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  1. Research

    Immunoproteasome deficiency alters microglial cytokine response and improves cognitive deficits in Alzheimer’s disease-like APPPS1 mice

    The immunoproteasome (iP) represents a specialized type of proteasomes, which plays an important role in the clearance of oxidant-damaged proteins under inflammatory and pathological conditions determining the...

    Lisa K. Wagner, Kate E. Gilling, Eileen Schormann, Peter M. Kloetzel, Frank L. Heppner, Elke Krüger and Stefan Prokop

    Acta Neuropathologica Communications 2017 5:52

    Published on: 24 June 2017

  2. Research

    Neonatal AAV delivery of alpha-synuclein induces pathology in the adult mouse brain

    Abnormal accumulation of alpha-synuclein (αsyn) is a pathological hallmark of Lewy body related disorders such as Parkinson’s disease and Dementia with Lewy body disease. During the past two decades, a myriad ...

    Marion Delenclos, Ayman H. Faroqi, Mei Yue, Aishe Kurti, Monica Castanedes-Casey, Linda Rousseau, Virginia Phillips, Dennis W. Dickson, John D. Fryer and Pamela J. McLean

    Acta Neuropathologica Communications 2017 5:51

    Published on: 23 June 2017

  3. Research

    Zika virus tropism and interactions in myelinating neural cell cultures: CNS cells and myelin are preferentially affected

    The recent global outbreak of Zika virus (ZIKV) infection has been linked to severe neurological disorders affecting the peripheral and central nervous systems (PNS and CNS, respectively). The pathobiology und...

    Stephanie L. Cumberworth, Jennifer A. Barrie, Madeleine E. Cunningham, Daniely Paulino Gomes de Figueiredo, Verena Schultz, Adrian J. Wilder-Smith, Benjamin Brennan, Lindomar J. Pena, Rafael Freitas de Oliveira França, Christopher Linington, Susan C. Barnett, Hugh J. Willison, Alain Kohl and Julia M. Edgar

    Acta Neuropathologica Communications 2017 5:50

    Published on: 23 June 2017

  4. Research

    Expression of endogenous mouse APP modulates β-amyloid deposition in hAPP-transgenic mice

    Amyloid-β (Aβ) deposition is one of the hallmarks of the amyloid hypothesis in Alzheimer’s disease (AD). Mouse models using APP-transgene overexpression to generate amyloid plaques have shown to model only cer...

    Johannes Steffen, Markus Krohn, Christina Schwitlick, Thomas Brüning, Kristin Paarmann, Claus U. Pietrzik, Henrik Biverstål, Baiba Jansone, Oliver Langer and Jens Pahnke

    Acta Neuropathologica Communications 2017 5:49

    Published on: 20 June 2017

  5. Research

    Novel oligodendroglial alpha synuclein viral vector models of multiple system atrophy: studies in rodents and nonhuman primates

    Multiple system atrophy (MSA) is a horrible and unrelenting neurodegenerative disorder with an uncertain etiology and pathophysiology. MSA is a unique proteinopathy in which alpha-synuclein (α-syn) accumulates...

    Ronald J. Mandel, David J. Marmion, Deniz Kirik, Yaping Chu, Clifford Heindel, Thomas McCown, Steven J. Gray and Jeffrey H. Kordower

    Acta Neuropathologica Communications 2017 5:47

    Published on: 16 June 2017

  6. Research

    Mechanisms underlying extensive Ser129-phosphorylation in α-synuclein aggregates

    Parkinson’s disease (PD) is characterized neuropathologically by intracellular aggregates of fibrillar α-synuclein, termed Lewy bodies (LBs). Approximately 90% of α-synuclein deposited as LBs is phosphorylated...

    Shigeki Arawaka, Hiroyasu Sato, Asuka Sasaki, Shingo Koyama and Takeo Kato

    Acta Neuropathologica Communications 2017 5:48

    Published on: 15 June 2017

  7. Research

    Brain-derived exosomes from dementia with Lewy bodies propagate α-synuclein pathology

    Proteins implicated in neurodegenerative conditions such as Alzheimer’s disease (AD) and Dementia with Lewy Bodies (DLB) have been identified in bodily fluids encased in extracellular vesicles called exosomes....

    Jennifer Ngolab, Ivy Trinh, Edward Rockenstein, Michael Mante, Jazmin Florio, Margarita Trejo, Deborah Masliah, Anthony Adame, Eliezer Masliah and Robert A. Rissman

    Acta Neuropathologica Communications 2017 5:46

    Published on: 9 June 2017

  8. Research

    Identification and description of three families with familial Alzheimer disease that segregate variants in the SORL1 gene

    Alzheimer disease (AD) is a progressive neurodegenerative disorder and the most common form of dementia. The majority of AD cases are sporadic, while up to 5% are families with an early onset AD (EOAD). Mutati...

