Issue 1/2014
Content (143 Articles)
The value of muscle biopsies in Pompe disease: identifying lipofuscin inclusions in juvenile- and adult-onset patients
Erin J Feeney, Stephanie Austin, Yin-Hsiu Chien, Hanna Mandel, Benedikt Schoser, Sean Prater, Wuh-Liang Hwu, Evelyn Ralston, Priya S Kishnani, Nina Raben
SIRT1 Activating compounds reduce oxidative stress mediated neuronal loss in viral induced CNS demyelinating disease
Reas S Khan, Kimberly Dine, Jayasri Das Sarma, Kenneth S Shindler
Generation of iPSC lines from archived non-cryoprotected biobanked dura mater
Andrew A Sproul, Lauren B Vensand, Carmen R Dusenberry, Samson Jacob, Jean Paul G Vonsattel, Daniel J Paull, Michael L Shelanski, John F Crary, Scott A Noggle
A new animal model of spontaneous autoimmune peripheral polyneuropathy: implications for Guillain-Barré syndrome
Mu Yang, Anthony Rainone, Xiang Qun Shi, Sylvie Fournier, Ji Zhang
Metabolic alterations due to IDH1 mutation in glioma: opening for therapeutic opportunities?
Dana A N Mustafa, Sigrid M Swagemakers, Laura Buise, Peter J van der Spek, Johan M Kros
Two cases of multinodular and vacuolating neuronal tumour
Istvan Bodi, Olimpia Curran, Richard Selway, Robert Elwes, Juan Burrone, Ross Laxton, Safa Al-Sarraj, Mrinalini Honavar
Distinct patterns of spread of prion infection in brains of mice expressing anchorless or anchored forms of prion protein
Alejandra Rangel, Brent Race, Katie Phillips, James Striebel, Nancy Kurtz, Bruce Chesebro
Loss of the neuroprotective factor Sphingosine 1-phosphate early in Alzheimer’s disease pathogenesis
Timothy A Couttas, Nupur Kain, Benjamin Daniels, Xin Ying Lim, Claire Shepherd, Jillian Kril, Russell Pickford, Hongyun Li, Brett Garner, Anthony S Don
Reduced sphingosine kinase-1 and enhanced sphingosine 1-phosphate lyase expression demonstrate deregulated sphingosine 1-phosphate signaling in Alzheimer’s disease
Johnatan Ceccom, Najat Loukh, Valérie Lauwers-Cances, Christian Touriol, Yvan Nicaise, Catherine Gentil, Emmanuelle Uro-Coste, Stuart Pitson, Claude Alain Maurage, Charles Duyckaerts, Olivier Cuvillier, Marie-Bernadette Delisle
Dendritic retraction, but not atrophy, is consistent in amyotrophic lateral sclerosis-comparison between Onuf’s neurons and other sacral motor neurons-
Takahiro Takeda, Toshiki Uchihara, Yuki Nakayama, Ayako Nakamura, Shoichi Sasaki, Shinji Kakei, Shinichiro Uchiyama, Charles Duyckaerts, Mari Yoshida
Neuron-to-neuron wild-type Tau protein transfer through a trans-synaptic mechanism: relevance to sporadic tauopathies
Simon Dujardin, Katia Lécolle, Raphaëlle Caillierez, Séverine Bégard, Nadège Zommer, Cédrick Lachaud, Sébastien Carrier, Noëlle Dufour, Gwennaëlle Aurégan, Joris Winderickx, Philippe Hantraye, Nicole Déglon, Morvane Colin, Luc Buée
Lipid dysfunction and pathogenesis of multiple system atrophy
Jonathan M Bleasel, Joanna H Wong, Glenda M Halliday, Woojin Scott Kim
Polycomb group gene BMI1 controls invasion of medulloblastoma cells and inhibits BMP-regulated cell adhesion
Ashirwad Merve, Adrian M Dubuc, Xinyu Zhang, Marc Remke, Patricia A Baxter, Xiao-Nan Li, Michael D Taylor, Silvia Marino
Posterior fossa and spinal gangliogliomas form two distinct clinicopathologic and molecular subgroups
Kirti Gupta, Wilda Orisme, Julie H Harreld, Ibrahim Qaddoumi, James D Dalton, Chandanamali Punchihewa, Racquel Collins-Underwood, Thomas Robertson, Ruth G Tatevossian, David W Ellison
Lesion of the subiculum reduces the spread of amyloid beta pathology to interconnected brain regions in a mouse model of Alzheimer’s disease
Sonia George, Annica Rönnbäck, Gunnar K Gouras, Géraldine H Petit, Fiona Grueninger, Bengt Winblad, Caroline Graff, Patrik Brundin
Autophagy-lysosome pathway associated neuropathology and axonal degeneration in the brains of alpha-galactosidase A-deficient mice
Michael P Nelson, Tonia E Tse, Darrel B O’Quinn, Stefanie M Percival, Edgar A Jaimes, David G Warnock, John J Shacka
D-2-Hydroxyglutarate producing neo-enzymatic activity inversely correlates with frequency of the type of isocitrate dehydrogenase 1 mutations found in glioma
Stefan Pusch, Leonille Schweizer, Ann-Christin Beck, Johanna-Marie Lehmler, Susanne Weissert, Jörg Balss, Aubry K Miller, Andreas von Deimling
The prognostic role of intragenic copy number breakpoints and identification of novel fusion genes in paediatric high grade glioma
Diana Carvalho, Alan Mackay, Lynn Bjerke, Richard G Grundy, Celeste Lopes, Rui M Reis, Chris Jones
Mechanism of metabolic stroke and spontaneous cerebral hemorrhage in glutaric aciduria type I
William J Zinnanti, Jelena Lazovic, Cathy Housman, David A Antonetti, David M Koeller, James R Connor, Lawrence Steinman
Phenotypic profile of alternative activation marker CD163 is different in Alzheimer’s and Parkinson’s disease
