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Acta Neuropathologica Communications

Ausgabe 1/2016

Inhalt (131 Artikel)

Open Access Research

Spatial genomic heterogeneity in diffuse intrinsic pontine and midline high-grade glioma: implications for diagnostic biopsy and targeted therapeutics

Lindsey M. Hoffman, Mariko DeWire, Scott Ryall, Pawel Buczkowicz, James Leach, Lili Miles, Arun Ramani, Michael Brudno, Shiva Senthil Kumar, Rachid Drissi, Phillip Dexheimer, Ralph Salloum, Lionel Chow, Trent Hummel, Charles Stevenson, Q. Richard Lu, Blaise Jones, David Witte, Bruce Aronow, Cynthia E. Hawkins, Maryam Fouladi

Open Access Erratum

Erratum: C9ORF72 expression and cellular localization over mouse development

Rachel A. K. Atkinson, Carmen M. Fernandez-Martos, Julie D. Atkin, James C. Vickers, Anna E. King

Open Access Research

Early and gender-specific differences in spinal cord mitochondrial function and oxidative stress markers in a mouse model of ALS

Daniel Cacabelos, Omar Ramírez-Núñez, Ana Belén Granado-Serrano, Pascual Torres, Victòria Ayala, Victoria Moiseeva, Mònica Povedano, Isidre Ferrer, Reinald Pamplona, Manuel Portero-Otin, Jordi Boada

Open Access Research

Farewell to GBM-O: Genomic and transcriptomic profiling of glioblastoma with oligodendroglioma component reveals distinct molecular subgroups

Benjamin H. Hinrichs, Scott Newman, Christina L. Appin, William Dunn, Lee Cooper, Rini Pauly, Jeanne Kowalski, Michael R. Rossi, Daniel J. Brat

Open Access Research

SF3B1 and EIF1AX mutations occur in primary leptomeningeal melanocytic neoplasms; yet another similarity to uveal melanomas

Heidi V. N. Küsters-Vandevelde, David Creytens, Adriana C. H. van Engen-van Grunsven, Marcel Jeunink, Veronique Winnepenninckx, Patricia J. T. A. Groenen, Benno Küsters, Pieter Wesseling, Willeke A. M. Blokx, Clemens F. M. Prinsen

Open Access Research

Low autophagy capacity implicated in motor system vulnerability to mutant superoxide dismutase

Eiichi Tokuda, Thomas Brännström, Peter M. Andersen, Stefan L. Marklund

Open Access Erratum

Erratum: Intracellular amyloid β oligomers impair organelle transport and induce dendritic spine loss in primary neurons

T Umeda, EM Ramser, M Yamashita, K Nakajima, H Mori, MA Silverman, T Tomiyama

Open Access Research

New insights into the protein aggregation pathology in myotilinopathy by combined proteomic and immunolocalization analyses

A. Maerkens, M. Olivé, A. Schreiner, S. Feldkirchner, J. Schessl, J. Uszkoreit, K. Barkovits, A. K. Güttsches, V. Theis, M. Eisenacher, M. Tegenthoff, L. G. Goldfarb, R. Schröder, B. Schoser, P. F. M. van der Ven, D. O. Fürst, M. Vorgerd, K. Marcus, R. A. Kley

Open Access Research

Diagnostically important muscle pathology in DNAJB6 mutated LGMD1D

Satu Sandell, Sanna Huovinen, Johanna Palmio, Olayinka Raheem, Mikaela Lindfors, Fang Zhao, Hannu Haapasalo, Bjarne Udd

Open Access Research

Emergence of two prion subtypes in ovine PrP transgenic mice infected with human MM2-cortical Creutzfeldt-Jakob disease prions

Jérôme Chapuis, Mohammed Moudjou, Fabienne Reine, Laetitia Herzog, Emilie Jaumain, Céline Chapuis, Isabelle Quadrio, Jacques Boulliat, Armand Perret-Liaudet, Michel Dron, Hubert Laude, Human Rezaei, Vincent Béringue

Open Access Research

Epidemiological pathology of Tau in the ageing brain: application of staging for neuropil threads (BrainNet Europe protocol) to the MRC cognitive function and ageing brain study

Stephen B. Wharton, Thais Minett, David Drew, Gillian Forster, Fiona Matthews, Carol Brayne, Paul G. Ince

Open Access Review

Animal modeling an oligodendrogliopathy – multiple system atrophy

Jonathan M. Bleasel, Glenda M. Halliday, Woojin Scott Kim

Open Access Erratum

Erratum: Spatial genomic heterogeneity in diffuse intrinsic pontine and midline high-grade glioma: implications for diagnostic biopsy and targeted therapeutics

Lindsey M. Hoffman, Mariko DeWire, Scott Ryall, Pawel Buczkowicz, James Leach, Lili Miles, Arun K. Ramani, Michael Brudno, Shiva Senthil Kumar, Rachid Drissi, Phillip Dexheimer, Ralph Salloum, Lionel Chow, Trent Hummel, Charles Stevenson, Q. Richard Lu, Blaise Jones, David Witte, Bruce Aronow, Cynthia E. Hawkins, Maryam Fouladi

Open Access Research

CSF complement 3 and factor H are staging biomarkers in Alzheimer’s disease

William T. Hu, Kelly D. Watts, Prashant Tailor, Trung P. Nguyen, Jennifer C. Howell, Raven C. Lee, Nicholas T. Seyfried, Marla Gearing, Chadwick M. Hales, Allan I. Levey, James J. Lah, Eva K. Lee

Open Access Research

Neuregulin 1 confers neuroprotection in SOD1-linked amyotrophic lateral sclerosis mice via restoration of C-boutons of spinal motor neurons

Jurate Lasiene, Okiru Komine, Noriko Fujimori-Tonou, Berit Powers, Fumito Endo, Seiji Watanabe, Jin Shijie, John Ravits, Philip Horner, Hidemi Misawa, Koji Yamanaka

Open Access Research

Unexpected partial correction of metabolic and behavioral phenotypes of Alzheimer’s APP/PSEN1 mice by gene targeting of diabetes/Alzheimer’s-related Sorcs1