    Håkan Thonberg, Huei-Hsin Chiang, Lena Lilius, Charlotte Forsell, Anna-Karin Lindström, Charlotte Johansson, Jenny Björkström, Steinunn Thordardottir, Kristel Sleegers, Christine Van Broeckhoven, Annica Rönnbäck and Caroline Graff

    Acta Neuropathologica Communications 2017 5:43

    Published on: 9 June 2017

  9. Methodology article

    Characterization of tau prion seeding activity and strains from formaldehyde-fixed tissue

    Tauopathies such as Alzheimer’s disease (AD) feature progressive intraneuronal deposition of aggregated tau protein. The cause is unknown, but in experimental systems trans-cellular propagation of tau patholog...

    Sarah K. Kaufman, Talitha L. Thomas, Kelly Del Tredici, Heiko Braak and Marc I. Diamond

    Acta Neuropathologica Communications 2017 5:41

    Published on: 7 June 2017

  10. Research

    Evaluation of a novel antibody to define histone 3.3 G34R mutant brain tumours

    Missense somatic mutations affecting histone H3.1 and H3.3 proteins are now accepted as the hallmark of paediatric diffuse intrinsic pontine gliomas (DIPG), non-brain stem paediatric high grade gliomas (pHGG) ...

    Farhana Haque, Pascale Varlet, Julien Puntonet, Lisa Storer, Aikaterini Bountali, Ruman Rahman, Jacques Grill, Angel M Carcaboso, Chris Jones, Robert Layfield and Richard G Grundy

    Acta Neuropathologica Communications 2017 5:45

    Published on: 6 June 2017

  11. Research

    PLGF, a placental marker of fetal brain defects after in utero alcohol exposure

    Most children with in utero alcohol exposure do not exhibit all features of fetal alcohol syndrome (FAS), and a challenge for clinicians is to make an early diagnosis of fetal alcohol spectrum disorders (FASD)...

    Matthieu Lecuyer, Annie Laquerrière, Soumeya Bekri, Céline Lesueur, Yasmina Ramdani, Sylvie Jégou, Arnaud Uguen, Pascale Marcorelles, Stéphane Marret and Bruno J. Gonzalez

    Acta Neuropathologica Communications 2017 5:44

    Published on: 6 June 2017

  12. Research

    Dysregulation of lysophosphatidic acids in multiple sclerosis and autoimmune encephalomyelitis

    Bioactive lipids contribute to the pathophysiology of multiple sclerosis. Here, we show that lysophosphatidic acids (LPAs) are dysregulated in multiple sclerosis (MS) and are functionally relevant in this dise...

    K. Schmitz, R. Brunkhorst, N. de Bruin, C. A. Mayer, A. Häussler, N. Ferreiros, S. Schiffmann, M. J. Parnham, S. Tunaru, J. Chun, S. Offermanns, C. Foerch, K. Scholich, J. Vogt, S. Wicker, J. Lötsch…

    Acta Neuropathologica Communications 2017 5:42

    Published on: 2 June 2017

  13. Research

    Rho-associated protein kinase 2 (ROCK2): a new target of autoimmunity in paraneoplastic encephalitis

    Onconeural antibodies are associated with cancer and paraneoplastic encephalitis. While their pathogenic role is still largely unknown, their high diagnostic value is undisputed. In this study we describe the ...

    Stoyan Popkirov, Ilya Ayzenberg, Stefanie Hahn, Jan Bauer, Yvonne Denno, Nicole Rieckhoff, Christiane Radzimski, Volkmar H. Hans, Sebastian Berg, Florian Roghmann, Joachim Noldus, Christian G. Bien, Sabine Skodda, Jörg Wellmer, Winfried Stöcker, Christos Krogias…

    Acta Neuropathologica Communications 2017 5:40

    Published on: 29 May 2017

  14. Research

    Multidimensional scaling of diffuse gliomas: application to the 2016 World Health Organization classification system with prognostically relevant molecular subtype discovery

    Recent updating of the World Health Organization (WHO) classification of central nervous system (CNS) tumors in 2016 demonstrates the first organized effort to restructure brain tumor classification by incorpo...

    Patrick J. Cimino, Michael Zager, Lisa McFerrin, Hans-Georg Wirsching, Hamid Bolouri, Bettina Hentschel, Andreas von Deimling, David Jones, Guido Reifenberger, Michael Weller and Eric C. Holland

    Acta Neuropathologica Communications 2017 5:39

    Published on: 22 May 2017

  15. Research

    Higher levels of myelin phospholipids in brains of neuronal α-Synuclein transgenic mice precede myelin loss

    α-Synuclein is a protein involved in the pathogenesis of synucleinopathies, including Parkinson’s disease (PD), dementia with Lewy bodies (DLB) and multiple system atrophy (MSA). We investigated the role of ne...

    Jessica Grigoletto, Katharina Pukaß, Ayelet Gamliel, Dana Davidi, Rachel Katz-Brull, Christiane Richter-Landsberg and Ronit Sharon

    Acta Neuropathologica Communications 2017 5:37

    Published on: 8 May 2017

  16. Research

    Hydrocephalus due to multiple ependymal malformations is caused by mutations in the MPDZ gene

    Congenital hydrocephalus is considered as either acquired due to haemorrhage, infection or neoplasia or as of developmental nature and is divided into two subgroups, communicating and obstructive. Congenital h...

