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Brain Pathology

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https://www.readbyqxmd.com/read/28557134/tgf%C3%AE-pathway-deregulation-and-abnormal-phospho-smad2-3-staining-in-hereditary-cerebral-hemorrhage-with-amyloidosis-dutch-type
#1
Laure Grand Moursel, Leon P Munting, Linda M van der Graaf, Sjoerd G van Duinen, Marie-Jose T H Goumans, Uwe Ueberham, Remco Natté, Mark A van Buchem, Willeke M C van Roon-Mom, Louise van der Weerd
Hereditary cerebral hemorrhage with amyloidosis-Dutch type (HCHWA-D) is an early onset hereditary form of cerebral amyloid angiopathy (CAA) pathology, caused by the E22Q mutation in the amyloid β (Aβ) peptide. Transforming Growth Factor β1 (TGFβ1) is a key player in vascular fibrosis and in the formation of angiopathic vessels in transgenic mice. Therefore we investigated whether the TGFβ pathway is involved in HCHWA-D pathogenesis in human postmortem brain tissue from frontal and occipital lobes. Components of the TGFβ pathway were analyzed with quantitative RT-PCR...
May 29, 2017: Brain Pathology
https://www.readbyqxmd.com/read/28557010/rapid-amyloid-%C3%AE-oligomer-and-protofibril-accumulation-in-traumatic-brain-injury
#2
Sami Abu Hamdeh, Erik Rollman Waara, Christer Möller, Linda Söderberg, Hans Basun, Irina Alafuzoff, Lars Hillered, Lars Lannfelt, Martin Ingelsson, Niklas Marklund
OBJECTIVE: Deposition of amyloid-β (Aβ) is central to Alzheimer's disease (AD) pathogenesis and associated with progressive neurodegeneration in traumatic brain injury (TBI). We analysed predisposing factors for Aβ deposition including monomeric Aβ40, Aβ42 and Aβ oligomers/protofibrils, Aβ species with pronounced neurotoxic properties, following human TBI. METHODS: Highly selective ELISAs were used to analyse N-terminally intact and truncated Aβ40 and Aβ42, as well as Aβ oligomers/protofibrils, in human brain tissue, surgically resected from severe TBI patients (n=12; mean age 49...
May 29, 2017: Brain Pathology
https://www.readbyqxmd.com/read/28481062/preclinical-transgenic-and-patient-derived-xenograft-models-recapitulate-the-radiological-features-of-human-adamantinomatous-craniopharyngioma
#3
Jessica K R Boult, John R Apps, Annett Hölsken, J Ciaran Hutchinson, Carreno Gabriela, Laura S Danielson, Laura M Smith, Tobias Bäuerle, Rolf Buslei, Michael Buchfelder, Alex K Virasami, Alexander Koers, Owen J Arthurs, Thomas S Jacques, Louis Chesler, Juan Pedro Martinez-Barbera, Simon P Robinson
AIM: To assess the clinical relevance of transgenic and patient-derived xenograft models of adamantinomatous craniopharyngioma (ACP) using serial magnetic resonance imaging (MRI) and high resolution post-mortem micro-computed tomography (μ-CT), with correlation with histology and human ACP imaging. METHODS: The growth patterns and radiological features of tumours arising in Hesx1(Cre/+) ;Ctnnb1(l) °(x(ex3)/+) transgenic mice, and of patient-derived ACP xenografts implanted in the cerebral cortex, were monitored longitudinally in vivo with anatomical and functional MRI, and by ex vivo μ-CT at study end...
May 8, 2017: Brain Pathology
https://www.readbyqxmd.com/read/28474749/loss-of-smarce1-expression-is-a-specific-diagnostic-marker-of-clear-cell-meningioma-a-comprehensive-immunophenotypical-and-molecular-analysis
#4
Arnault Tauziede-Espariat, Béatrice Parfait, Aurore Besnard, Joëlle Lacombe, Johan Pallud, Sanaa Tazi, Stéphanie Puget, Guillaume Lot, Benoît Terris, Julie Cohen, Michel Vidaud, Dominique Figarella-Branger, Franck Monnien, Marc Polivka, Homa Adle-Biassette, Pascale Varlet
Clear cell meningioma (CCM) is a rare grade II histopathological subtype that usually occurs in young patients and displays high recurrence rate. Germline SMARCE1 mutations have been described in hereditary forms of this disease and more recently in small syndromic and sporadic CCM series. The diagnostic value of SMARCE1 in distinguishing between CCM and other meningioma variants has not been yet established. The aim of our study was to investigate the status of SMARCE1 in a series of CCMs and its morphological mimickers...