Elysse M. Knight, Henry H. Ruiz, Soong Ho Kim, Jessica C. Harte, Wilson Hsieh, Charles Glabe, William L. Klein, Alan D. Attie, Christoph Buettner, Michelle E. Ehrlich, Sam Gandy

Open Access Research

Age-dependent neuroinflammation and cognitive decline in a novel Ala152Thr-Tau transgenic mouse model of PSP and AD

Astrid Sydow, Katja Hochgräfe, Stefanie Könen, Daniela Cadinu, Dorthe Matenia, Olga Petrova, Maria Joseph, Frank Johannes Dennissen, Eva-Maria Mandelkow

Open Access Research

Retention of hexanucleotide repeat-containing intron in C9orf72 mRNA: implications for the pathogenesis of ALS/FTD

Michael Niblock, Bradley N. Smith, Youn-Bok Lee, Valentina Sardone, Simon Topp, Claire Troakes, Safa Al-Sarraj, Claire S. Leblond, Patrick A. Dion, Guy A. Rouleau, Christopher E. Shaw, Jean-Marc Gallo

Open Access Research

A general homeostatic principle following lesion induced dendritic remodeling

Steffen Platschek, Hermann Cuntz, Mario Vuksic, Thomas Deller, Peter Jedlicka

Open Access Research

Adamantinomatous and papillary craniopharyngiomas are characterized by distinct epigenomic as well as mutational and transcriptomic profiles

Annett Hölsken, Martin Sill, Jessica Merkle, Leonille Schweizer, Michael Buchfelder, Jörg Flitsch, Rudolf Fahlbusch, Markus Metzler, Marcel Kool, Stefan M. Pfister, Andreas von Deimling, David Capper, David T. W. Jones, Rolf Buslei

Open Access Erratum

Erratum: Histological criteria for atypical pituitary adenomas – data from the German pituitary adenoma registry suggests modifications

Christian P. Miermeister, Stephan Petersenn, Michael Buchfelder, Rudolf Fahlbusch, Dieter K. Lüdecke, Annett Hölsken, Markus Bergmann, Ulrich Johannes Knappe, Volkmar H. Hans, Jörg Flitsch, Wolfgang Saeger, Rolf Buslei

Open Access Research

C1q-targeted inhibition of the classical complement pathway prevents injury in a novel mouse model of acute motor axonal neuropathy

Rhona McGonigal, Madeleine E. Cunningham, Denggao Yao, Jennifer A. Barrie, Sethu Sankaranarayanan, Simon N. Fewou, Koichi Furukawa, Ted A. Yednock, Hugh J. Willison

Open Access Research

Deposition of C-terminally truncated Aβ species Aβ37 and Aβ39 in Alzheimer’s disease and transgenic mouse models

Jochim Reinert, Bernhard C. Richard, Hans W. Klafki, Beate Friedrich, Thomas A. Bayer, Jens Wiltfang, Gabor G. Kovacs, Martin Ingelsson, Lars Lannfelt, Anders Paetau, Jonas Bergquist, Oliver Wirths

Open Access Research

Chronic Toxoplasma gondii infection enhances β-amyloid phagocytosis and clearance by recruited monocytes

Luisa Möhle, Nicole Israel, Kristin Paarmann, Markus Krohn, Sabine Pietkiewicz, Andreas Müller, Inna N. Lavrik, Jeffrey S. Buguliskis, Björn H. Schott, Dirk Schlüter, Eckart D. Gundelfinger, Dirk Montag, Ulrike Seifert, Jens Pahnke, Ildiko Rita Dunay

Open Access Research

Pneumococcal meningitis: Clinical-pathological correlations (meningene-path)

Joo-Yeon Engelen-Lee, Matthijs C. Brouwer, Eleonora Aronica, Diederik van de Beek

Open Access Research

High expression of α-synuclein in damaged mitochondria with PLA2G6 dysfunction

Hisae Sumi-Akamaru, Goichi Beck, Koei Shinzawa, Shinsuke Kato, Yuichi Riku, Mari Yoshida, Harutoshi Fujimura, Yoshihide Tsujimoto, Saburo Sakoda, Hideki Mochizuki

Open Access Research

Sphingosine-1-phosphate receptor inhibition prevents denervation-induced dendritic atrophy

Laurent M. Willems, Nadine Zahn, Nerea Ferreirós, Klaus Scholich, Nicola Maggio, Thomas Deller, Andreas Vlachos

Open Access Research

Super-resolution microscopy reveals γ-secretase at both sides of the neuronal synapse

Sophia Schedin-Weiss, Ina Caesar, Bengt Winblad, Hans Blom, Lars O. Tjernberg

Open Access Research

X-linked intellectual disability gene CASK regulates postnatal brain growth in a non-cell autonomous manner

Sarika Srivastava, Ryan McMillan, Jeffery Willis, Helen Clark, Vrushali Chavan, Chen Liang, Haiyan Zhang, Matthew Hulver, Konark Mukherjee

Open Access Research

Characteristics of gliomas in patients with somatic IDH mosaicism

Charlotte Bonnet, Laure Thomas, Dimitri Psimaras, Franck Bielle, Elodie Vauléon, Hugues Loiseau, Stéphanie Cartalat-Carel, David Meyronet, Caroline Dehais, Jérôme Honnorat, Marc Sanson, François Ducray

Open Access Research

Pathological tau deposition in Motor Neurone Disease and frontotemporal lobar degeneration associated with TDP-43 proteinopathy

Roya Behrouzi, Xiawei Liu, Dongyue Wu, Andrew C. Robinson, Sayuri Tanaguchi-Watanabe, Sara Rollinson, Jing Shi, Jinzhou Tian, Hisham H. M. Hamdalla, John Ealing, Anna Richardson, Matthew Jones, Stuart Pickering-Brown, Yvonne S. Davidson, Michael J. Strong, Masato Hasegawa, Julie S. Snowden, David M. A. Mann

Open Access Research

Upregulation of calpain activity precedes tau phosphorylation and loss of synaptic proteins in Alzheimer’s disease brain

Ksenia Kurbatskaya, Emma C. Phillips, Cara L. Croft, Giacomo Dentoni, Martina M. Hughes, Matthew A. Wade, Safa Al-Sarraj, Claire Troakes, Michael J. O’Neill, Beatriz G. Perez-Nievas, Diane P. Hanger, Wendy Noble