Peixuan Pey, Ronald KB Pearce, Michail E Kalaitzakis, W Sue T Griffin, Steve M Gentleman
MARK4 and MARK3 associate with early tau phosphorylation in Alzheimer’s disease granulovacuolar degeneration bodies
Harald Lund, Elin Gustafsson, Anne Svensson, Maria Nilsson, Margareta Berg, Dan Sunnemark, Gabriel von Euler
Critical role of TNF-alpha-TNFR1 signaling in intracranial aneurysm formation
Tomohiro Aoki, Miyuki Fukuda, Masaki Nishimura, Kazuhiko Nozaki, Shuh Narumiya
Deregulation of subcellular biometal homeostasis through loss of the metal transporter, Zip7, in a childhood neurodegenerative disorder
Alexandra Grubman, Grace E Lidgerwood, Clare Duncan, Laura Bica, Jiang-Li Tan, Sarah J Parker, Aphrodite Caragounis, Jodi Meyerowitz, Irene Volitakis, Diane Moujalled, Jeffrey R Liddell, James L Hickey, Malcolm Horne, Shoshanah Longmuir, Jari Koistinaho, Paul S Donnelly, Peter J Crouch, Imke Tammen, Anthony R White, Katja M Kanninen
α4-integrins control viral meningoencephalitis through differential recruitment of T helper cell subsets
Veit Rothhammer, Andreas Muschaweckh, Georg Gasteiger, Franziska Petermann, Sylvia Heink, Dirk H Busch, Mathias Heikenwälder, Bernhard Hemmer, Ingo Drexler, Thomas Korn
Distinct origins of dura mater graft-associated Creutzfeldt-Jakob disease: past and future problems
Atsushi Kobayashi, Yuichi Matsuura, Shirou Mohri, Tetsuyuki Kitamoto
Arsenic trioxide inhibits Hedgehog, Notch and stem cell properties in glioblastoma neurospheres
Dacheng Ding, Kah Suan Lim, Charles G Eberhart
Neuromyelitis optica MOG-IgG causes reversible lesions in mouse brain
Samira Saadoun, Patrick Waters, Gregory P Owens, Jeffrey L Bennett, Angela Vincent, Marios C Papadopoulos
Comparative analysis of single and combined APP/APLP knockouts reveals reduced spine density in APP-KO mice that is prevented by APPsα expression
Sascha W Weyer, Marta Zagrebelsky, Ulrike Herrmann, Meike Hick, Lennard Ganss, Julia Gobbert, Morna Gruber, Christine Altmann, Martin Korte, Thomas Deller, Ulrike C Müller
Golgi fragmentation precedes neuromuscular denervation and is associated with endosome abnormalities in SOD1-ALS mouse motor neurons
Vera van Dis, Marijn Kuijpers, Elize D Haasdijk, Eva Teuling, Scott A Oakes, Casper C Hoogenraad, Dick Jaarsma
Tau accumulation in the nucleus accumbens in tangle-predominant dementia
Ito Kawakami, Masato Hasegawa, Tetsuaki Arai, Kenji Ikeda, Kenichi Oshima, Kazuhiro Niizato, Naoya Aoki, Katsuse Omi, Shinji Higashi, Masato Hosokawa, Yoshio Hirayasu, Haruhiko Akiyama
Experimental treatment of NRAS-mutated neurocutaneous melanocytosis with MEK162, a MEK-inhibitor
Heidi VN Küsters-Vandevelde, Annelieke ECAB Willemsen, Patricia JTA Groenen, Benno Küsters, Martin Lammens, Pieter Wesseling, Melika Djafarihamedani, Jos Rijntjes, Hans Delye, Michel A Willemsen, Carla ML van Herpen, Willeke AM Blokx
In vivo imaging reveals sigmoidal growth kinetic of β-amyloid plaques
Steffen Burgold, Severin Filser, Mario M Dorostkar, Boris Schmidt, Jochen Herms
Novel monoclonal antibodies to normal and pathologically altered human TDP-43 proteins
Linda K Kwong, David J Irwin, Adam K Walker, Yan Xu, Dawn M Riddle, John Q Trojanowski, Virginia M Y Lee
NOTCH ligands JAG1 and JAG2 as critical pro-survival factors in childhood medulloblastoma
Giulio Fiaschetti, Christina Schroeder, Deborah Castelletti, Alexandre Arcaro, Frank Westermann, Martin Baumgartner, Tarek Shalaby, Michael A Grotzer
Layer-specific gene expression in epileptogenic type II focal cortical dysplasia: normal-looking neurons reveal the presence of a hidden laminar organization
Laura Rossini, Valentina Medici, Laura Tassi, Francesco Cardinale, Giovanni Tringali, Manuela Bramerio, Flavio Villani, Roberto Spreafico, Rita Garbelli
Optimization of electron microscopy for human brains with long-term fixation and fixed-frozen sections
Xiao-Bo Liu, Cynthia M Schumann
Oligomer-targeting with a conformational antibody fragment promotes toxicity in Aβ-expressing flies
Jessica Wacker, Raik Rönicke, Martin Westermann, Melanie Wulff, Klaus G Reymann, Christopher M Dobson, Uwe Horn, Damian C Crowther, Leila M Luheshi, Marcus Fändrich
Diagnostic effectiveness of quantitative [18F]flutemetamol PET imaging for detection of fibrillar amyloid β using cortical biopsy histopathology as the standard of truth in subjects with idiopathic normal pressure hydrocephalus
Ville Leinonen, Juha O Rinne, Dean F Wong, David A Wolk, John Q Trojanowski, Paul F Sherwin, Adrian Smith, Kerstin Heurling, Mandy Su, Igor D Grachev
Muscle histopathology in nebulin-related nemaline myopathy: ultrastrastructural findings correlated to disease severity and genotype
