Ausgabe 1/2018
Inhalt (68 Artikel)
α-Synuclein accumulation and GBA deficiency due to L444P GBA mutation contributes to MPTP-induced parkinsonism
Seung Pil Yun, Donghoon Kim, Sangjune Kim, SangMin Kim, Senthilkumar S. Karuppagounder, Seung-Hwan Kwon, Saebom Lee, Tae-In Kam, Suhyun Lee, Sangwoo Ham, Jae Hong Park, Valina L. Dawson, Ted M. Dawson, Yunjong Lee, Han Seok Ko
Molecular and functional signatures in a novel Alzheimer’s disease mouse model assessed by quantitative proteomics
Dong Kyu Kim, Joonho Park, Dohyun Han, Jinhee Yang, Ahbin Kim, Jongmin Woo, Youngsoo Kim, Inhee Mook-Jung
Parkinson disease-associated mutations in LRRK2 cause centrosomal defects via Rab8a phosphorylation
Jesús Madero-Pérez, Elena Fdez, Belén Fernández, Antonio J. Lara Ordóñez, Marian Blanca Ramírez, Patricia Gómez-Suaga, Dieter Waschbüsch, Evy Lobbestael, Veerle Baekelandt, Angus C. Nairn, Javier Ruiz-Martínez, Ana Aiastui, Adolfo López de Munain, Pawel Lis, Thomas Comptdaer, Jean-Marc Taymans, Marie-Christine Chartier-Harlin, Alexandria Beilina, Adriano Gonnelli, Mark R. Cookson, Elisa Greggio, Sabine Hilfiker
Piperine ameliorates SCA17 neuropathology by reducing ER stress
Jifeng Guo, Yiting Cui, Qiong Liu, Yang Yang, Yujing Li, Ling Weng, Beisha Tang, Peng Jin, Xiao-Jiang Li, Su Yang, Shihua Li
Mfn2 ablation causes an oxidative stress response and eventual neuronal death in the hippocampus and cortex
Sirui Jiang, Priya Nandy, Wenzhang Wang, Xiaopin Ma, Jeffrey Hsia, Chunyu Wang, Zhenlian Wang, Mengyue Niu, Sandra L. Siedlak, Sandy Torres, Hisashi Fujioka, Ying Xu, Hyoung-gon Lee, George Perry, Jun Liu, Xiongwei Zhu
BACE1 elevation engendered by GGA3 deletion increases β-amyloid pathology in association with APP elevation and decreased CHL1 processing in 5XFAD mice
WonHee Kim, Liang Ma, Selene Lomoio, Rachel Willen, Sylvia Lombardo, Jinghui Dong, Philip G. Haydon, Giuseppina Tesco
Pathological phosphorylation of tau and TDP-43 by TTBK1 and TTBK2 drives neurodegeneration
Laura M. Taylor, Pamela J. McMillan, Nicole F. Liachko, Timothy J. Strovas, Bernardino Ghetti, Thomas D. Bird, C. Dirk Keene, Brian C. Kraemer
Dysregulated phosphorylation of Rab GTPases by LRRK2 induces neurodegeneration
Ga Ram Jeong, Eun-Hae Jang, Jae Ryul Bae, Soyoung Jun, Ho Chul Kang, Chi-Hu Park, Joo-Ho Shin, Yukio Yamamoto, Keiko Tanaka-Yamamoto, Valina L. Dawson, Ted M. Dawson, Eun-Mi Hur, Byoung Dae Lee
Extracellular α-synuclein levels are regulated by neuronal activity
Kaoru Yamada, Takeshi Iwatsubo
Genetic ablation of dynactin p150Glued in postnatal neurons causes preferential degeneration of spinal motor neurons in aged mice
Jia Yu, Chen Lai, Hoon Shim, Chengsong Xie, Lixin Sun, Cai-Xia Long, Jinhui Ding, Yan Li, Huaibin Cai
Early long-term administration of the CSF1R inhibitor PLX3397 ablates microglia and reduces accumulation of intraneuronal amyloid, neuritic plaque deposition and pre-fibrillar oligomers in 5XFAD mouse model of Alzheimer’s disease
Justyna Sosna, Stephan Philipp, Ricardo Albay III, Jorge Mauricio Reyes-Ruiz, David Baglietto-Vargas, Frank M. LaFerla, Charles G. Glabe