May 5, 2017: Brain Pathology
https://www.readbyqxmd.com/read/28470822/pericyte-derived-bone-morphogenetic-protein-4-underlies-white-matter-damage-after-chronic-hypoperfusion
#5
Maiko T Uemura, Masafumi Ihara, Takakuni Maki, Takayuki Nakagomi, Seiji Kaji, Kengo Uemura, Tomohiro Matsuyama, Raj N Kalaria, Ayae Kinoshita, Ryosuke Takahashi
Subcortical small vessel disease (SVD) is characterized by white matter damage resulting from arteriolosclerosis and chronic hypoperfusion. Transforming growth factor beta 1 (TGFB1) is dysregulated in the hereditary SVD, CARASIL (cerebral autosomal recessive arteriopathy with subcortical infarcts and leukoencephalopathy). However, very little is known about the role of the largest group in the TGFB superfamily - the bone morphogenetic proteins (BMPs) - in SVD pathogenesis. The aim of this study was to characterize signaling abnormalities of BMPs in sporadic SVD...
May 4, 2017: Brain Pathology
https://www.readbyqxmd.com/read/28452087/cytotoxic-cd8-t-cells-ablation-enhances-the-capacity-of-regulatory-t-cells-to-delay-viral-elimination-in-theiler-s-murine-encephalomyelitis
#6
Malgorzata Ciurkiewicz, Vanessa Herder, Muhammad Akram Khan, Ann-Kathrin Uhde, René Teich, Stephan Floess, Wolfgang Baumgärtner, Jochen Huehn, Andreas Beineke
Theiler's murine encephalomyelitis (TME) of susceptible mouse strains is a commonly used infectious animal model for multiple sclerosis. The study aim was to test the hypothesis whether cytotoxic T cell responses account for the limited impact of regulatory T cells on antiviral immunity in TME virus-induced demyelinating disease (TMEV-IDD) resistant C57BL/6 mice. TME virus-infected C57BL/6 mice were treated with (i) interleukin-2/-anti-interleukin-2-antibody-complexes to expand regulatory T cells ('Treg-expansion'), (ii) anti-CD8-antibodies to deplete cytotoxic T cells ('CD8-depletion') or (iii) with a combination of Treg-expansion and CD8-depletion ('combined treatment') prior to infection...
April 27, 2017: Brain Pathology
https://www.readbyqxmd.com/read/28401686/axonal-loss-in-the-multiple-sclerosis-spinal-cord-revisited
#7
Natalia Petrova, Daniele Carassiti, Daniel R Altmann, David Baker, Klaus Schmierer
Preventing chronic disease deterioration is an unmet need in people with multiple sclerosis, where axonal loss is considered a key substrate of disability. Clinically, chronic multiple sclerosis often presents as progressive myelopathy. Spinal cord cross-sectional area (CSA) assessed using MRI predicts increasing disability and has, by inference, been proposed as an indirect index of axonal degeneration. However, the association between CSA and axonal loss, and their correlation with demyelination, have never been systematically investigated using human post mortem tissue...
April 12, 2017: Brain Pathology
https://www.readbyqxmd.com/read/28380661/characterising-subtypes-of-hippocampal-sclerosis-and-reorganization-correlation-with-pre-and-postoperative-memory-deficit
#8
Anaclara Prada Jardim, Joan Liu, Jack Baber, Zuzanna Michalak, Cheryl Reeves, Matthew Ellis, Jan Novy, Jane de Tisi, Andrew McEvoy, Anna Miserocchi, Elza Marcia Targas Yacubian, Sanjay Sisodiya, Pamela Thompson, Maria Thom
Neuropathological subtypes of hippocampal sclerosis (HS) in temporal lobe epilepsy (The 2013 International League Against Epilepsy classification) are based on the qualitative assessment of patterns of neuronal loss with NeuN. In practice, some cases appear indeterminate between type 1 (CA1 and CA4 loss) and type 2 HS (CA1 loss) and we predicted that MAP2 would enable a more stringent classification. HS subtypes, as well as the accompanying alteration of axonal networks, regenerative capacity and neurodegeneration have been previously correlated with outcome and memory deficits and may provide prognostic clinical information...