Edoardo Malfatti, Vilma-Lotta Lehtokari, Johann Böhm, Josine M De Winter, Ursula Schäffer, Brigitte Estournet, Susana Quijano-Roy, Soledad Monges, Fabiana Lubieniecki, Remi Bellance, Mai Thao Viou, Angéline Madelaine, Bin Wu, Ana Lía Taratuto, Bruno Eymard, Katarina Pelin, Michel Fardeau, Coen AC Ottenheijm, Carina Wallgren-Pettersson, Jocelyn Laporte, Norma B Romero
Neuromyelitis optica pathology in rats following intraperitoneal injection of NMO-IgG and intracerebral needle injury
Nithi Asavapanumas, AS Verkman
Interaction of transactive response DNA binding protein 43 with nuclear factor κB in mild cognitive impairment with episodic memory deficits
Yasuyuki Ohta, Cyntia Tremblay, Julie A Schneider, David A Bennett, Frederic Calon, Jean-Pierre Julien
The instability of the BTB-KELCH protein Gigaxonin causes Giant Axonal Neuropathy and constitutes a new penetrant and specific diagnostic test
Alexia Boizot, Yasmina Talmat-Amar, Deborah Morrogh, Nancy L Kuntz, Cecile Halbert, Brigitte Chabrol, Henry Houlden, Tanya Stojkovic, Brenda A Schulman, Bernd Rautenstrauss, Pascale Bomont
Early white matter changes in CADASIL: evidence of segmental intramyelinic oedema in a pre-clinical mouse model
Emmanuel Cognat, Sabine Cleophax, Valérie Domenga-Denier, Anne Joutel
Phosphorylation of serine 349 of p62 in Alzheimer’s disease brain
Kunikazu Tanji, Yasuo Miki, Taku Ozaki, Atsushi Maruyama, Hidemi Yoshida, Junsei Mimura, Tomoh Matsumiya, Fumiaki Mori, Tadaatsu Imaizumi, Ken Itoh, Akiyoshi Kakita, Hitoshi Takahashi, Koichi Wakabayashi
Ischemia induces different levels of hypoxia inducible factor-1α protein expression in interneurons and pyramidal neurons
Prabhu Ramamoorthy, Honglian Shi
Non-invasive assessment of retinal alterations in mouse models of infantile and juvenile neuronal ceroid lipofuscinosis by spectral domain optical coherence tomography
Janos Groh, David Stadler, Mathias Buttmann, Rudolf Martini
Early and widespread injury of astrocytes in the absence of demyelination in acute haemorrhagic leukoencephalitis
Christopher A Robinson, Reginald C Adiele, Mylyne Tham, Claudia F Lucchinetti, Bogdan FGh Popescu
Conformational specificity of the C4F6 SOD1 antibody; low frequency of reactivity in sporadic ALS cases
Jacob I Ayers, Guilian Xu, Olga Pletnikova, Juan C Troncoso, P John Hart, David R Borchelt
Complement activation in multiple sclerosis plaques: an immunohistochemical analysis
Gillian Ingram, Sam Loveless, Owain W Howell, Svetlana Hakobyan, Bethan Dancey, Claire L Harris, Neil P Robertson, James W Neal, B Paul Morgan
Blocking the apoE/Aβ interaction ameliorates Aβ-related pathology in APOE ε2 and ε4 targeted replacement Alzheimer model mice
Joanna E Pankiewicz, Maitea Guridi, Jungsu Kim, Ayodeji A Asuni, Sandrine Sanchez, Patrick M Sullivan, David M Holtzman, Martin J Sadowski
Reducing hippocampal extracellular matrix reverses early memory deficits in a mouse model of Alzheimer’s disease
Marlene J Végh, Céline M Heldring, Willem Kamphuis, Sara Hijazi, Arie J Timmerman, Ka Wan Li, Pim van Nierop, Huibert D Mansvelder, Elly M Hol, August B Smit, Ronald E van Kesteren
A neuronal function of the tumor suppressor protein merlin
Alexander Schulz, Ansgar Zoch, Helen Morrison
Zebrafish regenerate full thickness optic nerve myelin after demyelination, but this fails with increasing age
Eva Jolanda Münzel, Catherina G Becker, Thomas Becker, Anna Williams
Possible involvement of lysosomal dysfunction in pathological changes of the brain in aged progranulin-deficient mice
Yoshinori Tanaka, James K Chambers, Takashi Matsuwaki, Keitaro Yamanouchi, Masugi Nishihara
Altered distribution of the EphA4 kinase in hippocampal brain tissue of patients with Alzheimer’s disease correlates with pathology
Andrea FN Rosenberger, Annemieke JM Rozemuller, Wiesje M van der Flier, Philip Scheltens, Saskia M van der Vies, Jeroen JM Hoozemans
Neurotrophin receptors expression in mesial temporal lobe epilepsy with and without psychiatric comorbidities and their relation with seizure type and surgical outcome
Ludmyla Kandratavicius, Jaime Eduardo Hallak, Carlos Gilberto Carlotti, Joao Alberto Assirati, Joao Pereira Leite
Mutation and expression analysis in medulloblastoma yields prognostic variants and a putative mechanism of disease for i17q tumors
Gabriel A Bien-Willner, Robi D Mitra
Polymicrogyria: pathology, fetal origins and mechanisms
Waney Squier, Anna Jansen
Angiogenesis in multiple sclerosis and experimental autoimmune encephalomyelitis
Francesco Girolamo, Cristiana Coppola, Domenico Ribatti, Maria Trojano
Genetic drivers of metastatic dissemination in sonic hedgehog medulloblastoma
Noah C Jenkins, Ricky R Kalra, Adrian Dubuc, Walavan Sivakumar, Carolyn A Pedone, Xiaochong Wu, Michael D Taylor, Daniel W Fults
Systemic delivery of P42 peptide: a new weapon to fight Huntington’s disease