Targeting Hif1a rescues cone degeneration and prevents subretinal neovascularization in a model of chronic hypoxia
Maya Barben, Christian Schori, Marijana Samardzija, Christian Grimm
Tau oligomers mediate α-synuclein toxicity and can be targeted by immunotherapy
Julia E. Gerson, Kathleen M. Farmer, Natalie Henson, Diana L. Castillo-Carranza, Mariana Carretero Murillo, Urmi Sengupta, Alan Barrett, Rakez Kayed
Cognitive impairment in metabolically-obese, normal-weight rats: identification of early biomarkers in peripheral blood mononuclear cells
Margalida Cifre, Andreu Palou, Paula Oliver
Amyloid-beta modulates microglial responses by binding to the triggering receptor expressed on myeloid cells 2 (TREM2)
Li Zhong, Zongqi Wang, Daxin Wang, Zhe Wang, Yuka A. Martens, Linbei Wu, Ying Xu, Kai Wang, Jianguo Li, Ruizhi Huang, Dan Can, Huaxi Xu, Guojun Bu, Xiao-Fen Chen
Loss of XBP1 accelerates age-related decline in retinal function and neurodegeneration
Todd McLaughlin, Marek Falkowski, Jae Whan Park, Stephen Keegan, Michael Elliott, Joshua J. Wang, Sarah X. Zhang
Apolipoprotein E4 impairs spontaneous blood brain barrier repair following traumatic brain injury
Bevan S. Main, Sonia Villapol, Stephanie S. Sloley, David J. Barton, Maia Parsadanian, Chinyere Agbaegbu, Kathryn Stefos, Mondona S. McCann, Patricia M. Washington, Olga C. Rodriguez, Mark P. Burns
Structural and mechanistic aspects influencing the ADAM10-mediated shedding of the prion protein
Luise Linsenmeier, Behnam Mohammadi, Sebastian Wetzel, Berta Puig, Walker S. Jackson, Alexander Hartmann, Keiji Uchiyama, Suehiro Sakaguchi, Kristina Endres, Jörg Tatzelt, Paul Saftig, Markus Glatzel, Hermann C. Altmeppen
Modulation of GSK-3 provides cellular and functional neuroprotection in the rd10 mouse model of retinitis pigmentosa
Alonso Sánchez-Cruz, Beatriz Villarejo-Zori, Miguel Marchena, Josefa Zaldivar-Díez, Valle Palomo, Carmen Gil, Ignacio Lizasoain, Pedro de la Villa, Ana Martínez, Enrique J. de la Rosa, Catalina Hernández-Sánchez
Vibrational spectroscopy: a promising approach to discriminate neurodegenerative disorders
Maria Paraskevaidi, Pierre L. Martin-Hirsch, Francis L. Martin
Inoculation of α-synuclein preformed fibrils into the mouse gastrointestinal tract induces Lewy body-like aggregates in the brainstem via the vagus nerve
Norihito Uemura, Hisashi Yagi, Maiko T. Uemura, Yusuke Hatanaka, Hodaka Yamakado, Ryosuke Takahashi
AMPA-ergic regulation of amyloid-β levels in an Alzheimer’s disease mouse model
Jane C. Hettinger, Hyo Lee, Guojun Bu, David M. Holtzman, John R. Cirrito
Differential induction of mutant SOD1 misfolding and aggregation by tau and α-synuclein pathology
Michael C. Pace, Guilian Xu, Susan Fromholt, John Howard, Benoit I. Giasson, Jada Lewis, David R. Borchelt
Identification and therapeutic modulation of a pro-inflammatory subset of disease-associated-microglia in Alzheimer’s disease
Srikant Rangaraju, Eric B. Dammer, Syed Ali Raza, Priyadharshini Rathakrishnan, Hailian Xiao, Tianwen Gao, Duc M. Duong, Michael W. Pennington, James J. Lah, Nicholas T. Seyfried, Allan I. Levey
