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  1. Content type: Research

    We have sought histological evidence, using TDP-43 and p62 immunohistochemistry, for the presence of expansions in C9ORF72 among 200 patients with pathologically confirmed AD.

    Authors: Yvonne S Davidson, Andrew C Robinson, Julie S Snowden and David MA Mann

    Citation: Acta Neuropathologica Communications 2013 1:50

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  2. Content type: Research

    TAR DNA-binding protein 43 (TDP-43) is a protein that is involved in the pathology of Amyotrophic Lateral Sclerosis (ALS) and Frontotemporal Lobar Degeneration (FTLD). In patients with these neurodegenerative ...

    Authors: Rui Liu, Guang Yang, Takashi Nonaka, Tetsuaki Arai, William Jia and Max S Cynader

    Citation: Acta Neuropathologica Communications 2013 1:49

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  3. Content type: Research

    Basement membranes in the walls of cerebral capillaries and arteries form a major lymphatic drainage pathway for fluid and solutes from the brain. Amyloid-β (Aβ) draining from the brain is deposited in such pe...

    Authors: Roxana Octavia Carare, Jessica Liesbeth Teeling, Cheryl A Hawkes, Ursula Püntener, Roy O Weller, James AR Nicoll and Victor Hugh Perry

    Citation: Acta Neuropathologica Communications 2013 1:48

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  4. Content type: Research

    Focal cortical dysplasias (FCD) are local disturbances of neocortical architecture and a common cause of pharmaco-resistant focal epilepsy. Little is known about the pathomechanisms leading to architectural ab...

    Authors: Susanne Fauser, Ute Häussler, Catharina Donkels, Susanne Huber, Julia Nakagawa, Marco Prinz, Andreas Schulze-Bonhage, Josef Zentner and Carola A Haas

    Citation: Acta Neuropathologica Communications 2013 1:47

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  5. Content type: Research

    Presenilin-1 (PS1) is the active component of the amyloid precursor protein cleaving γ-secretase complex. PS1 protein is a transmembrane protein containing multiple hydrophobic regions which presence in cerebr...

    Authors: María-Salud García-Ayllón, María-Letizia Campanari, Gunnar Brinkmalm, Alberto Rábano, Jordi Alom, Carlos A Saura, Niels Andreasen, Kaj Blennow and Javier Sáez-Valero

    Citation: Acta Neuropathologica Communications 2013 1:46

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  6. Content type: Case report

    Monosomy 1p36 is the most common subtelomeric chromosomal deletion linked to mental retardation and seizures. Neuroimaging studies suggest that monosomy 1p36 is associated with brain malformations including po...

    Authors: Naoko Shiba, Ray AM Daza, Lisa G Shaffer, A James Barkovich, William B Dobyns and Robert F Hevner

    Citation: Acta Neuropathologica Communications 2013 1:45

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  7. Content type: Methodology article

    PrPSc, the only known constituent of prions, the infectious agents causing prion diseases, can be detected by real-time quaking-induced conversion (RT-QuIC). However, there is no efficient method to quantify the ...

    Authors: Song Shi, Gerda Mitteregger-Kretzschmar, Armin Giese and Hans A Kretzschmar

    Citation: Acta Neuropathologica Communications 2013 1:44

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  8. Content type: Research

    Foamy macrophages, containing myelin degradation products, are abundantly found in active multiple sclerosis (MS) lesions. Recent studies have described an altered phenotype of macrophages after myelin interna...

    Authors: Jeroen FJ Bogie, Winde Jorissen, Jo Mailleux, Philip G Nijland, Noam Zelcer, Tim Vanmierlo, Jack Van Horssen, Piet Stinissen, Niels Hellings and Jerome JA Hendriks

    Citation: Acta Neuropathologica Communications 2013 1:43

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  9. Content type: Research

    A pathological hallmark of most amyotrophic lateral sclerosis (ALS) cases are intracellular aggregates of the protein TDP-43. The pathophysiological relevance of TDP-43 is underlined by familial ALS cases caus...