April 5, 2017: Brain Pathology
https://www.readbyqxmd.com/read/28585389/an-11-year-old-boy-with-a-leptomeningeal-tumor
#9
Cyril Habougit, Fabien Forest, Claire Boutet, Catherine Douchet, Jean-Louis Stephan, Marie-Laure Stachowicz, Francois Vassal, Michel Péoc'h
No abstract text is available yet for this article.
July 2017: Brain Pathology
https://www.readbyqxmd.com/read/28585388/a-32-year-old-man-with-headache-and-visual-loss
#10
Fabio Rogerio, Luciano de Souza Queiroz, Fabiano Reis, Aya Fukuda, Nivaldo Adolfo Silva, Andrei Fernandes Joaquim
No abstract text is available yet for this article.
July 2017: Brain Pathology
https://www.readbyqxmd.com/read/28585387/a-53-year-old-woman-with-a-subfascial-mass-of-the-back-that-lasted-for-years
#11
Elia Guadagno, Domenico Solari, Gianpiero Iannuzzo, Eduardo Clery, Mararosaria Cervasio, Annarosaria De Chiara, Marialaura Del Basso De Caro
No abstract text is available yet for this article.
July 2017: Brain Pathology
https://www.readbyqxmd.com/read/28585386/understanding-neurodevelopmental-disorders-using-human-pluripotent-stem-cell-derived-neurons
#12
Claudia Tamburini, Meng Li
Research into psychiatric disorders has long been hindered by the lack of appropriate models. Induced pluripotent stem cells (iPSCs) offer an unlimited source of patient-specific cells, which in principle can be differentiated into all disease-relevant somatic cell types to create in vitro models of the disorder of interest. Here, neuronal differentiation protocols available for this purpose and the current progress on iPSCs-based models of schizophrenia, autism spectrum disorders and bipolar disorder were reviewed...
July 2017: Brain Pathology
https://www.readbyqxmd.com/read/28585384/modeling-the-c9orf72-repeat-expansion-mutation-using-human-induced-pluripotent-stem-cells
#13
Bhuvaneish T Selvaraj, Matthew R Livesey, Siddharthan Chandran
C9ORF72 repeat expansion is the most frequent causal genetic mutation giving rise to amyotrophic lateral sclerosis (ALS) and fronto-temporal dementia (FTD). The relatively recent discovery of the C9ORF72 repeat expansion in 2011 and the complexity of the mutation have meant that animal models that successfully recapitulate human C9ORF72 repeat expansion-mediated disease are only now emerging. Concurrent advances in the use of patient-derived induced pluripotent stem cells (iPSCs) to model aspects of neurological disease offers an additional approach for the study of C9ORF72 mutation...
July 2017: Brain Pathology
https://www.readbyqxmd.com/read/28585383/19-year-old-male-with-headaches-and-a-possible-seizure
#14
MacLean P Nasrallah, Ilya M Nasrallah, Marisa S Prelack, Margaret O Johnson, Travis B Lewis, Michael Rubenstein, Jane E Minturn, Arati Desai, Paul Marcotte, Mariarita Santi, Maria Martinez-Lage
No abstract text is available yet for this article.
July 2017: Brain Pathology
https://www.readbyqxmd.com/read/28585382/modeling-tau-pathology-in-human-stem-cell-derived-neurons
#15
Selina Wray
Tau pathology is a defining characteristic of multiple neurodegenerative disorders including Alzheimer's disease (AD) and Frontotemporal Dementia (FTD) with tau pathology. There is strong evidence from genetics and experimental models to support a central role for tau dysfunction in neuronal death, suggesting tau is a promising therapeutic target for AD and FTD. However, the development of tau pathology can precede symptom onset by several years, so understanding the earliest molecular events in tauopathy is a priority area of research...