Yoan Arribat, Yasmina Talmat-Amar, Alexia Paucard, Pierre Lesport, Nathalie Bonneaud, Caroline Bauer, Nicole Bec, Marie-Laure Parmentier, Lorraine Benigno, Christian Larroque, Patrick Maurel, Florence Maschat
Fetal syringomyelia
Anne Guo, David Chitayat, Susan Blaser, Sarah Keating, Patrick Shannon
Pathological alpha-synuclein propagates through neural networks
Masami Masuda-Suzukake, Takashi Nonaka, Masato Hosokawa, Maki Kubo, Aki Shimozawa, Haruhiko Akiyama, Masato Hasegawa
Microglial phenotypes and toll-like receptor 2 in the substantia nigra and hippocampus of incidental Lewy body disease cases and Parkinson’s disease patients
Karlijn J Doorn, Tim Moors, Benjamin Drukarch, Wilma DJ van de Berg, Paul J Lucassen, Anne-Marie van Dam
Sequestration of latent TGF-β binding protein 1 into CADASIL-related Notch3-ECD deposits
Jessica Kast, Patrizia Hanecker, Nathalie Beaufort, Armin Giese, Anne Joutel, Martin Dichgans, Christian Opherk, Christof Haffner
Soluble amyloid beta levels are elevated in the white matter of Alzheimer’s patients, independent of cortical plaque severity
Lyndsey E Collins-Praino, Yitshak I Francis, Erica Y Griffith, Anne F Wiegman, Jonathan Urbach, Arlene Lawton, Lawrence S Honig, Etty Cortes, Jean Paul G Vonsattel, Peter D Canoll, James E Goldman, Adam M Brickman
Discrepancy in CCL2 and CCR2 expression in white versus grey matter hippocampal lesions of Multiple Sclerosis patients
Marloes Prins, Ranjan Dutta, Bart Baselmans, John J P Brevé, John G J M Bol, Sadie A Deckard, Paul van der Valk, Sandra Amor, Bruce D Trapp, Helga E de Vries, Benjamin Drukarch, Anne-Marie van Dam
Axonal degeneration in multiple sclerosis: can we predict and prevent permanent disability?
Jae Young Lee, Kasra Taghian, Steven Petratos
Protease-resistant SOD1 aggregates in amyotrophic lateral sclerosis demonstrated by paraffin-embedded tissue (PET) blot
Petra Steinacker, Christian Berner, Dietmar R Thal, Johannes Attems, Albert C Ludolph, Markus Otto
Amyloid β and Tau Alzheimer’s disease related pathology is reduced by Toll-like receptor 9 stimulation
Henrieta Scholtzova, Peter Chianchiano, Jason Pan, Yanjie Sun, Fernando Goñi, Pankaj D Mehta, Thomas Wisniewski
Hirano body expression impairs spatial working memory in a novel mouse model
Matthew Furgerson, Jason K. Clark, Jonathon D. Crystal, John J. Wagner, Marcus Fechheimer, Ruth Furukawa
Integrated multi-cohort transcriptional meta-analysis of neurodegenerative diseases
Matthew D Li, Terry C Burns, Alexander A Morgan, Purvesh Khatri
Behavioral deficits, early gliosis, dysmyelination and synaptic dysfunction in a mouse model of mucolipidosis IV
Yulia Grishchuk, Sarmi Sri, Nikita Rudinskiy, Weiyuan Ma, Katherine G. Stember, Matthew W. Cottle, Ellen Sapp, Marian Difiglia, Alona Muzikansky, Rebecca A. Betensky, Andrew M. S. Wong, Brian J. Bacskai, Bradley T. Hyman, Raymond J. Kelleher, Jonathan D. Cooper, Susan A. Slaugenhaupt
MotorPlex provides accurate variant detection across large muscle genes both in single myopathic patients and in pools of DNA samples
Marco Savarese, Giuseppina Di Fruscio, Margherita Mutarelli, Annalaura Torella, Francesca Magri, Filippo Maria Santorelli, Giacomo Pietro Comi, Claudio Bruno, Vincenzo Nigro
Relocation of p25α/tubulin polymerization promoting protein from the nucleus to the perinuclear cytoplasm in the oligodendroglia of sporadic and COQ2 mutant multiple system atrophy
Kiyobumi Ota, Masato Obayashi, Kokoro Ozaki, Shizuko Ichinose, Akiyoshi Kakita, Mari Tada, Hitoshi Takahashi, Noboru Ando, Yoshinobu Eishi, Hidehiro Mizusawa, Kinya Ishikawa
New α- and γ-synuclein immunopathological lesions in human brain
Irina Surgucheva, Kathy L. Newell, Jeffrey Burns, Andrei Surguchov
Axial mitochondrial myopathy in a patient with rapidly progressive adult-onset scoliosis
Annie Hiniker, Lee-Jun Wong, Sigurd Berven, Cavatina K Truong, Adekunle M Adesina, Marta Margeta
Categorization of multiple sclerosis relapse subtypes by B cell profiling in the blood
Christopher Hohmann, Bianca Milles, Michael Schinke, Michael Schroeter, Jochen Ulzheimer, Peter Kraft, Christoph Kleinschnitz, Paul V Lehmann, Stefanie Kuerten
Inconsistencies and Controversies Surrounding the Amyloid Hypothesis of Alzheimer's Disease
Gary P Morris, Ian A Clark, Bryce Vissel
Dystonia type 6 gene product Thap1: identification of a 50 kDa DNA-binding species in neuronal nuclear fractions
Maitane Ortiz-Virumbrales, Marta Ruiz, Eugene Hone, Georgia Dolios, Rong Wang, Andrika Morant, Jessica Kottwitz, Laurie J Ozelius, Sam Gandy, Michelle E Ehrlich
Stereological study of the neuronal number and volume of 38 brain subdivisions of subjects diagnosed with autism reveals significant alterations restricted to the striatum, amygdala and cerebellum
