2017: Acta Neuropathologica Communications
https://www.readbyqxmd.com/read/29187238/eurotau-towing-scientists-to-tau-without-tautology
#16
LETTER
Amrit Mudher, Jean-Pierre Brion, Jesus Avila, Miguel Medina, Luc Buée
No abstract text is available yet for this article.
November 29, 2017: Acta Neuropathologica Communications
https://www.readbyqxmd.com/read/29178933/the-chronically-inflamed-central-nervous-system-provides-niches-for-long-lived-plasma-cells
#17
Karolin Pollok, Ronja Mothes, Carolin Ulbricht, Alina Liebheit, Jan David Gerken, Sylvia Uhlmann, Friedemann Paul, Raluca Niesner, Helena Radbruch, Anja Erika Hauser
Although oligoclonal bands in the cerebrospinal fluid have been a hallmark of multiple sclerosis diagnosis for over three decades, the role of antibody-secreting cells in multiple sclerosis remains unclear. T and B cells are critical for multiple sclerosis pathogenesis, but increasing evidence suggests that plasma cells also contribute, through secretion of autoantibodies. Long-lived plasma cells are known to drive various chronic inflammatory conditions as e.g. systemic lupus erythematosus, however, to what extent they are present in autoimmune central nervous system inflammation has not yet been investigated...
November 25, 2017: Acta Neuropathologica Communications
https://www.readbyqxmd.com/read/29169405/atypical-creutzfeldt-jakob-disease-with-prp-amyloid-plaques-in-white-matter-molecular-characterization-and-transmission-to-bank-voles-show-the-m1-strain-signature
#18
Marcello Rossi, Daniela Saverioni, Michele Di Bari, Simone Baiardi, Afina Willemina Lemstra, Laura Pirisinu, Sabina Capellari, Annemieke Rozemuller, Romolo Nonno, Piero Parchi
Amyloid plaques formed by abnormal prion protein (PrPSc) aggregates occur with low frequency in Creutzfeldt-Jakob disease, but represent a pathological hallmark of three relatively rare disease histotypes, namely variant CJD, sporadic CJDMV2K (methionine/valine at PRNP codon 129, PrPSc type 2 and kuru-type amyloid plaques) and iatrogenic CJDMMiK (MM at codon 129, PrPSc of intermediate type and kuru plaques). According to recent studies, however, PrP-amyloid plaques involving the subcortical and deep nuclei white matter may also rarely occur in CJDMM1 (MM at codon 129 and PrPSc type 1), the most common CJD histotype...
November 23, 2017: Acta Neuropathologica Communications
https://www.readbyqxmd.com/read/29166931/elevated-lrrk2-autophosphorylation-in-brain-derived-and-peripheral-exosomes-in-lrrk2-mutation-carriers
#19
Shijie Wang, Zhiyong Liu, Tao Ye, Omar S Mabrouk, Tyler Maltbie, Jan Aasly, Andrew B West
Missense mutations in the leucine-rich repeat kinase 2 (LRRK2) gene can cause late-onset Parkinson disease (PD). LRRK2 mutations increase LRRK2 kinase activities that may increase levels of LRRK2 autophosphorylation at serine 1292 (pS1292) and neurotoxicity in model systems. pS1292-LRRK2 protein can be packaged into exosomes and measured in biobanked urine. Herein we provide evidence that pS1292-LRRK2 protein is robustly expressed in cerebral spinal fluid (CSF) exosomes. In a novel cohort of Norwegian subjects with and without the G2019S-LRRK2 mutation, with and without PD, we quantified levels of pS1292-LRRK2, total LRRK2, and other exosome proteins in urine from 132 subjects and in CSF from 82 subjects...
November 22, 2017: Acta Neuropathologica Communications
https://www.readbyqxmd.com/read/29162163/%C3%AE-synuclein-fibrils-recruit-peripheral-immune-cells-in-the-rat-brain-prior-to-neurodegeneration
#20
Ashley S Harms, Vedad Delic, Aaron D Thome, Nicole Bryant, Zhiyong Liu, Sidhanth Chandra, Asta Jurkuvenaite, Andrew B West
Genetic variation in a major histocompatibility complex II (MHCII)-encoding gene (HLA-DR) increases risk for Parkinson disease (PD), and the accumulation of MHCII-expressing immune cells in the brain correlates with α-synuclein inclusions. However, the timing of MHCII-cell recruitment with respect to ongoing neurodegeneration, and the types of cells that express MHCII in the PD brain, has been difficult to understand. Recent studies show that the injection of short α-synuclein fibrils into the rat substantia nigra pars compacta (SNpc) induces progressive inclusion formation in SNpc neurons that eventually spread to spiny projection neurons in the striatum...
November 21, 2017: Acta Neuropathologica Communications
journal
journal
47911
1
2
Fetch more papers »
Fetching more papers... Fetching...
Read by QxMD. Sign in or create an account to discover new knowledge that matter to you.
Remove bar
Read by QxMD icon Read
×

Search Tips

Use Boolean operators: AND/OR

diabetic AND foot
diabetes OR diabetic

Exclude a word using the 'minus' sign

Virchow -triad

Use Parentheses

water AND (cup OR glass)

Add an asterisk (*) at end of a word to include word stems

Neuro* will search for Neurology, Neuroscientist, Neurological, and so on

Use quotes to search for an exact phrase

"primary prevention of cancer"
(heart or cardiac or cardio*) AND arrest -"American Heart Association"