Open Access Research

Evaluation of alpha-synuclein immunohistochemical methods for the detection of Lewy-type synucleinopathy in gastrointestinal biopsies

Anne-Gaëlle Corbillé, Franck Letournel, Jeffrey H. Kordower, John Lee, Elisheva Shanes, Michel Neunlist, David G. Munoz, Pascal Derkinderen, Thomas G. Beach

Open Access Research

Chorea as a clinical feature of the basophilic inclusion body disease subtype of fused-in-sarcoma-associated frontotemporal lobar degeneration

Ito Kawakami, Zen Kobayashi, Tetsuaki Arai, Osamu Yokota, Takashi Nonaka, Naoya Aoki, Kazuhiro Niizato, Kenichi Oshima, Shinji Higashi, Omi Katsuse, Masato Hosokawa, Masato Hasegawa, Haruhiko Akiyama

Open Access Research

C9orf72 is differentially expressed in the central nervous system and myeloid cells and consistently reduced in C9orf72, MAPT and GRN mutation carriers

Patrizia Rizzu, Cornelis Blauwendraat, Sasja Heetveld, Emily M. Lynes, Melissa Castillo-Lizardo, Ashutosh Dhingra, Elwira Pyz, Markus Hobert, Matthis Synofzik, Javier Simón-Sánchez, Margherita Francescatto, Peter Heutink

Open Access Research

Rewiring neuronal microcircuits of the brain via spine head protrusions-a role for synaptopodin and intracellular calcium stores

David Verbich, Denise Becker, Andreas Vlachos, Peter Mundel, Thomas Deller, R. Anne McKinney

Open Access Research

Fasudil attenuates aggregation of α-synuclein in models of Parkinson’s disease

Lars Tatenhorst, Katrin Eckermann, Vivian Dambeck, Luis Fonseca-Ornelas, Hagen Walle, Tomás Lopes da Fonseca, Jan C. Koch, Stefan Becker, Lars Tönges, Mathias Bähr, Tiago F. Outeiro, Markus Zweckstetter, Paul Lingor

Open Access Research

TP53 hotspot mutations are predictive of survival in primary central nervous system lymphoma patients treated with combination chemotherapy

Helga D. Munch-Petersen, Fazila Asmar, Konstantinos Dimopoulos, Aušrinė Areškevičiūtė, Peter Brown, Mia Seremet Girkov, Anja Pedersen, Lene D. Sjö, Steffen Heegaard, Helle Broholm, Lasse S. Kristensen, Elisabeth Ralfkiaer, Kirsten Grønbæk

Open Access Research

Neuronal accumulation of unrepaired DNA in a novel specific chromatin domain: structural, molecular and transcriptional characterization

Jorge Mata-Garrido, Iñigo Casafont, Olga Tapia, Maria T. Berciano, Miguel Lafarga

Open Access Research

Clobetasol promotes remyelination in a mouse model of neuromyelitis optica

Xiaoming Yao, Tao Su, A. S. Verkman

Open Access Research

ALS-linked misfolded SOD1 species have divergent impacts on mitochondria

Sarah Pickles, Sabrina Semmler, Helen R. Broom, Laurie Destroismaisons, Laurine Legroux, Nathalie Arbour, Elizabeth Meiering, Neil R. Cashman, Christine Vande Velde

Open Access Research

Downstream effects of plectin mutations in epidermolysis bullosa simplex with muscular dystrophy

Lilli Winter, Matthias Türk, Patrick N. Harter, Michel Mittelbronn, Cornelia Kornblum, Fiona Norwood, Heinz Jungbluth, Christian T. Thiel, Ursula Schlötzer-Schrehardt, Rolf Schröder

Open Access Research

Differential roles of hypoxia and innate immunity in juvenile and adult dermatomyositis

Corinna Preuße, Yves Allenbach, Olaf Hoffmann, Hans-Hilmar Goebel, Debora Pehl, Josefine Radke, Alexandra Doeser, Udo Schneider, Rieke H.E. Alten, Tilmann Kallinich, Olivier Benveniste, Arpad von Moers, Benedikt Schoser, Ulrike Schara, Werner Stenzel

Open Access Research

Dorsal root ganglia in Friedreich ataxia: satellite cell proliferation and inflammation

Arnulf H. Koeppen, R. Liane Ramirez, Alyssa B. Becker, Joseph E. Mazurkiewicz

Open Access Research

Gene-specific mitochondria dysfunctions in human TARDBP and C9ORF72 fibroblasts

Elisa Onesto, Claudia Colombrita, Valentina Gumina, Maria Orietta Borghi, Sabrina Dusi, Alberto Doretti, Gigliola Fagiolari, Federica Invernizzi, Maurizio Moggio, Valeria Tiranti, Vincenzo Silani, Antonia Ratti

Open Access Research

Sustained expression of MCP-1 by low wall shear stress loading concomitant with turbulent flow on endothelial cells of intracranial aneurysm

Tomohiro Aoki, Kimiko Yamamoto, Miyuki Fukuda, Yuji Shimogonya, Shunichi Fukuda, Shuh Narumiya

Open Access Research

Critical residues involved in tau binding to fyn: implications for tau phosphorylation in Alzheimer’s disease

Dawn H. W. Lau, Marte Hogseth, Emma C. Phillips, Michael J. O’Neill, Amy M. Pooler, Wendy Noble, Diane P. Hanger

Open Access Research

V-akt murine thymoma viral oncogene homolog 3 (AKT3) contributes to poor disease outcome in humans and mice with pneumococcal meningitis

Mercedes Valls Serón, Bart Ferwerda, JooYeon Engelen-Lee, Madelijn Geldhoff, Valery Jaspers, Aeilko H. Zwinderman, Michael W. Tanck, Frank Baas, Arie van der Ende, Matthijs C. Brouwer, Diederik van de Beek

Open Access Research

The ALS/FTLD associated protein C9orf72 associates with SMCR8 and WDR41 to regulate the autophagy-lysosome pathway

Peter M. Sullivan, Xiaolai Zhou, Adam M. Robins, Daniel H. Paushter, Dongsung Kim, Marcus B. Smolka, Fenghua Hu