Jerzy Wegiel, Michael Flory, Izabela Kuchna, Krzysztof Nowicki, Shuang Yong Ma, Humi Imaki, Jarek Wegiel, Ira L Cohen, Eric London, Thomas Wisniewski, William Ted Brown
β-amyloid induces a dying-back process and remote trans-synaptic alterations in a microfluidic-based reconstructed neuronal network
Bérangére Deleglise, Sebastien Magnifico, Eric Duplus, Pauline Vaur, Vanessa Soubeyre, Morgane Belle, Maeva Vignes, Jean-Louis Viovy, Etienne Jacotot, Jean-Michel Peyrin, Bernard Brugg
Microglial pathology
Wolfgang J Streit, Qing-Shan Xue, Jasmin Tischer, Ingo Bechmann
Abundant kif21b is associated with accelerated progression in neurodegenerative diseases
Karim L Kreft, Marjan van Meurs, Annet F Wierenga-Wolf, Marie-Jose Melief, Miriam E van Strien, Elly M Hol, Ben A Oostra, Jon D Laman, Rogier Q Hintzen
Traumatic brain injury causes selective, CD74-dependent peripheral lymphocyte activation that exacerbates neurodegeneration
Richard P Tobin, Sanjib Mukherjee, Jessica M Kain, Susannah K Rogers, Stephanie K Henderson, Heather L Motal, M Karen Newell Rogers, Lee A Shapiro
Pax3 expression enhances PDGF-B-induced brainstem gliomagenesis and characterizes a subset of brainstem glioma
Katherine L. Misuraca, Kelly L. Barton, Alexander Chung, Alexander K. Diaz, Simon J. Conway, David L. Corcoran, Suzanne J. Baker, Oren J. Becher
Frequent and symmetric deposition of misfolded tau oligomers within presynaptic and postsynaptic terminals in Alzheimer’s disease
Hwan-Ching Tai, Bo Y Wang, Alberto Serrano-Pozo, Matthew P Frosch, Tara L Spires-Jones, Bradley T Hyman
p38 MAP kinase-mediated NMDA receptor-dependent suppression of hippocampal hypersynchronicity in a mouse model of Alzheimer’s disease
Arne A Ittner, Amadeus Gladbach, Josefine Bertz, Lisa S Suh, Lars M Ittner
Oncogenic codon 13 NRAS mutation in a primary mesenchymal brain neoplasm and nevus of a child with neurocutaneous melanosis
Francis Shih, Stephen Yip, Patrick J McDonald, Albert E Chudley, Marc R Del Bigio
The prion protein protease sensitivity, stability and seeding activity in variably protease sensitive prionopathy brain tissue suggests molecular overlaps with sporadic Creutzfeldt-Jakob disease
Alexander H Peden, Deep P Sarode, Carl R Mulholland, Marcelo A Barria, Diane L Ritchie, James W Ironside, Mark W Head
Myofiber HLA-DR expression is a distinctive biomarker for antisynthetase-associated myopathy
Jessie Aouizerate, Marie De Antonio, Guillaume Bassez, Romain K Gherardi, Francis Berenbaum, Loïc Guillevin, Alice Berezne, Dominique Valeyre, Thierry Maisonobe, Odile Dubourg, Anne Cosnes, Olivier Benveniste, François Jérôme Authier
Decontamination of medical devices from pathological amyloid-β-, tau- and α-synuclein aggregates
Achim Thomzig, Katja Wagenführ, Martin L Daus, Marion Joncic, Walter J Schulz-Schaeffer, Marc Thanheiser, Martin Mielke, Michael Beekes
Altered lipid levels provide evidence for myelin dysfunction in multiple system atrophy
Anthony S Don, Jen-Hsiang T Hsiao, Jonathan M Bleasel, Timothy A Couttas, Glenda M Halliday, Woojin Scott Kim
Accumulation of 2-hydroxyglutarate in gliomas correlates with survival: a study by 3.0-tesla magnetic resonance spectroscopy
Manabu Natsumeda, Hironaka Igarashi, Toshiharu Nomura, Ryosuke Ogura, Yoshihiro Tsukamoto, Tsutomu Kobayashi, Hiroshi Aoki, Kouichirou Okamoto, Akiyoshi Kakita, Hitoshi Takahashi, Tsutomu Nakada, Yukihiko Fujii
Hippocampal changes produced by overexpression of the human CHRNA5/A3/B4 gene cluster may underlie cognitive deficits rescued by nicotine in transgenic mice
Susanna Molas, Thomas Gener, Jofre Güell, Mairena Martín, Inmaculada Ballesteros-Yáñez, Maria V Sanchez-Vives, Mara Dierssen
The role of Galectin-3 in α-synuclein-induced microglial activation
Antonio Boza-Serrano, Juan F Reyes, Nolwen L Rey, Hakon Leffler, Luc Bousset, Ulf Nilsson, Patrik Brundin, Jose Luis Venero, Miguel Angel Burguillos, Tomas Deierborg
Decreased EAAT2 protein expression in the essential tremor cerebellar cortex
Michelle Lee, Melody M Cheng, Chi-Ying Lin, Elan D Louis, Phyllis L Faust, Sheng-Han Kuo
Neuropathological features of genetically confirmed DYT1 dystonia: investigating disease-specific inclusions
Reema Paudel, Aoife Kiely, Abi Li, Tammaryn Lashley, Rina Bandopadhyay, John Hardy, Hyder A Jinnah, Kailash Bhatia, Henry Houlden, Janice L Holton
The problem of axonal injury in the brains of veterans with histories of blast exposure
Jiwon Ryu, Iren Horkayne-Szakaly, Leyan Xu, Olga Pletnikova, Francesco Leri, Charles Eberhart, Juan C Troncoso, Vassilis E Koliatsos
FMRpolyG-positive inclusions in CNS and non-CNS organs of a fragile X premutation carrier with fragile X-associated tremor/ataxia syndrome
Ronald AM Buijsen, Chantal Sellier, Lies-Anne WFM Severijnen, Mustapha Oulad-Abdelghani, Rob FM Verhagen, Robert F Berman, Nicolas Charlet-Berguerand, Rob Willemsen, Renate K Hukema