    Pascale Saugier-Veber, Florent Marguet, François Lecoquierre, Homa Adle-Biassette, Fabien Guimiot, Sara Cipriani, Sophie Patrier, Marie Brasseur-Daudruy, Alice Goldenberg, Valérie Layet, Yline Capri, Marion Gérard, Thierry Frébourg and Annie Laquerrière

    Acta Neuropathologica Communications 2017 5:36

    Published on: 1 May 2017

  17. Letter to the Editor

    Response to Simon et al.,

    Wei Wei, Michael J. Keogh, James W. Ironside and Patrick F. Chinnery

    Acta Neuropathologica Communications 2017 5:34

    Published on: 29 April 2017

  18. Research

    Altered Ca2+ homeostasis induces Calpain-Cathepsin axis activation in sporadic Creutzfeldt-Jakob disease

    Sporadic Creutzfeldt-Jakob disease (sCJD) is the most prevalent form of human prion disease and it is characterized by the presence of neuronal loss, spongiform degeneration, chronic inflammation and the accum...

    Franc Llorens, Katrin Thüne, Beata Sikorska, Matthias Schmitz, Waqas Tahir, Natalia Fernández-Borges, Maria Cramm, Nadine Gotzmann, Margarita Carmona, Nathalie Streichenberger, Uwe Michel, Saima Zafar, Anna-Lena Schuetz, Ashish Rajput, Olivier Andréoletti, Stefan Bonn…

    Acta Neuropathologica Communications 2017 5:35

    Published on: 27 April 2017

  19. Research

    Enhanced neuroinvasion by smaller, soluble prions

    Infectious prion aggregates can propagate from extraneural sites into the brain with remarkable efficiency, likely transported via peripheral nerves. Yet not all prions spread into the brain, and the physical ...

    Cyrus Bett, Jessica Lawrence, Timothy D. Kurt, Christina Orru, Patricia Aguilar-Calvo, Anthony E. Kincaid, Witold K. Surewicz, Byron Caughey, Chengbiao Wu and Christina J. Sigurdson

    Acta Neuropathologica Communications 2017 5:32

    Published on: 21 April 2017

  20. Research

    Heterogeneous ribonuclear protein A3 (hnRNP A3) is present in dipeptide repeat protein containing inclusions in Frontotemporal Lobar Degeneration and Motor Neurone disease associated with expansions in C9orf72 gene

    Frontotemporal Lobar Degeneration (FTLD) encompasses certain related neurodegenerative disorders which alter behaviour, personality and language. Heterogeneous ribonuclear proteins (hnRNPs) maintain RNA metabo...

    Yvonne S. Davidson, Louis Flood, Andrew C. Robinson, Yoshihiro Nihei, Kohji Mori, Sara Rollinson, Anna Richardson, Bridget C. Benson, Matthew Jones, Julie S. Snowden, Stuart Pickering-Brown, Christian Haass, Tammaryn Lashley and David M. A. Mann

    Acta Neuropathologica Communications 2017 5:31

    Published on: 21 April 2017

  21. Research

    Tau phosphorylation induced by severe closed head traumatic brain injury is linked to the cellular prion protein

    Studies in vivo and in vitro have suggested that the mechanism underlying Alzheimer’s disease (AD) neuropathogenesis is initiated by an interaction between the cellular prion protein (PrPC) and amyloid-β oligomer...

    Richard Rubenstein, Binggong Chang, Natalia Grinkina, Eleanor Drummond, Peter Davies, Meir Ruditzky, Deep Sharma, Kevin Wang and Thomas Wisniewski

    Acta Neuropathologica Communications 2017 5:30

    Published on: 18 April 2017

  22. Research

    Bidirectional nucleolar dysfunction in C9orf72 frontotemporal lobar degeneration

    An intronic GGGGCC expansion in C9orf72 is the most common known cause of both frontotemporal lobar degeneration (FTLD) and amyotrophic lateral sclerosis (ALS). The repeat expansion leads to the generation of sen...

    Sarah Mizielinska, Charlotte E. Ridler, Rubika Balendra, Annora Thoeng, Nathan S. Woodling, Friedrich A. Grässer, Vincent Plagnol, Tammaryn Lashley, Linda Partridge and Adrian M. Isaacs

    Acta Neuropathologica Communications 2017 5:29

    Published on: 18 April 2017

  23. Research

    Detection of Histone H3 mutations in cerebrospinal fluid-derived tumor DNA from children with diffuse midline glioma

    Diffuse midline gliomas (including diffuse intrinsic pontine glioma, DIPG) are highly morbid glial neoplasms of the thalamus or brainstem that typically arise in young children and are not surgically resectabl...

    Tina Y. Huang, Andrea Piunti, Rishi R. Lulla, Jin Qi, Craig M. Horbinski, Tadanori Tomita, C. David James, Ali Shilatifard and Amanda M. Saratsis

    Acta Neuropathologica Communications 2017 5:28

    Published on: 17 April 2017

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