Open Access Research

Altered localization and functionality of TAR DNA Binding Protein 43 (TDP-43) in niemann- pick disease type C

A. Dardis, S. Zampieri, S. Canterini, K. L. Newell, C. Stuani, J. R. Murrell, B. Ghetti, M. T. Fiorenza, B. Bembi, E. Buratti

Open Access Research

Purkinje cell injury, structural plasticity and fusion in patients with Friedreich’s ataxia

Kevin C. Kemp, Amelia J. Cook, Juliana Redondo, Kathreena M. Kurian, Neil J. Scolding, Alastair Wilkins

Open Access Research

DNA methylation analysis of paediatric low-grade astrocytomas identifies a tumour-specific hypomethylation signature in pilocytic astrocytomas

Jennie N. Jeyapalan, Gabriel T. Doctor, Tania A. Jones, Samuel N. Alberman, Alexander Tep, Chirag M. Haria, Edward C. Schwalbe, Isabel C. F. Morley, Alfred A. Hill, Magdalena LeCain, Diego Ottaviani, Steven C. Clifford, Ibrahim Qaddoumi, Ruth G. Tatevossian, David W. Ellison, Denise Sheer

Open Access Research

NHERF1/EBP50 and NF2 as diagnostic markers for choroid plexus tumors

Maria-Magdalena Georgescu, Bret C. Mobley, Brent A. Orr, Ping Shang, Norman L. Lehman, Xiaoping Zhu, Thomas J. O’Neill, Veena Rajaram, Kimmo J. Hatanpaa, Charles F. Timmons, Jack M. Raisanen

Open Access Research

Germline or somatic GPR101 duplication leads to X-linked acrogigantism: a clinico-pathological and genetic study

Donato Iacovazzo, Richard Caswell, Benjamin Bunce, Sian Jose, Bo Yuan, Laura C. Hernández-Ramírez, Sonal Kapur, Francisca Caimari, Jane Evanson, Francesco Ferraù, Mary N. Dang, Plamena Gabrovska, Sarah J. Larkin, Olaf Ansorge, Celia Rodd, Mary L. Vance, Claudia Ramírez-Renteria, Moisés Mercado, Anthony P. Goldstone, Michael Buchfelder, Christine P. Burren, Alper Gurlek, Pinaki Dutta, Catherine S. Choong, Timothy Cheetham, Giampaolo Trivellin, Constantine A. Stratakis, Maria-Beatriz Lopes, Ashley B. Grossman, Jacqueline Trouillas, James R. Lupski, Sian Ellard, Julian R. Sampson, Federico Roncaroli, Márta Korbonits

Open Access Letter to the Editor

Imaging Invasion: Micro-CT imaging of adamantinomatous craniopharyngioma highlights cell type specific spatial relationships of tissue invasion

John R. Apps, J. Ciaran Hutchinson, Owen J. Arthurs, Alex Virasami, Abhijit Joshi, Berit Zeller-Plumhoff, Dale Moulding, Thomas S. Jacques, Neil J. Sebire, Juan Pedro Martinez-Barbera

Open Access Research

An autoradiographic evaluation of AV-1451 Tau PET in dementia

Val J. Lowe, Geoffry Curran, Ping Fang, Amanda M. Liesinger, Keith A. Josephs, Joseph E. Parisi, Kejal Kantarci, Bradley F. Boeve, Mukesh K. Pandey, Tyler Bruinsma, David S. Knopman, David T. Jones, Leonard Petrucelli, Casey N. Cook, Neill R. Graff-Radford, Dennis W. Dickson, Ronald C. Petersen, Clifford R. Jack Jr., Melissa E. Murray

Open Access Letter to the Editor

ARTAG in the basal forebrain: widening the constellation of astrocytic tau pathology

Alan King Lun Liu, Marc H. Goldfinger, Hayleigh E. Questari, Ronald K. B. Pearce, Steve M. Gentleman

Open Access Research

ATRX immunostaining predicts IDH and H3F3A status in gliomas

Azadeh Ebrahimi, Marco Skardelly, Irina Bonzheim, Ines Ott, Helmut Mühleisen, Franziska Eckert, Ghazaleh Tabatabai, Jens Schittenhelm

Open Access Research

Heterogeneity of cerebral TDP-43 pathology in sporadic amyotrophic lateral sclerosis: Evidence for clinico-pathologic subtypes

Ryoko Takeuchi, Mari Tada, Atsushi Shiga, Yasuko Toyoshima, Takuya Konno, Tomoe Sato, Hiroaki Nozaki, Taisuke Kato, Masao Horie, Hiroshi Shimizu, Hirohide Takebayashi, Osamu Onodera, Masatoyo Nishizawa, Akiyoshi Kakita, Hitoshi Takahashi

Open Access Research

Uncoupling neuronal death and dysfunction in Drosophila models of neurodegenerative disease

Amit K. Chouhan, Caiwei Guo, Yi-Chen Hsieh, Hui Ye, Mumine Senturk, Zhongyuan Zuo, Yarong Li, Shreyasi Chatterjee, Juan Botas, George R. Jackson, Hugo J. Bellen, Joshua M. Shulman

Open Access Research

Increased tauopathy drives microglia-mediated clearance of beta-amyloid

Wesley Chen, Edsel A. Abud, Stephen T. Yeung, Anita Lakatos, Trevor Nassi, Jane Wang, David Blum, Luc Buée, Wayne W. Poon, Mathew Blurton-Jones

Open Access Research

Notch1 hallmarks fibrillary depositions in sporadic Alzheimer’s disease

Emanuele Brai, Noemi Alina Raio, Lavinia Alberi

Open Access Research

The relationship between complement factor C3, APOE ε4, amyloid and tau in Alzheimer’s disease

Luke W. Bonham, Rahul S. Desikan, Jennifer S. Yokoyama

Open Access Research

Analysis of primary visual cortex in dementia with Lewy bodies indicates GABAergic involvement associated with recurrent complex visual hallucinations