Treg cells mediate recovery from EAE by controlling effector T cell proliferation and motility in the CNS
Michail Koutrolos, Kerstin Berer, Naoto Kawakami, Hartmut Wekerle, Gurumoorthy Krishnamoorthy
Accumulation of α-synuclein in dementia with Lewy bodies is associated with decline in the α-synuclein-degrading enzymes kallikrein-6 and calpain-1
J Scott Miners, Ruth Renfrew, Marta Swirski, Seth Love
Germline mutations in RYR1 are associated with foetal akinesia deformation sequence/lethal multiple pterygium syndrome
Arthur B McKie, Atif Alsaedi, Julie Vogt, Kyra E Stuurman, Marjan M Weiss, Hassan Shakeel, Louise Tee, Neil V Morgan, Peter G J Nikkels, Gijs van Haaften, Soo-Mi Park, Jasper J van der Smagt, Marianna Bugiani, Eamonn R Maher
Immunoreactivity of valosin-containing protein in sporadic amyotrophic lateral sclerosis and in a case of its novel mutant
Takashi Ayaki, Hidefumi Ito, Hiroko Fukushima, Takeshi Inoue, Takayuki Kondo, Akito Ikemoto, Takeshi Asano, Akemi Shodai, Takuji Fujita, Satoshi Fukui, Hiroyuki Morino, Satoshi Nakano, Hirofumi Kusaka, Hirofumi Yamashita, Masafumi Ihara, Riki Matsumoto, Jun Kawamata, Makoto Urushitani, Hideshi Kawakami, Ryosuke Takahashi
Astroglial PGC-1alpha increases mitochondrial antioxidant capacity and suppresses inflammation: implications for multiple sclerosis
Philip G Nijland, Maarten E Witte, Bert van het Hof, Susanne van der Pol, Jan Bauer, Hans Lassmann, Paul van der Valk, Helga E de Vries, Jack van Horssen
Transcript profiling of different types of multiple sclerosis lesions yields FGF1 as a promoter of remyelination
Hema Mohan, Anita Friese, Stefanie Albrecht, Markus Krumbholz, Christina L Elliott, Ariel Arthur, Ramesh Menon, Cinthia Farina, Andreas Junker, Christine Stadelmann, Susan C Barnett, Inge Huitinga, Hartmut Wekerle, Reinhard Hohlfeld, Hans Lassmann, Tanja Kuhlmann, Chris Linington, Edgar Meinl
Ultrastructural differences in pretangles between Alzheimer disease and corticobasal degeneration revealed by comparative light and electron microscopy
Shinsui Tatsumi, Toshiki Uchihara, Ikuko Aiba, Yasushi Iwasaki, Maya Mimuro, Ryosuke Takahashi, Mari Yoshida
RETRACTED ARTICLE: 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 WFM Severijnen, Dieter Edbauer, Alex Maas, Nicolas Charlet-Berguerand, Rob Willemsen, John C van Swieten
Models of α-synuclein aggregation in Parkinson’s disease
Rosa María Giráldez-Pérez, Mónica Antolín-Vallespín, María Dolores Muñoz, Amelia Sánchez-Capelo
Mitochondrial abnormalities and low grade inflammation are present in the skeletal muscle of a minority of patients with amyotrophic lateral sclerosis; an observational myopathology study
Safa Al-Sarraj, Andrew King, Matt Cleveland, Pierre-François Pradat, Andrea Corse, Jeffrey D Rothstein, Peter Nigel Leigh, Bams Abila, Stewart Bates, Jens Wurthner, Vincent Meininger
Analysis of microRNA from archived formalin-fixed paraffin-embedded specimens of amyotrophic lateral sclerosis
Koichi Wakabayashi, Fumiaki Mori, Akiyoshi Kakita, Hitoshi Takahashi, Jun Utsumi, Hidenao Sasaki
Spontaneous white matter damage, cognitive decline and neuroinflammation in middle-aged hypertensive rats: an animal model of early-stage cerebral small vessel disease
Daniel Kaiser, Gesa Weise, Karoline Möller, Johanna Scheibe, Claudia Pösel, Sebastian Baasch, Matthias Gawlitza, Donald Lobsien, Kai Diederich, Jens Minnerup, Alexander Kranz, Johannes Boltze, Daniel-Christoph Wagner
Glial cells in familial amyloidotic polyneuropathy
Nádia Pereira Gonçalves, Susete Costelha, Maria João Saraiva
Silencing BMI1 eliminates tumor formation of pediatric glioma CD133+ cells not by affecting known targets but by down-regulating a novel set of core genes
Patricia A Baxter, Qi Lin, Hua Mao, Mari Kogiso, Xiumei Zhao, Zhigang Liu, Yulun Huang, Horatiu Voicu, Sivashankarappa Gurusiddappa, Jack M Su, Adekunle M Adesina, Laszlo Perlaky, Robert C Dauser, Hon-chiu Eastwood Leung, Karin M Muraszko, Jason A Heth, Xing Fan, Ching C Lau, Tsz-Kwong Man, Murali Chintagumpala, Xiao-Nan Li
Glucose transporter 3 is a rab11-dependent trafficking cargo and its transport to the cell surface is reduced in neurons of CAG140 Huntington’s disease mice
Hollis McClory, Dana Williams, Ellen Sapp, Leah W Gatune, Ping Wang, Marian DiFiglia, Xueyi Li
TDP-43 pathology in the basal forebrain and hypothalamus of patients with amyotrophic lateral sclerosis
Matthew D Cykowski, Hidehiro Takei, Paul E Schulz, Stanley H Appel, Suzanne Z Powell
Sporadic hemangioblastomas are characterized by cryptic VHL inactivation