    Authors: Axel Freischmidt, Kathrin Müller, Albert C Ludolph and Jochen H Weishaupt

    Citation: Acta Neuropathologica Communications 2013 1:42

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  10. Content type: Research

    Precocious development of Alzheimer-type neuropathological changes in epilepsy patients, especially in APOE ϵ4,4 carriers is well known, but not the ways in which other APOE allelic combinations influence this ou...

    Authors: Orwa Aboud, Robert E Mrak, Frederick A Boop and W Sue T Griffin

    Citation: Acta Neuropathologica Communications 2013 1:41

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  11. Content type: Research

    Central pontine myelinolysis (CPM) is a demyelinating disorder of the central basis pontis that is often associated with osmotic stress. The aquaporin water channels (AQPs) have been pathogenically implicated ...

    Authors: Bogdan F Gh Popescu, Reem F Bunyan, Yong Guo, Joseph E Parisi, Vanda A Lennon and Claudia F Lucchinetti

    Citation: Acta Neuropathologica Communications 2013 1:40

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  12. Content type: Research

    Aberrant neuron/glia interactions can contribute to a variety of neurodegenerative diseases and we have previously demonstrated that enhanced activation of Erb B2, which is a member of the epidermal growth fac...

    Authors: Pan Pan and Rick T Dobrowsky

    Citation: Acta Neuropathologica Communications 2013 1:39

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  13. Content type: Research

    α-Synuclein (αS) is the major component of several types of brain inclusions including Lewy bodies, a hallmark of Parkinson’s disease. Aberrant aggregation of αS also is associated with cellular demise in mult...

    Authors: Amanda N Sacino, Mieu Brooks, Nicholas H McGarvey, Alex B McKinney, Michael A Thomas, Yona Levites, Yong Ran, Todd E Golde and Benoit I Giasson

    Citation: Acta Neuropathologica Communications 2013 1:38

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  14. Content type: Research

    Calreticulin (CRT) is a chaperone protein, which aids correct folding of glycosylated proteins in the endoplasmic reticulum (ER). Under conditions of ER stress, CRT is upregulated and may be displayed on the s...

    Authors: Mary Ní Fhlathartaigh, Jill McMahon, Richard Reynolds, David Connolly, Eibhlín Higgins, Timothy Counihan and Una FitzGerald

    Citation: Acta Neuropathologica Communications 2013 1:37

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  15. Content type: Research

    Frontotemporal lobar degeneration (FTLD) is the second most common cause of dementia in individuals under 65 years old and manifests as alterations in behavior, personality, or language secondary to degenerati...

    Authors: Johanna I Busch, Maria Martinez-Lage, Emily Ashbridge, Murray Grossman, Vivianna M Van Deerlin, Fenghua Hu, Virginia MY Lee, John Q Trojanowski and Alice S Chen-Plotkin

    Citation: Acta Neuropathologica Communications 2013 1:36

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  16. Content type: Research

    Medulloblastomas, the most frequent malignant brain tumours affecting children, comprise at least 4 distinct clinicogenetic subgroups. Aberrant sonic hedgehog (SHH) signalling is observed in approximately 25% ...

    Authors: Maria Łastowska, Hani Al-Afghani, Haya H Al-Balool, Harsh Sheth, Emma Mercer, Jonathan M Coxhead, Chris PF Redfern, Heiko Peters, Alastair D Burt, Mauro Santibanez-Koref, Chris M Bacon, Louis Chesler, Alistair G Rust, David J Adams, Daniel Williamson, Steven C Clifford…

    Citation: Acta Neuropathologica Communications 2013 1:35

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  17. Content type: Research

    Tau is a microtubule stabilizing protein and is mainly expressed in neurons. Tau aggregation into oligomers and tangles is considered an important pathological event in tauopathies, such as frontotemporal deme...

    Authors: Josien Levenga, Pavan Krishnamurthy, Hameetha Rajamohamedsait, Helen Wong, Thomas F Franke, Peter Cain, Einar M Sigurdsson and Charles A Hoeffer

    Citation: Acta Neuropathologica Communications 2013 1:34

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  18. Content type: Research

    Two commercially available TDP43 antibodies (phosphorylated or pTDP43, non-phosphorylated or iTDP43) are currently in use for the neuropathological classification of FTLD-TDP cases into pathological subtypes. ...