Ahmad A. Khundakar, Peter S. Hanson, Daniel Erskine, Nichola Z. Lax, Joseph Roscamp, Evangelia Karyka, Eliona Tsefou, Preeti Singh, Simon J. Cockell, Andrew Gribben, Lynne Ramsay, Peter G. Blain, Urs P. Mosimann, Deborah J. Lett, Matthias Elstner, Douglass M. Turnbull, Charles C. Xiang, Michael J. Brownstein, John T. O’Brien, John-Paul Taylor, Johannes Attems, Alan J. Thomas, Ian G. McKeith, Christopher M. Morris

Open Access Erratum

Erratum to: ‘Purkinje cell injury, structural plasticity and fusion in patients with Friedreich’s ataxia’

Kevin C. Kemp, Amelia J. Cook, Juliana Redondo, Kathreena M. Kurian, Neil J. Scolding, Alastair Wilkins

Open Access Research

LRP1 expression in microglia is protective during CNS autoimmunity

Tzu-Ying Chuang, Yong Guo, Scott M. Seki, Abagail M. Rosen, David M. Johanson, James W. Mandell, Claudia F. Lucchinetti, Alban Gaultier

Open Access Research

Modeling Alexander disease with patient iPSCs reveals cellular and molecular pathology of astrocytes

Takayuki Kondo, Misato Funayama, Michiyo Miyake, Kayoko Tsukita, Takumi Era, Hitoshi Osaka, Takashi Ayaki, Ryosuke Takahashi, Haruhisa Inoue

Open Access Review

From animal models to human disease: a genetic approach for personalized medicine in ALS

Vincent Picher-Martel, Paul N. Valdmanis, Peter V. Gould, Jean-Pierre Julien, Nicolas Dupré

Open Access Research

A comprehensive next generation sequencing-based virome assessment in brain tissue suggests no major virus - tumor association

Michael J. Strong, Eugene Blanchard 4th, Zhen Lin, Cindy A. Morris, Melody Baddoo, Christopher M. Taylor, Marcus L. Ware, Erik K. Flemington

Open Access Research

Deletion of the type-1 interferon receptor in APPSWE/PS1ΔE9 mice preserves cognitive function and alters glial phenotype

Myles R. Minter, Zachery Moore, Moses Zhang, Kate M. Brody, Nigel C. Jones, Sandy R. Shultz, Juliet M. Taylor, Peter J. Crack

Open Access Case report

BK virus encephalopathy and sclerosing vasculopathy in a patient with hypohidrotic ectodermal dysplasia and immunodeficiency

Armine Darbinyan, Eugene O. Major, Susan Morgello, Steven Holland, Caroline Ryschkewitsch, Maria Chiara Monaco, Thomas P. Naidich, Joshua Bederson, Joanna Malaczynska, Fei Ye, Ronald Gordon, Charlotte Cunningham-Rundles, Mary Fowkes, Nadejda M. Tsankova

Open Access Research

Reduced Tau protein expression is associated with frontotemporal degeneration with progranulin mutation

Anthony Papegaey, Sabiha Eddarkaoui, Vincent Deramecourt, Francisco-Jose Fernandez-Gomez, Pierre Pantano, Hélène Obriot, Camille Machala, Vincent Anquetil, Agnès Camuzat, Alexis Brice, Claude-Alain Maurage, Isabelle Le Ber, Charles Duyckaerts, Luc Buée, Nicolas Sergeant, Valérie Buée-Scherrer

Open Access Research

Role of IL-33 and ST2 signalling pathway in multiple sclerosis: expression by oligodendrocytes and inhibition of myelination in central nervous system

Debbie Allan, Karen J. Fairlie-Clarke, Christina Elliott, Cornelia Schuh, Susan C. Barnett, Hans Lassmann, Christopher Linnington, Hui-Rong Jiang

Open Access Research

Astroglial NF-kB contributes to white matter damage and cognitive impairment in a mouse model of vascular dementia

Raman Saggu, Toni Schumacher, Florian Gerich, Cordula Rakers, Khalid Tai, Andrea Delekate, Gabor C. Petzold

Open Access Research

Human neuronal changes in brain edema and increased intracranial pressure

Nóra Faragó, Ágnes Katalin Kocsis, Csilla Braskó, Sándor Lovas, Márton Rózsa, Judith Baka, Balázs Kovács, Katalin Mikite, Viktor Szemenyei, Gábor Molnár, Attila Ozsvár, Gáspár Oláh, Ildikó Piszár, Ágnes Zvara, Attila Patócs, Pál Barzó, László G. Puskás, Gábor Tamás

Open Access Research

A combination of TERT promoter mutation and MGMT methylation status predicts clinically relevant subgroups of newly diagnosed glioblastomas

Hideyuki Arita, Kai Yamasaki, Yuko Matsushita, Taishi Nakamura, Asanao Shimokawa, Hirokazu Takami, Shota Tanaka, Akitake Mukasa, Mitsuaki Shirahata, Saki Shimizu, Kaori Suzuki, Kuniaki Saito, Keiichi Kobayashi, Fumi Higuchi, Takeo Uzuka, Ryohei Otani, Kaoru Tamura, Kazutaka Sumita, Makoto Ohno, Yasuji Miyakita, Naoki Kagawa, Naoya Hashimoto, Ryusuke Hatae, Koji Yoshimoto, Naoki Shinojima, Hideo Nakamura, Yonehiro Kanemura, Yoshiko Okita, Manabu Kinoshita, Kenichi Ishibashi, Tomoko Shofuda, Yoshinori Kodama, Kanji Mori, Yusuke Tomogane, Junya Fukai, Koji Fujita, Yuzo Terakawa, Naohiro Tsuyuguchi, Shusuke Moriuchi, Masahiro Nonaka, Hiroyoshi Suzuki, Makoto Shibuya, Taketoshi Maehara, Nobuhito Saito, Motoo Nagane, Nobutaka Kawahara, Keisuke Ueki, Toshiki Yoshimine, Etsuo Miyaoka, Ryo Nishikawa, Takashi Komori, Yoshitaka Narita, Koichi Ichimura

Open Access Letter to the Editor

Disseminated necrotizing leukoencephalopathy eight months after alemtuzumab treatment for multiple sclerosis

Imke Metz, Peter Rieckmann, Boris-Alexander Kallmann, Wolfgang Brück

Open Access Research

Aquaporin 4-specific T cells and NMO-IgG cause primary retinal damage in experimental NMO/SD

