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  1. Research

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

    In vivo imaging of fibrillar β-amyloid deposits may assist clinical diagnosis of Alzheimer’s disease (AD), aid treatment selection for patients, assist clinical trials of therapeutic drugs through subject sele...

    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 and Adrian P. L. Smith

    Acta Neuropathologica Communications 2016 4:130

    Published on: 12 December 2016

  2. 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 and John C. van Swieten

    Acta Neuropathologica Communications 2016 4:129

    Published on: 9 December 2016

    The original article was published in Acta Neuropathologica Communications 2014 2:166

  3. Research

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

    α-synuclein (aSyn) is associated with both sporadic and familial forms of Parkinson’s disease (PD), the second most common neurodegenerative disorder after Alzheimer’s disease. In particular, multiplications a...

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

    Acta Neuropathologica Communications 2016 4:128

    Published on: 9 December 2016

  4. Research

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

    Abnormally elevated hippocampal Caspase-6 (Casp6) activity is intimately associated with age-related cognitive impairment in humans and in mice. In humans, these high levels of Casp6 activity are initially loc...

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

    Acta Neuropathologica Communications 2016 4:127

    Published on: 8 December 2016

  5. 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

    Anti-Dopamine-2 receptor (D2R) antibodies have been recently identified in a subgroup of children with autoimmune movement and psychiatric disorders, however the epitope(s) and mechanism of pathogenicity remai...

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

    Acta Neuropathologica Communications 2016 4:126

    Published on: 1 December 2016

  6. Research

    The identification of human pituitary adenoma-initiating cells

    Classified as benign central nervous system (CNS) tumors, pituitary adenomas account for 10% of diagnosed intracranial neoplasms. Although surgery is often curative, patients with invasive macroadenomas contin...

    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 and Sheila K. Singh

    Acta Neuropathologica Communications 2016 4:125

    Published on: 28 November 2016

  7. Research

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

    Mild traumatic brain injury (mTBI) is the signature injury in warfighters exposed to explosive blasts. The pathology underlying mTBI is poorly understood, as this condition is rarely fatal and thus postmortem ...

    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 and Nihal C. de Lanerolle

    Acta Neuropathologica Communications 2016 4:124

    Published on: 24 November 2016

  8. Research

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

    Amyotrophic lateral sclerosis (ALS) is a progressive neurodegenerative disorder of upper and lower motor neurons. Mutations in the gene encoding the nuclear matrix protein Matrin 3 have been found in familial ...

    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 and Jada Lewis

    Acta Neuropathologica Communications 2016 4:122

    Published on: 18 November 2016

  9. Review

    Review of RyR1 pathway and associated pathomechanisms

    Ryanodine receptor isoform-1 (RyR1) is a major calcium channel in skeletal muscle important for excitation-contraction coupling. Mutations in the RYR1 gene yield RyR1 protein dysfunction that manifests clinically...

    Jessica W. Witherspoon and Katherine G. Meilleur

    Acta Neuropathologica Communications 2016 4:121

    Published on: 17 November 2016

  10. Research

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

    Loss of intracellular compartmentalization of potassium is a biochemical feature of Alzheimer’s disease indicating a loss of membrane integrity and mitochondrial dysfunction. We examined potassium and rubidium...

    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 and Dominic J. Hare

    Acta Neuropathologica Communications 2016 4:119

    Published on: 14 November 2016

  11. Case report

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

    Postural instability and supranuclear gaze palsy represent the key symptoms of Richardson’s syndrome, the most frequent clinical manifestation of progressive supranuclear palsy (PSP). However, a proportion of ...

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

    Acta Neuropathologica Communications 2016 4:120

    Published on: 14 November 2016

  12. Research

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

    Concomitant deposition of amyloid -beta protein (Aβ) and neuronal tau as neurofibrillary tangles in the human brain is a hallmark of Alzheimer disease (AD). Because these deposits increase during normal aging,...

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

    Acta Neuropathologica Communications 2016 4:118

    Published on: 14 November 2016

  13. Research

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

    A given cell makes exchanges with its neighbors through a variety of means ranging from diffusible factors to vesicles. Cells use also tunneling nanotubes (TNTs), filamentous-actin-containing membranous struct...

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

    Acta Neuropathologica Communications 2016 4:117

    Published on: 4 November 2016

  14. Research

    Nerve pathologic features differentiate POEMS syndrome from CIDP

    The objective of this study is to determine if the nerve pathology in patients with POEMS syndrome is different from CIDP. We hypothesized that nerve biopsies from patients with POEMS syndrome would have more ...

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

    Acta Neuropathologica Communications 2016 4:116

    Published on: 31 October 2016

  15. Research

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

    Human prion diseases are fatal neurodegenerative disorders with a genetic, sporadic or infectiously acquired aetiology. Neuropathologically, human prion diseases are characterized by deposition of misfolded pr...

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

    Acta Neuropathologica Communications 2016 4:113

    Published on: 28 October 2016

  16. Research

    Microglial neuroinflammation contributes to tau accumulation in chronic traumatic encephalopathy

    The chronic effects of repetitive head impacts (RHI) on the development of neuroinflammation and its relationship to chronic traumatic encephalopathy (CTE) are unknown. Here we set out to determine the relatio...

    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 and Thor D. Stein

    Acta Neuropathologica Communications 2016 4:112

    Published on: 28 October 2016

  17. Research

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

    Cerebrovascular (CV) dysfunction is emerging as a critical component of Alzheimer’s disease (AD), including altered CV coverage. Angiogenic growth factors (AGFs) are key for controlling CV coverage, especially...

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

    Acta Neuropathologica Communications 2016 4:111

    Published on: 27 October 2016

  18. Research

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

    Substantial data indicate that amyloid-β (Aβ), the major component of senile plaques, plays a central role in Alzheimer’s Disease and indeed the assembly of naturally occurring amyloid peptides into cytotoxic ...

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

    Acta Neuropathologica Communications 2016 4:110

    Published on: 10 October 2016

  19. Research

    pGluAβ increases accumulation of Aβ in vivo and exacerbates its toxicity

    Several species of β-amyloid peptides (Aβ) exist as a result of differential cleavage from amyloid precursor protein (APP) to yield various C-terminal Aβ peptides. Several N-terminal modified Aβ peptides have ...

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

    Acta Neuropathologica Communications 2016 4:109

    Published on: 7 October 2016

  20. Research

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

    Isolated generalized dystonia is a central motor network disorder characterized by twisted movements or postures. The most frequent genetic cause is a GAG deletion in the Tor1a (DYT1) gene encoding torsinA with a...

    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 and Jens Volkmann

    Acta Neuropathologica Communications 2016 4:108

    Published on: 3 October 2016

  21. Research

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

    In the present study, we performed a comprehensive analysis to clarify the clinicopathological characteristics of patients with amyotrophic lateral sclerosis (ALS) that had progressed to result in a totally lo...

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

    Acta Neuropathologica Communications 2016 4:107

    Published on: 30 September 2016

  22. Research

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

    In order to treat progressive paralysis in ALS patients, it is critical to develop a mouse that closely models human ALS in both pathology and also in the timing of these events. We have recently generated new...

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

    Acta Neuropathologica Communications 2016 4:105

    Published on: 29 September 2016

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