    Authors: Rachel H Tan, Claire E Shepherd, Jillian J Kril, Heather McCann, Andrew McGeachie, Ciara McGinley, Andrew Affleck and Glenda M Halliday

    Citation: Acta Neuropathologica Communications 2013 1:33

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  19. Content type: Research

    Neuroinflammation regulates both disease pathogenesis and repair in multiple sclerosis. In early multiple sclerosis lesion development, neuroinflammation causes demyelination and axonal injury, the likely fina...

    Authors: Vilija G Jokubaitis, Melissa M Gresle, Dennis A Kemper, William Doherty, Victoria M Perreau, Tania L Cipriani, Anna Jonas, Gerry Shaw, Tanja Kuhlmann, Trevor J Kilpatrick and Helmut Butzkueven

    Citation: Acta Neuropathologica Communications 2013 1:32

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  20. Content type: Research

    Progressive supranuclear palsy (PSP) is a neurodegenerative disorder pathologically characterized by intracellular tangles of hyperphosphorylated tau protein distributed throughout the neocortex, basal ganglia...

    Authors: Lauren D Stutzbach, Sharon X Xie, Adam C Naj, Roger Albin, Sid Gilman, Virginia M Y Lee, John Q Trojanowski, Bernie Devlin and Gerard D Schellenberg

    Citation: Acta Neuropathologica Communications 2013 1:31

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  21. Content type: Research

    Early atrophy of the cingulate cortex is a feature of both behavioral variant frontotemporal dementia (bvFTD) and Alzheimer’s disease (AD), with degeneration of the anterior cingulate region increasingly recog...

    Authors: Rachel H Tan, Karen Pok, Stephanie Wong, Daniel Brooks, Glenda M Halliday and Jillian J Kril

    Citation: Acta Neuropathologica Communications 2013 1:30

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  22. Content type: Research

    Inclusion body myositis (IBM) is a slowly progressive inflammatory myopathy of the elderly that does not show significant clinical improvement in response to steroid therapy. Distinguishing IBM from polymyosit...

    Authors: Annie Hiniker, Brianne H Daniels, Han S Lee and Marta Margeta

    Citation: Acta Neuropathologica Communications 2013 1:29

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  23. Content type: Research

    A critical and as-yet unmet need in Alzheimer disease (AD) research is the development of novel markers that can identify individuals at risk for cognitive decline due to AD. This would aid intervention trials...

    Authors: Alison A Motsinger-Reif, Hongjie Zhu, Mitchel A Kling, Wayne Matson, Swati Sharma, Oliver Fiehn, David M Reif, Dina H Appleby, P Murali Doraiswamy, John Q Trojanowski, Rima Kaddurah-Daouk and Steven E Arnold

    Citation: Acta Neuropathologica Communications 2013 1:28

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  24. Content type: Research

    Reelin and its downstream signaling members are important modulators of actin and microtubule cytoskeleton dynamics, a fundamental prerequisite for proper neurodevelopment and adult neuronal functions. Reducti...

    Authors: Tina Notter and Irene Knuesel

    Citation: Acta Neuropathologica Communications 2013 1:27

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  25. Content type: Research

    Friedreich ataxia (FA) causes distinctive lesions of dorsal root ganglia (DRG), including neuronal atrophy, satellite cell hyperplasia, and absorption of dying nerve cells into residual nodules. Two mechanisms...

    Authors: Arnulf H Koeppen, Erik C Kuntzsch, Sarah T Bjork, R Liane Ramirez, Joseph E Mazurkiewicz and Paul J Feustel

    Citation: Acta Neuropathologica Communications 2013 1:26

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  26. Content type: Research

    In some prion diseases, misfolded aggregated protease-resistant prion protein (PrPres) is found in brain as amyloid, which can cause cerebral amyloid angiopathy. Small diffusible precursors of PrPres amyloid m...