Ganesh M Shankar, Amaro Taylor-Weiner, Nina Lelic, Robert T Jones, James C Kim, Joshua M Francis, Malak Abedalthagafi, Lawrence F Borges, Jean-Valery Coumans, William T Curry, Brian V Nahed, John H Shin, Sun Ha Paek, Sung-Hye Park, Chip Stewart, Michael S Lawrence, Kristian Cibulskis, Aaron R Thorner, Paul Van Hummelen, Anat O Stemmer-Rachamimov, Tracy T Batchelor, Scott L Carter, Mai P Hoang, Sandro Santagata, David N Louis, Fred G Barker, Matthew Meyerson, Gad Getz, Priscilla K Brastianos, Daniel P Cahill
WNT activation by lithium abrogates TP53 mutation associated radiation resistance in medulloblastoma
Nataliya Zhukova, Vijay Ramaswamy, Marc Remke, Dianna C Martin, Pedro Castelo-Branco, Cindy H Zhang, Michael Fraser, Ken Tse, Raymond Poon, David JH Shih, Berivan Baskin, Peter N Ray, Eric Bouffet, Peter Dirks, Andre O von Bueren, Elke Pfaff, Andrey Korshunov, David TW Jones, Paul A Northcott, Marcel Kool, Trevor J Pugh, Scott L Pomeroy, Yoon-Jae Cho, Torsten Pietsch, Marco Gessi, Stefan Rutkowski, Laszlo Bognár, Byung-Kyu Cho, Charles G Eberhart, Cecile Faure Conter, Maryam Fouladi, Pim J French, Wieslawa A Grajkowska, Nalin Gupta, Peter Hauser, Nada Jabado, Alexandre Vasiljevic, Shin Jung, Seung-Ki Kim, Almos Klekner, Toshihiro Kumabe, Boleslaw Lach, Jeffrey R Leonard, Linda M Liau, Luca Massimi, Ian F Pollack, Young Shin Ra, Joshua B Rubin, Erwin G Van Meir, Kyu-Chang Wang, William A Weiss, Karel Zitterbart, Robert G Bristow, Benjamin Alman, Cynthia E Hawkins, David Malkin, Steven C Clifford, Stefan M Pfister, Michael D Taylor, Uri Tabori
Longitudinal testing of hippocampal plasticity reveals the onset and maintenance of endogenous human Aß-induced synaptic dysfunction in individual freely behaving pre-plaque transgenic rats: rapid reversal by anti-Aß agents
Yingjie Qi, Igor Klyubin, Sarah C Harney, NengWei Hu, William K Cullen, Marianne K Grant, Julia Steffen, Edward N Wilson, Sonia Do Carmo, Stefan Remy, Martin Fuhrmann, Karen H Ashe, A Claudio Cuello, Michael J Rowan
Increased 5-hydroxymethylcytosine and decreased 5-methylcytosine are indicators of global epigenetic dysregulation in diffuse intrinsic pontine glioma
Sama Ahsan, Eric H Raabe, Michael C Haffner, Ajay Vaghasia, Katherine E Warren, Martha Quezado, Leomar Y Ballester, Javad Nazarian, Charles G Eberhart, Fausto J Rodriguez
The formation of tau pore-like structures is prevalent and cell specific: possible implications for the disease phenotypes
Cristian A Lasagna-Reeves, Urmi Sengupta, Diana Castillo-Carranza, Julia E Gerson, Marcos Guerrero-Munoz, Juan C Troncoso, George R Jackson, Rakez Kayed
A new sensitive PCR assay for one-step detection of 12 IDH1/2 mutations in glioma
Aurélie Catteau, Hélène Girardi, Florence Monville, Cécile Poggionovo, Sabrina Carpentier, Véronique Frayssinet, Jesse Voss, Robert Jenkins, Blandine Boisselier, Karima Mokhtari, Marc Sanson, Hélène Peyro-Saint-Paul, Caterina Giannini
Overcoming multiple drug resistance mechanisms in medulloblastoma
Ramadhan T Othman, Ioanna Kimishi, Tracey D Bradshaw, Lisa CD Storer, Andrey Korshunov, Stefan M Pfister, Richard G Grundy, Ian D Kerr, Beth Coyle
Intracellular Aβ pathology and early cognitive impairments in a transgenic rat overexpressing human amyloid precursor protein: a multidimensional study
M Florencia Iulita, Simon Allard, Luise Richter, Lisa-Marie Munter, Adriana Ducatenzeiler, Christoph Weise, Sonia Do Carmo, William L Klein, Gerhard Multhaup, A Claudio Cuello
Adenosine triphosphate drives head and neck cancer pain through P2X2/3 heterotrimers
Yi Ye, Kentaro Ono, Daniel G Bernabé, Chi T Viet, Victoria Pickering, John C Dolan, Markus Hardt, Anthony P Ford, Brian L Schmidt
Obesity and diabetes cause cognitive dysfunction in the absence of accelerated β-amyloid deposition in a novel murine model of mixed or vascular dementia
Dana M Niedowicz, Valerie L Reeves, Thomas L Platt, Katharina Kohler, Tina L Beckett, David K Powell, Tiffany L Lee, Travis R Sexton, Eun Suk Song, Lawrence D Brewer, Caitlin S Latimer, Susan D Kraner, Kara L Larson, Sabire Ozcan, Christopher M Norris, Louis B Hersh, Nada M Porter, Donna M Wilcock, Michael Paul Murphy
Characterization of tau oligomeric seeds in progressive supranuclear palsy
Julia E Gerson, Urmi Sengupta, Cristian A Lasagna-Reeves, Marcos J Guerrero-Muñoz, Juan Troncoso, Rakez Kayed
High-throughput, automated quantification of white matter neurons in mild malformation of cortical development in epilepsy
Joan YW Liu, Matthew Ellis, Hannah Brooke-Ball, Jane de Tisi, Sofia H Eriksson, Sebastian Brandner, Sanjay M Sisodiya, Maria Thom
Temporal dystrophic remodeling within the intrinsic cardiac nervous system of the streptozotocin-induced diabetic rat model
Chantalle E Menard, Melanie Durston, Elena Zherebitskaya, Darrell R Smith, Darren Freed, Gordon W Glazner, Ganghong Tian, Paul Fernyhough, Rakesh C Arora
Platelets, a reliable source for peripheral Alzheimer’s disease biomarkers?