Bleranda Zeka, Maria Hastermann, Nathalie Kaufmann, Kathrin Schanda, Marko Pende, Tatsuro Misu, Paulus Rommer, Kazuo Fujihara, Ichiro Nakashima, Charlotte Dahle, Fritz Leutmezer, Markus Reindl, Hans Lassmann, Monika Bradl

Open Access Letter to the Editor

Pilocytic astrocytoma and glioneuronal tumor with histone H3 K27M mutation

Cordelia Orillac, Cheddhi Thomas, Yosef Dastagirzada, Eveline Teresa Hidalgo, John G. Golfinos, David Zagzag, Jeffrey H. Wisoff, Matthias A. Karajannis, Matija Snuderl

Open Access Letter to the Editor

Diminishing evidence for torsinA-positive neuronal inclusions in DYT1 dystonia

Drew Pratt, Karin Mente, Shervin Rahimpour, Nancy A. Edwards, Sule Tinaz, Brian D. Berman, Mark Hallett, Abhik Ray-Chaudhury

Open Access Research

The retina as an early biomarker of neurodegeneration in a rotenone-induced model of Parkinson’s disease: evidence for a neuroprotective effect of rosiglitazone in the eye and brain

Eduardo Maria Normando, Benjamin Michael Davis, Lies De Groef, Shereen Nizari, Lisa A. Turner, Nivedita Ravindran, Milena Pahlitzsch, Jonathan Brenton, Giulia Malaguarnera, Li Guo, Satyanarayana Somavarapu, Maria Francesca Cordeiro

Open Access Research

Telomere shortening leads to an acceleration of synucleinopathy and impaired microglia response in a genetic mouse model

Annika Scheffold, Inge R. Holtman, Sandra Dieni, Nieske Brouwer, Sarah-Fee Katz, Billy Michael Chelliah Jebaraj, Philipp J. Kahle, Bastian Hengerer, André Lechel, Stephan Stilgenbauer, Erik W. G. M. Boddeke, Bart J. L. Eggen, Karl-Lenhard Rudolph, Knut Biber

Open Access Research

Chromosome 1q gain and tenascin-C expression are candidate markers to define different risk groups in pediatric posterior fossa ependymoma

Asuka Araki, Monika Chocholous, Johannes Gojo, Christian Dorfer, Thomas Czech, Harald Heinzl, Karin Dieckmann, Inge M. Ambros, Peter F. Ambros, Irene Slavc, Christine Haberler

Open Access Research

Axonal plasticity underpins the functional recovery following surgical decompression in a rat model of cervical spondylotic myelopathy

Rana S. Dhillon, John Parker, Yasir A. Syed, Steve Edgley, Adam Young, James W. Fawcett, Nick D. Jeffery, Robin J. M. Franklin, Mark R. N. Kotter

Open Access Research

Revisiting rodent models: Octodon degus as Alzheimer’s disease model?

Johannes Steffen, Markus Krohn, Kristin Paarmann, Christina Schwitlick, Thomas Brüning, Rita Marreiros, Andreas Müller-Schiffmann, Carsten Korth, Katharina Braun, Jens Pahnke

Open Access Research

Spatio-temporal activation of caspase-8 in myeloid cells upon ischemic stroke

Johanna Rodhe, Miguel A. Burguillos, Rocio M. de Pablos, Edel Kavanagh, Annette Persson, Elisabet Englund, Tomas Deierborg, Jose L. Venero, Bertrand Joseph

Open Access Research

Targeted detection of genetic alterations reveal the prognostic impact of H3K27M and MAPK pathway aberrations in paediatric thalamic glioma

Scott Ryall, Rahul Krishnatry, Anthony Arnoldo, Pawel Buczkowicz, Matthew Mistry, Robert Siddaway, Cino Ling, Sanja Pajovic, Man Yu, Joshua B. Rubin, Juliette Hukin, Paul Steinbok, Ute Bartels, Eric Bouffet, Uri Tabori, Cynthia Hawkins

Open Access Research

Developmental delay in motor skill acquisition in Niemann-Pick C1 mice reveals abnormal cerebellar morphogenesis

Paola Caporali, Francesco Bruno, Giampiero Palladino, Jessica Dragotto, Laura Petrosini, Franco Mangia, Robert P. Erickson, Sonia Canterini, Maria Teresa Fiorenza

Open Access Research

Systemic inflammation disrupts oligodendrocyte gap junctions and induces ER stress in a model of CNS manifestations of X-linked Charcot-Marie-Tooth disease

Margarita Olympiou, Irene Sargiannidou, Kyriaki Markoullis, Christos Karaiskos, Alexia Kagiava, Styliana Kyriakoudi, Charles K. Abrams, Kleopas A. Kleopa

Open Access Research

Comprehensive protein tyrosine phosphatase mRNA profiling identifies new regulators in the progression of glioma

Annika M. Bourgonje, Kiek Verrijp, Jan T. G. Schepens, Anna C. Navis, Jolanda A. F. Piepers, Chantal B. C. Palmen, Monique van den Eijnden, Rob Hooft van Huijsduijnen, Pieter Wesseling, William P. J. Leenders, Wiljan J. A. J. Hendriks

Open Access Research

Neuropathology of supercentenarians - four autopsy case studies

Masaki Takao, Nobuyoshi Hirose, Yasumichi Arai, Ban Mihara, Masaru Mimura

Open Access Research

Rare TREM2 variants associated with Alzheimer’s disease display reduced cell surface expression

Daniel W. Sirkis, Luke W. Bonham, Renan E. Aparicio, Ethan G. Geier, Eliana Marisa Ramos, Qing Wang, Anna Karydas, Zachary A. Miller, Bruce L. Miller, Giovanni Coppola, Jennifer S. Yokoyama

Open Access Review

Pathogenesis of FUS-associated ALS and FTD: insights from rodent models

Matthew Nolan, Kevin Talbot, Olaf Ansorge

Open Access Research

Multiplex immunoassay characterization and species comparison of inflammation in acute and non-acute ischemic infarcts in human and mouse brain tissue