    Authors: Alejandra Rangel, Brent Race, Mikael Klingeborn, James Striebel and Bruce Chesebro

    Citation: Acta Neuropathologica Communications 2013 1:25

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  27. Content type: Research

    Protein aggregation and the formation of intracellular inclusions are a central feature of many neurodegenerative disorders, but precise knowledge about their pathogenic role is lacking in most instances. Here...

    Authors: Marijn Kuijpers, Vera van Dis, Elize D Haasdijk, Martin Harterink, Karin Vocking, Jan A Post, Wiep Scheper, Casper C Hoogenraad and Dick Jaarsma

    Citation: Acta Neuropathologica Communications 2013 1:24

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  28. Content type: Research

    In utero exposure of the fetal non-human primate (NHP) brain to alcohol on a single occasion during early or late third-trimester gestation triggers widespread acute apoptotic death of cells in both gray and whi...

    Authors: Catherine E Creeley, Krikor T Dikranian, Stephen A Johnson, Nuri B Farber and John W Olney

    Citation: Acta Neuropathologica Communications 2013 1:23

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  29. Content type: Methodology article

    Our aim was to develop a new protocol for MGMT immunohistochemistry with good agreement between observers and good correlation with molecular genetic tests of tumour methylation. We examined 40 primary brain t...

    Authors: Elinor Burke, Mariana Grobler, Kay Elderfield, Frances Bond, Matthew Crocker, Rohan Taylor and Leslie R Bridges

    Citation: Acta Neuropathologica Communications 2013 1:22

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  30. Content type: Research

    Emerging evidence suggests that innate immunity and increased oxidative stress contribute to pathomechanisms in amyotrophic lateral sclerosis (ALS). The aim of the present study was to verify the involvement o...

    Authors: Motoko Kawaguchi-Niida, Tomoko Yamamoto, Yoichiro Kato, Yuri Inose and Noriyuki Shibata

    Citation: Acta Neuropathologica Communications 2013 1:21

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  31. Content type: Methodology article

    High frequencies of the BRAF V600E mutation have been reported in pleomorphic xanthoastrocytoma (PXA). Recently, a BRAF V600E mutation-specific antibody has been developed and validated. We evaluated the immun...

    Authors: Cristiane M Ida, Julie A Vrana, Fausto J Rodriguez, Mark E Jentoft, Alissa A Caron, Sarah M Jenkins and Caterina Giannini

    Citation: Acta Neuropathologica Communications 2013 1:20

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  32. Content type: Research

    Medulloblastoma is a leading cause of childhood cancer-related deaths. Current aggressive treatments frequently lead to cognitive and neurological disabilities in survivors. Novel targeted therapies are requir...

    Authors: Kristian W Pajtler, Christina Weingarten, Theresa Thor, Annette Künkele, Lukas C Heukamp, Reinhard Büttner, Takayoshi Suzuki, Naoki Miyata, Michael Grotzer, Anja Rieb, Annika Sprüssel, Angelika Eggert, Alexander Schramm and Johannes H Schulte

    Citation: Acta Neuropathologica Communications 2013 1:19

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  33. Content type: Research

    Point mutations in genes encoding NADP+-dependent isocitrate dehydrogenases (especially IDH1) are common in lower grade diffuse gliomas and secondary glioblastomas and occur early during tumor development. The co...

    Authors: Anna C Navis, Simone P Niclou, Fred Fack, Daniel Stieber, Sanne van Lith, Kiek Verrijp, Alan Wright, Jonathan Stauber, Bastiaan Tops, Irene Otte-Holler, Ron A Wevers, Arno van Rooij, Stefan Pusch, Andreas von Deimling, Wikky Tigchelaar, Cornelis JF van Noorden…

    Citation: Acta Neuropathologica Communications 2013 1:18

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  34. Content type: Research

    Pilocytic astrocytomas occur predominantly in childhood. In contrast to the posterior fossa location, hypothalamo-chiasmatic pilocytic astrocytomas display a worse prognosis often leading to multiple surgical ...