July 2017: Brain Pathology
https://www.readbyqxmd.com/read/28585381/modeling-parkinson-s-disease-with-induced-pluripotent-stem-cells-harboring-%C3%AE-synuclein-mutations
#16
Karamjit Singh Dolt, Fella Hammachi, Tilo Kunath
Parkinson's disease (PD) is a common neurodegenerative condition affecting more than 8 million people worldwide. Although, the majority of PD cases are sporadic in nature, there are a growing number of monogenic mutations identified to cause PD in a highly penetrant manner. Many of these familial mutations give rise to a condition that is clinically and neuropathologically similar, if not identical, to sporadic PD. Mutations in genes such as SNCA cause PD in an autosomal dominant manner and patients have motor and non-motor symptoms that are typical for sporadic PD...
July 2017: Brain Pathology
https://www.readbyqxmd.com/read/28585380/astrocytes-in-a-dish-using-pluripotent-stem-cells-to-model-neurodegenerative-and-neurodevelopmental-disorders
#17
Lucy A Crompton, Oscar Cordero-Llana, Maeve A Caldwell
Neuroscience and Neurobiology have historically been neuron biased, yet up to 40% of the cells in the brain are astrocytes. These cells are heterogeneous and regionally diverse but universally essential for brain homeostasis. Astrocytes regulate synaptic transmission as part of the tripartite synapse, provide metabolic and neurotrophic support, recycle neurotransmitters, modulate blood flow and brain blood barrier permeability and are implicated in the mechanisms of neurodegeneration. Using pluripotent stem cells (PSC), it is now possible to study regionalised human astrocytes in a dish and to model their contribution to neurodevelopmental and neurodegenerative disorders...
July 2017: Brain Pathology
https://www.readbyqxmd.com/read/27537110/mango-leaf-extract-improves-central-pathology-and-cognitive-impairment-in-a-type-2-diabetes-mouse-model
#18
Carmen Infante-Garcia, Juan Jose Ramos-Rodriguez, Yolanda Marin-Zambrana, Maria Teresa Fernandez-Ponce, Lourdes Casas, Casimiro Mantell, Monica Garcia-Alloza
Epidemiological studies reveal that metabolic disorders, and specifically type 2 diabetes (T2D), are relevant risk factors to develop Alzheimer's disease (AD) and vascular dementia (VaD), the most common causes of dementia. AD patients are in a tremendous need of new therapeutic options because of the limited success of available treatments. Natural polyphenols, and concretely Mangifera indica Linn extract (MGF), have been reported to have antiinflammatory, antioxidant and antidiabetic activities. The role of MGF in central complications associated with T2D, after long-term treatment of db/db mice with MGF was analyzed...
July 2017: Brain Pathology
https://www.readbyqxmd.com/read/27514013/long-term-survival-and-regeneration-of-neuronal-and-vasculature-cells-inside-the-core-region-after-ischemic-stroke-in-adult-mice
#19
Michael Qize Jiang, Ying-Ying Zhao, Wenyuan Cao, Zheng Zachory Wei, Xiaohuan Gu, Ling Wei, Shan Ping Yu
Focal cerebral ischemia results in an ischemic core surrounded by the peri-infarct region (penumbra). Most research attention has been focused on penumbra while the pattern of cell fates inside the ischemic core is poorly defined. In the present investigation, we tested the hypothesis that, inside the ischemic core, some neuronal and vascular cells could survive the initial ischemic insult while regenerative niches might exist many days after stroke in the adult brain. Adult mice were subjected to focal cerebral ischemia induced by permanent occlusion of distal branches of the middle cerebral artery (MCA) plus transient ligations of bilateral common carotid artery (CCA)...
July 2017: Brain Pathology
https://www.readbyqxmd.com/read/27495267/tdp-43-pathology-in-alzheimer-s-disease-dementia-with-lewy-bodies-and-ageing
#20
Kirsty E McAleese, Lauren Walker, Daniel Erskine, Alan J Thomas, Ian G McKeith, Johannes Attems
Intracellular inclusions consisting of TAR DNA binding protein-43 (TDP-43 pathology) are present in up to 57% of Alzheimer's disease (AD) cases and follow a distinct topographical pattern of progression described in the TDP-43 in AD staging scheme. This scheme has not been applied to the assessment of TDP-43 pathology in dementia with Lewy bodies (DLB) and aged controls. We investigated TDP-43 pathology prevalence and severity in AD, DLB, mixed AD/DLB (Mx AD/DLB) and aged controls. One hundred and nineteen human post-mortem brains were included, neuropathologically diagnosed as AD: 46, DLB: 15, Mx AD/DLB: 19 and aged controls: 39...
July 2017: Brain Pathology
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