Thuy-Vi V. Nguyen, Jennifer B. Frye, Jacob C. Zbesko, Kristina Stepanovic, Megan Hayes, Alex Urzua, Geidy Serrano, Thomas G. Beach, Kristian P. Doyle

Open Access Erratum

Erratum to: Modeling Alexander disease with patient iPSCs reveals cellular and molecular pathology of astrocytes

Takayuki Kondo, Misato Funayama, Michiyo Miyake, Kayoko Tsukita, Takumi Era, Hitoshi Osaka, Takashi Ayaki, Ryosuke Takahashi, Haruhisa Inoue

Open Access Research

Microglial CX3CR1 promotes adult neurogenesis by inhibiting Sirt 1/p65 signaling independent of CX3CL1

Sabine Sellner, Ricardo Paricio-Montesinos, Alena Spieß, Annette Masuch, Daniel Erny, Laura A. Harsan, Dominik v. Elverfeldt, Marius Schwabenland, Knut Biber, Ori Staszewski, Sergio Lira, Steffen Jung, Marco Prinz, Thomas Blank

Open Access Research

Lipid microdomain modification sustains neuronal viability in models of Alzheimer’s disease

Silke Herzer, Sascha Meldner, Klara Rehder, Hermann-Josef Gröne, Viola Nordström

Open Access Erratum

Erratum to: Multiplex immunoassay characterization and species comparison of inflammation in acute and non-acute ischemic infarcts in human and mouse brain tissue

Thuy-Vi V. Nguyen, Jennifer B. Frye, Jacob C. Zbesko, Kristina Stepanovic, Megan Hayes, Alex Urzua, Geidy Serrano, Thomas G. Beach, Kristian P. Doyle

Open Access Research

Progression of motor neuron disease is accelerated and the ability to recover is compromised with advanced age in rNLS8 mice

Krista J. Spiller, Clark R. Restrepo, Tahiyana Khan, Anna M. Stieber, Linda K. Kwong, John Q. Trojanowski, Virginia M.-Y. Lee

Open Access Research

Evaluation of the brain-penetrant microtubule-stabilizing agent, dictyostatin, in the PS19 tau transgenic mouse model of tauopathy

Vishruti Makani, Bin Zhang, Heeoon Han, Yuemang Yao, Pierrik Lassalas, Kevin Lou, Ian Paterson, Virginia M. Y. Lee, John Q. Trojanowski, Carlo Ballatore, Amos B. Smith III, Kurt R. Brunden

Open Access Research

Clinicopathological characteristics of patients with amyotrophic lateral sclerosis resulting in a totally locked-in state (communication Stage V)

Kentaro Hayashi, Yoko Mochizuki, Ryoko Takeuchi, Toshio Shimizu, Masahiro Nagao, Kazuhiko Watabe, Nobutaka Arai, Kiyomitsu Oyanagi, Osamu Onodera, Masaharu Hayashi, Hitoshi Takahashi, Akiyoshi Kakita, Eiji Isozaki

Open Access Research

Tor1a+/- mice develop dystonia-like movements via a striatal dopaminergic dysregulation triggered by peripheral nerve injury

Chi Wang Ip, Ioannis U. Isaias, Burak B. Kusche-Tekin, Dennis Klein, Janos Groh, Aet O’Leary, Susanne Knorr, Takahiro Higuchi, James B. Koprich, Jonathan M. Brotchie, Klaus V. Toyka, Andreas Reif, Jens Volkmann

Open Access Research

pGluAβ increases accumulation of Aβ in vivo and exacerbates its toxicity

Oyinkan Sofola-Adesakin, Mobina Khericha, Inge Snoeren, Leo Tsuda, Linda Partridge

Open Access Research

Amyloid-β 1–24 C-terminal truncated fragment promotes amyloid-β 1–42 aggregate formation in the healthy brain

Sonia Mazzitelli, Fabia Filipello, Marco Rasile, Eliana Lauranzano, Chiara Starvaggi-Cucuzza, Matteo Tamborini, Davide Pozzi, Isabella Barajon, Toni Giorgino, Antonino Natalello, Michela Matteoli

Open Access Research

Epidermal growth factor prevents APOE4 and amyloid-beta-induced cognitive and cerebrovascular deficits in female mice

Riya Thomas, Paulina Zuchowska, Alan W. J. Morris, Felecia M. Marottoli, Sangeeta Sunny, Ryan Deaton, Peter H. Gann, Leon M. Tai

Open Access Research

Microglial neuroinflammation contributes to tau accumulation in chronic traumatic encephalopathy

Jonathan D. Cherry, Yorghos Tripodis, Victor E. Alvarez, Bertrand Huber, Patrick T. Kiernan, Daniel H. Daneshvar, Jesse Mez, Philip H. Montenigro, Todd M. Solomon, Michael L. Alosco, Robert A. Stern, Ann C. McKee, Thor D. Stein

Open Access Research

Activation of the unfolded protein response and granulovacuolar degeneration are not common features of human prion pathology

Vera I. Wiersma, Wim van Hecke, Wiep Scheper, Martijn A. J. van Osch, Will J. M. Hermsen, Annemieke J. M. Rozemuller, Jeroen J. M. Hoozemans

Open Access Research

Activation of the Keap1/Nrf2 stress response pathway in autophagic vacuolar myopathies

Steve Duleh, Xianhong Wang, Allison Komirenko, Marta Margeta

Open Access Research

Nerve pathologic features differentiate POEMS syndrome from CIDP

Ezequiel A. Piccione, Janean Engelstad, Peter J. Dyck, Michelle L. Mauermann, Angela Dispenzieri, P. James B. Dyck

Open Access Research

Tunneling nanotube (TNT)-mediated neuron-to neuron transfer of pathological Tau protein assemblies

Meryem Tardivel, Séverine Bégard, Luc Bousset, Simon Dujardin, Audrey Coens, Ronald Melki, Luc Buée, Morvane Colin

Open Access Research

Tau pathology in aged cynomolgus monkeys is progressive supranuclear palsy/corticobasal degeneration- but not Alzheimer disease-like -Ultrastructural mapping of tau by EDX-

Toshiki Uchihara, Kentaro Endo, Hiromi Kondo, Sachi Okabayashi, Nobuhiro Shimozawa, Yasuhiro Yasutomi, Eijiro Adachi, Nobuyuki Kimura