    Authors: Sandy Mercurio, Laetitia Padovani, Carole Colin, Manon Carré, Aurélie Tchoghandjian, Didier Scavarda, Sally Lambert, Nathalie Baeza-Kallee, Carla Fernandez, Céline Chappé, Nicolas André and Dominique Figarella-Branger

    Citation: Acta Neuropathologica Communications 2013 1:17

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  35. Content type: Research

    Frontotemporal lobar degeneration (FTLD) is a heterogeneous group of neurodegenerative diseases associated with personality changes and progressive dementia. Loss-of-function mutations in the growth factor pro...

    Authors: Julia Banzhaf-Strathmann, Rainer Claus, Oliver Mücke, Kristin Rentzsch, Julie van der Zee, Sebastiaan Engelborghs, Peter P De Deyn, Marc Cruts, Christine van Broeckhoven, Christoph Plass and Dieter Edbauer

    Citation: Acta Neuropathologica Communications 2013 1:16

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  36. Content type: Research

    A reduction in peripheral nervous system (PNS) insulin signaling is a proposed mechanism that may contribute to sensory neuron dysfunction and diabetic neuropathy. Neuronal insulin resistance is associated wit...

    Authors: Caleb W Grote, Anna L Groover, Janelle M Ryals, Paige C Geiger, Eva L Feldman and Douglas E Wright

    Citation: Acta Neuropathologica Communications 2013 1:15

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  37. Content type: Research

    White matter hyperintensities (WMH) lesions on T2/FLAIR brain MRI are frequently seen in healthy elderly people. Whether these radiological lesions correspond to irreversible histological changes is still a ma...

    Authors: Sven Haller, Enikö Kövari, François R Herrmann, Victor Cuvinciuc, Ann-Marie Tomm, Gilbert B Zulian, Karl-Olof Lovblad, Panteleimon Giannakopoulos and Constantin Bouras

    Citation: Acta Neuropathologica Communications 2013 1:14

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  38. Content type: Research

    Environmental toxins are suspected to play a role in the pathogenesis of amyotrophic lateral sclerosis (ALS). In an attempt to determine which pathways these toxins can use to enter motor neurons we compared t...

    Authors: Roger Pamphlett and Stephen Kum Jew

    Citation: Acta Neuropathologica Communications 2013 1:13

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  39. Content type: Case report

    Infantile neuroaxonal dystrophy (INAD) is a rare autosomal-recessive neurodegenerative disorder. Patients with INAD usually show neurological symptoms with infant onset and die in childhood. Recently, it was r...

    Authors: Yuichi Riku, Takeshi Ikeuchi, Hiroyo Yoshino, Maya Mimuro, Kazuo Mano, Yoji Goto, Nobutaka Hattori, Gen Sobue and Mari Yoshida

    Citation: Acta Neuropathologica Communications 2013 1:12

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  40. Content type: Research

    ATP13A2 (PARK9) loss of function mutations are a genetic cause of an early-onset form of Parkinson’s disease (PD), with in vitro studies showing that ATP13A2 deficits lead to lysosomal and mitochondrial dysfunct...

    Authors: Karen E Murphy, Louise Cottle, Amanda M Gysbers, Antony A Cooper and Glenda M Halliday

    Citation: Acta Neuropathologica Communications 2013 1:11

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  41. Content type: Research

    Solitary Fibrous Tumours (SFT) and haemangiopericytomas (HPC) are rare meningeal tumours that have to be distinguished from meningiomas and more rarely from synovial sarcomas. We recently found that ALDH1A1 was o...

    Authors: Corinne Bouvier, François Bertucci, Philippe Métellus, Pascal Finetti, André Maues de Paula, Fabien Forest, Karima Mokhtari, Catherine Miquel, Daniel Birnbaum, Alexandre Vasiljevic, Anne Jouvet, Jean-Michel Coindre, Anderson Loundou and Dominique Figarella-Branger

    Citation: Acta Neuropathologica Communications 2013 1:10

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  42. Content type: Research

    Multiple neurodegenerative diseases are characterized by the abnormal accumulation of FUS protein including various subtypes of frontotemporal lobar degeneration with FUS inclusions (FTLD-FUS). These subtypes ...