Large-scale transcriptomic analysis reveals that pridopidine reverses aberrant gene expression and activates neuroprotective pathways in the YAC128 HD mouse
Rebecca Kusko, Jennifer Dreymann, Jermaine Ross, Yoonjeong Cha, Renan Escalante-Chong, Marta Garcia-Miralles, Liang Juin Tan, Michael E. Burczynski, Ben Zeskind, Daphna Laifenfeld, Mahmoud Pouladi, Michal Geva, Iris Grossman, Michael R. Hayden
Tolerogenic bone marrow-derived dendritic cells induce neuroprotective regulatory T cells in a model of Parkinson’s disease
Charles R. Schutt, Howard E. Gendelman, R. Lee Mosley
2D versus 3D human induced pluripotent stem cell-derived cultures for neurodegenerative disease modelling
Eduarda G Z Centeno, Helena Cimarosti, Angela Bithell
Targeting energy metabolism via the mitochondrial pyruvate carrier as a novel approach to attenuate neurodegeneration
Emmanuel Quansah, Wouter Peelaerts, J. William Langston, David K. Simon, Jerry Colca, Patrik Brundin
The Trem2 R47H variant confers loss-of-function-like phenotypes in Alzheimer’s disease
Paul J. Cheng-Hathaway, Erin G. Reed-Geaghan, Taylor R. Jay, Brad T. Casali, Shane M. Bemiller, Shweta S. Puntambekar, Victoria E. von Saucken, Roxanne Y. Williams, J. Colleen Karlo, Miguel Moutinho, Guixiang Xu, Richard M. Ransohoff, Bruce T. Lamb, Gary E. Landreth
Protective paraspeckle hyper-assembly downstream of TDP-43 loss of function in amyotrophic lateral sclerosis
Tatyana A. Shelkovnikova, Michail S. Kukharsky, Haiyan An, Pasquale Dimasi, Svetlana Alexeeva, Osman Shabir, Paul R. Heath, Vladimir L. Buchman
Transcriptional profiling and biomarker identification reveal tissue specific effects of expanded ataxin-3 in a spinocerebellar ataxia type 3 mouse model
Lodewijk J. A. Toonen, Maurice Overzier, Melvin M. Evers, Leticia G. Leon, Sander A. J. van der Zeeuw, Hailiang Mei, Szymon M. Kielbasa, Jelle J. Goeman, Kristina M. Hettne, Olafur Th. Magnusson, Marion Poirel, Alexandre Seyer, Peter A. C. ‘t Hoen, Willeke M. C. van Roon-Mom
Partial Tmem106b reduction does not correct abnormalities due to progranulin haploinsufficiency
Andrew E. Arrant, Alexandra M. Nicholson, Xiaolai Zhou, Rosa Rademakers, Erik D. Roberson
iPSC-derived familial Alzheimer’s PSEN2 N141I cholinergic neurons exhibit mutation-dependent molecular pathology corrected by insulin signaling
Cesar L. Moreno, Lucio Della Guardia, Valeria Shnyder, Maitane Ortiz-Virumbrales, Ilya Kruglikov, Bin Zhang, Eric E. Schadt, Rudolph E. Tanzi, Scott Noggle, Christoph Buettner, Sam Gandy
Quantitative proteomics of acutely-isolated mouse microglia identifies novel immune Alzheimer’s disease-related proteins
Srikant Rangaraju, Eric B. Dammer, Syed Ali Raza, Tianwen Gao, Hailian Xiao, Ranjita Betarbet, Duc M. Duong, James A. Webster, Chadwick M. Hales, James J. Lah, Allan I. Levey, Nicholas T. Seyfried
TMEM106B haplotypes have distinct gene expression patterns in aged brain
Yingxue Ren, Marka van Blitterswijk, Mariet Allen, Minerva M. Carrasquillo, Joseph S. Reddy, Xue Wang, Thomas G. Beach, Dennis W. Dickson, Nilüfer Ertekin-Taner, Yan W. Asmann, Rosa Rademakers
Clinical spectrum and genetic landscape for hereditary spastic paraplegias in China