Open Access Research

Rubidium and potassium levels are altered in Alzheimer’s disease brain and blood but not in cerebrospinal fluid

Blaine R. Roberts, James D. Doecke, Alan Rembach, L. Fernanda Yévenes, Christopher J. Fowler, Catriona A. McLean, Monica Lind, Irene Volitakis, Colin L. Masters, Ashley I. Bush, Dominic J. Hare

Open Access Case report

An autopsy-confirmed case of progressive supranuclear palsy with predominant postural instability

Carolin Kurz, Georg Ebersbach, Gesine Respondek, Armin Giese, Thomas Arzberger, Günter Ulrich Höglinger

Open Access Review

Review of RyR1 pathway and associated pathomechanisms

Jessica W. Witherspoon, Katherine G. Meilleur

Open Access Research

RETRACTED ARTICLE: Transgenic mice overexpressing the ALS-linked protein Matrin 3 develop a profound muscle phenotype

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

Open Access Letter to the Editor

LRRK2 contributes to monocyte dysregulation in Parkinson’s disease

Corinna Bliederhaeuser, Lisa Zondler, Veselin Grozdanov, Wolfgang P. Ruf, David Brenner, Heather L. Melrose, Peter Bauer, Albert C. Ludolph, Frank Gillardon, Jan Kassubek, Jochen H. Weishaupt, Karin M. Danzer

Open Access Research

Neuronal and glial changes in the brain resulting from explosive blast in an experimental model

James A. Goodrich, Jung H. Kim, Robert Situ, Wesley Taylor, Ted Westmoreland, Fu Du, Steven Parks, Geoffrey Ling, Jung Y. Hwang, Amedeo Rapuano, Faris A. Bandak, Nihal C. de Lanerolle

Open Access Research

The identification of human pituitary adenoma-initiating cells

Branavan Manoranjan, Sujeivan Mahendram, Saleh A. Almenawer, Chitra Venugopal, Nicole McFarlane, Robin Hallett, Thusyanth Vijayakumar, Almunder Algird, Naresh K. Murty, Doron D. Sommer, John P. Provias, Kesava Reddy, Sheila K. Singh

Open Access Research

Dopamine-2 receptor extracellular N-terminus regulates receptor surface availability and is the target of human pathogenic antibodies from children with movement and psychiatric disorders

Nese Sinmaz, Fiona Tea, Deepti Pilli, Alicia Zou, Mazen Amatoury, Tina Nguyen, Vera Merheb, Sudarshini Ramanathan, Sandra T. Cooper, Russell C. Dale, Fabienne Brilot

Open Access Research

Increased Caspase-6 activity in the human anterior olfactory nuclei of the olfactory bulb is associated with cognitive impairment

Benedicte Foveau, Steffen Albrecht, David A. Bennett, José A. Correa, Andrea C. LeBlanc

Open Access Research

The effects of the novel A53E alpha-synuclein mutation on its oligomerization and aggregation

Diana F. Lázaro, Mariana Castro Dias, Anita Carija, Susanna Navarro, Carolina Silva Madaleno, Sandra Tenreiro, Salvador Ventura, Tiago F. Outeiro

Open Access Retraction Note

Retraction Note to: A new inducible transgenic mouse model for C9orf72-associated GGGGCC repeat expansion supports a gain-of-function mechanism in C9orf72-associated ALS and FTD

Renate K. Hukema, Fréderike W. Riemslagh, Shamiram Melhem, Herma C. van der Linde, Lies-Anne W. F. M. Severijnen, Dieter Edbauer, Alex Maas, Nicolas Charlet-Berguerand, Rob Willemsen, John C. van Swieten

Open Access Research

Post-mortem histopathology underlying β-amyloid PET imaging following flutemetamol F 18 injection

Milos D. Ikonomovic, Chris J. Buckley, Kerstin Heurling, Paul Sherwin, Paul A. Jones, Michelle Zanette, Chester A. Mathis, William E. Klunk, Aruna Chakrabarty, James Ironside, Azzam Ismail, Colin Smith, Dietmar R. Thal, Thomas G. Beach, Gill Farrar, Adrian P. L. Smith

Open Access Research

β-Amyloid triggers aberrant over-scaling of homeostatic synaptic plasticity

James Gilbert, Shu Shu, Xin Yang, Youming Lu, Ling-Qiang Zhu, Heng-Ye Man

Leitlinien kompakt für die Neurologie

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Seit 2022 gehört die medbee GmbH zum Springer Medizin Verlag

Akuter Schwindel: Wann lohnt sich eine MRT?

28.04.2024 Schwindel Nachrichten

Akuter Schwindel stellt oft eine diagnostische Herausforderung dar. Wie nützlich dabei eine MRT ist, hat eine Studie aus Finnland untersucht. Immerhin einer von sechs Patienten wurde mit akutem ischämischem Schlaganfall diagnostiziert.

Niedriger diastolischer Blutdruck erhöht Risiko für schwere kardiovaskuläre Komplikationen

25.04.2024 Hypotonie Nachrichten

Wenn unter einer medikamentösen Hochdrucktherapie der diastolische Blutdruck in den Keller geht, steigt das Risiko für schwere kardiovaskuläre Ereignisse: Darauf deutet eine Sekundäranalyse der SPRINT-Studie hin.

Frühe Alzheimertherapie lohnt sich

25.04.2024 AAN-Jahrestagung 2024 Nachrichten

Ist die Tau-Last noch gering, scheint der Vorteil von Lecanemab besonders groß zu sein. Und beginnen Erkrankte verzögert mit der Behandlung, erreichen sie nicht mehr die kognitive Leistung wie bei einem früheren Start. Darauf deuten neue Analysen der Phase-3-Studie Clarity AD.

Viel Bewegung in der Parkinsonforschung

25.04.2024 Parkinson-Krankheit Nachrichten

Neue arznei- und zellbasierte Ansätze, Frühdiagnose mit Bewegungssensoren, Rückenmarkstimulation gegen Gehblockaden – in der Parkinsonforschung tut sich einiges. Auf dem Deutschen Parkinsonkongress ging es auch viel um technische Innovationen.

Update Neurologie

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