    Authors: Edward B Lee, Jenny Russ, Hyunjoo Jung, Lauren B Elman, Lama M Chahine, Daniel Kremens, Bruce L Miller, H Branch Coslett, John Q Trojanowski, Vivianna M Van Deerlin and Leo F McCluskey

    Citation: Acta Neuropathologica Communications 2013 1:9

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  43. Content type: Research

    Sporadic, inherited and acquired prion diseases show distinct histological patterns of abnormal prion protein (PrP) deposits. Many of the inherited prion diseases show striking histological patterns, which oft...

    Authors: Lilla Reiniger, Ilaria Mirabile, Ana Lukic, Jonathan DF Wadsworth, Jacqueline M Linehan, Michael Groves, Jessica Lowe, Ronald Druyeh, Peter Rudge, John Collinge, Simon Mead and Sebastian Brandner

    Citation: Acta Neuropathologica Communications 2013 1:8

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  44. Content type: Research

    Neuron Glial 2 (NG2) cells are glial cells known to serve as oligodendrocyte progenitors as well as modulators of the neuronal network. Altered NG2 cell morphology and up-regulation as well as increased sheddi...

    Authors: Henrietta M Nielsen, Danyal Ek, Una Avdic, Camilla Orbjörn, Oskar Hansson, Robert Veerhuis, Annemieke JM Rozemuller, Arne Brun, Lennart Minthon and Malin Wennström

    Citation: Acta Neuropathologica Communications 2013 1:7

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  45. Content type: Methodology article

    Rat models of Parkinson’s disease are widely used to elucidate the mechanisms underlying disease etiology or to investigate therapeutic approaches. Models were developed using toxins such as MPTP or 6-OHDA to ...

    Authors: Hemi Dimant, Suneil K Kalia, Lorraine V Kalia, Liya N Zhu, Laura Kibuuka, Darius Ebrahimi-Fakhari, Nikolaus R McFarland, Zhanyun Fan, Bradley T Hyman and Pamela J McLean

    Citation: Acta Neuropathologica Communications 2013 1:6

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  46. Content type: Research

    Neuromyelitis optica (NMO) is a severe, disabling disease of the central nervous system (CNS) characterized by the formation of astrocyte-destructive, neutrophil-dominated inflammatory lesions in the spinal co...

    Authors: Maja Kitic, Sonja Hochmeister, Isabella Wimmer, Jan Bauer, Tatsuro Misu, Simone Mader, Markus Reindl, Kazuo Fujihara, Hans Lassmann and Monika Bradl

    Citation: Acta Neuropathologica Communications 2013 1:5

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  47. Content type: Research

    To investigate the association of DNA nucleotide excision repair (NER) defects with neurological degeneration, cachexia and cancer, we performed autopsies on 4 adult xeroderma pigmentosum (XP) patients with di...

    Authors: Jin-Ping Lai, Yen-Chun Liu, Meghna Alimchandani, Qingyan Liu, Phyu Phyu Aung, Kant Matsuda, Chyi-Chia R Lee, Maria Tsokos, Stephen Hewitt, Elisabeth J Rushing, Deborah Tamura, David L Levens, John J DiGiovanna, Howard A Fine, Nicholas Patronas, Sikandar G Khan…

    Citation: Acta Neuropathologica Communications 2013 1:4

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  48. Content type: Research

    The development of disease-modifying therapies for Alzheimer’s disease is hampered by our lack of understanding of the early pathogenic mechanisms and the lack of early biomarkers and risk factors.

    Authors: Sharon C Yates, Amen Zafar, Paul Hubbard, Sheila Nagy, Sarah Durant, Roy Bicknell, Gordon Wilcock, Sharon Christie, Margaret M Esiri, A David Smith and Zsuzsanna Nagy

    Citation: Acta Neuropathologica Communications 2013 1:3

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  49. Content type: Review

    Parkinson’s disease (PD) is a progressive neurodegenerative disorder typified by the presence of intraneuronal inclusions containing aggregated alpha synuclein (αsyn). The progression of parkinsonian pathology...

    Authors: Naomi P Visanji, Patricia L Brooks, Lili-Naz Hazrati and Anthony E Lang

    Citation: Acta Neuropathologica Communications 2013 1:2

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