En-Lin Dong, Chong Wang, Shuang Wu, Ying-Qian Lu, Xiao-Hong Lin, Hui-Zhen Su, Miao Zhao, Jin He, Li-Xiang Ma, Ning Wang, Wan-Jin Chen, Xiang Lin
Replication of progressive supranuclear palsy genome-wide association study identifies SLCO1A2 and DUSP10 as new susceptibility loci
Monica Y. Sanchez-Contreras, Naomi Kouri, Casey N. Cook, Daniel J. Serie, Michael G. Heckman, NiCole A. Finch, Richard J. Caselli, Ryan J. Uitti, Zbigniew K. Wszolek, Neill Graff-Radford, Leonard Petrucelli, Li-San Wang, Gerard D. Schellenberg, Dennis W. Dickson, Rosa Rademakers, Owen A. Ross
Polygenic analysis of inflammatory disease variants and effects on microglia in the aging brain
Daniel Felsky, Ellis Patrick, Julie A. Schneider, Sara Mostafavi, Chris Gaiteri, Nikolaos Patsopoulos, David A. Bennett, Philip L. De Jager
Transcriptional profiling of HERV-K(HML-2) in amyotrophic lateral sclerosis and potential implications for expression of HML-2 proteins
Jens Mayer, Christian Harz, Laura Sanchez, Gavin C. Pereira, Esther Maldener, Sara R. Heras, Lyle W. Ostrow, John Ravits, Ranjan Batra, Eckart Meese, Jose Luis García-Pérez, John L. Goodier
A new hypothesis for Parkinson’s disease pathogenesis: GTPase-p38 MAPK signaling and autophagy as convergence points of etiology and genomics
Julia Obergasteiger, Giulia Frapporti, Peter P. Pramstaller, Andrew A. Hicks, Mattia Volta
Joint genome-wide association study of progressive supranuclear palsy identifies novel susceptibility loci and genetic correlation to neurodegenerative diseases
Jason A. Chen, Zhongbo Chen, Hyejung Won, Alden Y. Huang, Jennifer K. Lowe, Kevin Wojta, Jennifer S. Yokoyama, Gilbert Bensimon, P. Nigel Leigh, Christine Payan, Aleksey Shatunov, Ashley R. Jones, Cathryn M. Lewis, Panagiotis Deloukas, Philippe Amouyel, Christophe Tzourio, Jean-Francois Dartigues, Albert Ludolph, Adam L. Boxer, Jeff M. Bronstein, Ammar Al-Chalabi, Daniel H. Geschwind, Giovanni Coppola
Counteracting roles of MHCI and CD8+ T cells in the peripheral and central nervous system of ALS SOD1G93A mice
Giovanni Nardo, Maria Chiara Trolese, Mattia Verderio, Alessandro Mariani, Massimiliano de Paola, Nilo Riva, Giorgia Dina, Nicolò Panini, Eugenio Erba, Angelo Quattrini, Caterina Bendotti
Immunotherapy targeting toll-like receptor 2 alleviates neurodegeneration in models of synucleinopathy by modulating α-synuclein transmission and neuroinflammation
Changyoun Kim, Brian Spencer, Edward Rockenstein, Hodaka Yamakado, Michael Mante, Anthony Adame, Jerel Adam Fields, Deborah Masliah, Michiyo Iba, He-Jin Lee, Robert A. Rissman, Seung-Jae Lee, Eliezer Masliah
PU.1 regulates Alzheimer’s disease-associated genes in primary human microglia
Justin Rustenhoven, Amy M. Smith, Leon C. Smyth, Deidre Jansson, Emma L. Scotter, Molly E. V. Swanson, Miranda Aalderink, Natacha Coppieters, Pritika Narayan, Renee Handley, Chris Overall, Thomas I. H. Park, Patrick Schweder, Peter Heppner, Maurice A. Curtis, Richard L. M. Faull, Mike Dragunow
Subretinal macrophages produce classical complement activator C1q leading to the progression of focal retinal degeneration