Michael Veitinger, Balazs Varga, Sheila B Guterres, Maria Zellner
Brain distribution of dipeptide repeat proteins in frontotemporal lobar degeneration and motor neurone disease associated with expansions in C9ORF72
Yvonne S Davidson, Holly Barker, Andrew C Robinson, Jennifer C Thompson, Jenny Harris, Claire Troakes, Bradley Smith, Safa Al-Saraj, Chris Shaw, Sara Rollinson, Masami Masuda-Suzukake, Masato Hasegawa, Stuart Pickering-Brown, Julie S Snowden, David M Mann
Prognostic and predictive markers in recurrent high grade glioma; results from the BR12 randomised trial
Vincent Peter Collins, Koichi Ichimura, Ying Di, Danita Pearson, Ray Chan, Lindsay C Thompson, Rhian Gabe, Michael Brada, Sally P Stenning
Selective vulnerability of the cerebral vasculature to blast injury in a rat model of mild traumatic brain injury
Miguel A Gama Sosa, Rita De Gasperi, Pierce L Janssen, Frank J Yuk, Pamela C Anazodo, Paul E Pricop, Alejandro J Paulino, Bridget Wicinski, Michael C Shaughness, Eric Maudlin-Jeronimo, Aaron A Hall, Dara L Dickstein, Richard M McCarron, Mikulas Chavko, Patrick R Hof, Stephen T Ahlers, Gregory A Elder
CNS-targeted glucocorticoid reduces pathology in mouse model of amyotrophic lateral sclerosis
Matthew C Evans, Pieter J Gaillard, Marco de Boer, Chantal Appeldoorn, Rick Dorland, Nicola R Sibson, Martin R Turner, Daniel C Anthony, Helen B Stolp
Tau pathology does not affect experience-driven single-neuron and network-wide Arc/Arg3.1 responses
Nikita Rudinskiy, Jonathan M Hawkes, Susanne Wegmann, Kishore V Kuchibhotla, Alona Muzikansky, Rebecca A Betensky, Tara L Spires-Jones, Bradley T Hyman
Evidence for mTOR pathway activation in a spectrum of epilepsy-associated pathologies
Joan Liu, Cheryl Reeves, Zuzanna Michalak, Antonietta Coppola, Beate Diehl, Sanjay M Sisodiya, Maria Thom
Mutations in tubulin genes are frequent causes of various foetal malformations of cortical development including microlissencephaly
Catherine Fallet-Bianco, Annie Laquerrière, Karine Poirier, Ferechte Razavi, Fabien Guimiot, Patricia Dias, Laurence Loeuillet, Karine Lascelles, Cherif Beldjord, Nathalie Carion, Aurélie Toussaint, Nicole Revencu, Marie-Claude Addor, Benoit Lhermitte, Marie Gonzales, Jelena Martinovich, Bettina Bessieres, Maryse Marcy-Bonnière, Frédérique Jossic, Pascale Marcorelles, Philippe Loget, Jamel Chelly, Nadia Bahi-Buisson
The identification of mitochondrial DNA variants in glioblastoma multiforme
Ka Yu Yeung, Adam Dickinson, Jacqueline F Donoghue, Galina Polekhina, Stefan J White, Dimitris K Grammatopoulos, Matthew McKenzie, Terrance G Johns, Justin C St John
Caspase-mediated cleavage of actin and tubulin is a common feature and sensitive marker of axonal degeneration in neural development and injury
Jennifer D Sokolowski, Kanchana K Gamage, Daniel S Heffron, Andrea C LeBlanc, Christopher D Deppmann, James W Mandell
Alpha-synuclein spreading in M83 mice brain revealed by detection of pathological α-synuclein by enhanced ELISA
Dominique Bétemps, Jérémy Verchère, Sébastien Brot, Eric Morignat, Luc Bousset, Damien Gaillard, Latifa Lakhdar, Ronald Melki, Thierry Baron
Concomitant progressive supranuclear palsy and chronic traumatic encephalopathy in a boxer
Helen Ling, Eleanna Kara, Tamas Revesz, Andrew J Lees, Gordon T Plant, Davide Martino, Henry Houlden, John Hardy, Janice L Holton
Neuronal injury biomarkers and prognosis in ADNI subjects with normal cognition
Jon B Toledo, Michael W Weiner, David A Wolk, Xiao Da, Kewei Chen, Steven E Arnold, William Jagust, Clifford Jack, Eric M Reiman, Christos Davatzikos, Leslie M Shaw, John Q Trojanowski
Brain-region–specific alterations of the trajectories of neuronal volume growth throughout the lifespan in autism
Jerzy Wegiel, Michael Flory, Izabela Kuchna, Krzysztof Nowicki, Shuang Yong Ma, Humi Imaki, Jarek Wegiel, Ira L Cohen, Eric London, W Ted Brown, Thomas Wisniewski