Haihan Jiao, Matt Rutar, Nilisha Fernando, Ted Yednock, Sethu Sankaranarayanan, Riemke Aggio-Bruce, Jan Provis, Riccardo Natoli
Long-read sequencing across the C9orf72 ‘GGGGCC’ repeat expansion: implications for clinical use and genetic discovery efforts in human disease
Mark T. W. Ebbert, Stefan L. Farrugia, Jonathon P. Sens, Karen Jansen-West, Tania F. Gendron, Mercedes Prudencio, Ian J. McLaughlin, Brett Bowman, Matthew Seetin, Mariely DeJesus-Hernandez, Jazmyne Jackson, Patricia H. Brown, Dennis W. Dickson, Marka van Blitterswijk, Rosa Rademakers, Leonard Petrucelli, John D. Fryer
Alzheimer-associated cerebrospinal fluid fragments of neurogranin are generated by Calpain-1 and prolyl endopeptidase
Bruno Becker, Faisal Hayat Nazir, Gunnar Brinkmalm, Elena Camporesi, Hlin Kvartsberg, Erik Portelius, Martina Boström, Marie Kalm, Kina Höglund, Maria Olsson, Henrik Zetterberg, Kaj Blennow
Early lysosomal maturation deficits in microglia triggers enhanced lysosomal activity in other brain cells of progranulin knockout mice
Julia K. Götzl, Alessio-Vittorio Colombo, Katrin Fellerer, Anika Reifschneider, Georg Werner, Sabina Tahirovic, Christian Haass, Anja Capell
The Trem2 R47H Alzheimer’s risk variant impairs splicing and reduces Trem2 mRNA and protein in mice but not in humans
Xianyuan Xiang, Thomas M. Piers, Benedikt Wefers, Kaichuan Zhu, Anna Mallach, Bettina Brunner, Gernot Kleinberger, Wilbur Song, Marco Colonna, Jochen Herms, Wolfgang Wurst, Jennifer M. Pocock, Christian Haass
Modifying Rap1-signalling by targeting Pde6δ is neuroprotective in models of Alzheimer’s disease
Michael Dumbacher, Tom Van Dooren, Katrien Princen, Koen De Witte, Mélissa Farinelli, Sam Lievens, Jan Tavernier, Wim Dehaen, Stefaan Wera, Joris Winderickx, Sara Allasia, Amuri Kilonda, Stéphane Spieser, Arnaud Marchand, Patrick Chaltin, Casper C. Hoogenraad, Gerard Griffioen
Deep proteomic network analysis of Alzheimer’s disease brain reveals alterations in RNA binding proteins and RNA splicing associated with disease
Erik C. B. Johnson, Eric B. Dammer, Duc M. Duong, Luming Yin, Madhav Thambisetty, Juan C. Troncoso, James J. Lah, Allan I. Levey, Nicholas T. Seyfried
ABI3 and PLCG2 missense variants as risk factors for neurodegenerative diseases in Caucasians and African Americans
Olivia J Conway, Minerva M Carrasquillo, Xue Wang, Jenny M Bredenberg, Joseph S Reddy, Samantha L Strickland, Curtis S Younkin, Jeremy D Burgess, Mariet Allen, Sarah J Lincoln, Thuy Nguyen, Kimberly G Malphrus, Alexandra I Soto, Ronald L Walton, Bradley F Boeve, Ronald C Petersen, John A Lucas, Tanis J Ferman, William P Cheshire, Jay A van Gerpen, Ryan J Uitti, Zbigniew K Wszolek, Owen A Ross, Dennis W Dickson, Neill R Graff-Radford, Nilüfer Ertekin-Taner
Deregulation of neuronal miRNAs induced by amyloid-β or TAU pathology
Annerieke Sierksma, Ashley Lu, Evgenia Salta, Elke Vanden Eynden, Zsuzsanna Callaerts-Vegh, Rudi D’Hooge, David Blum, Luc Buée, Mark Fiers, Bart De Strooper
Progranulin reduces insoluble TDP-43 levels, slows down axonal degeneration and prolongs survival in mutant TDP-43 mice
Sander Beel, Sarah Herdewyn, Raheem Fazal, Mathias De Decker, Matthieu Moisse, Wim Robberecht, Ludo Van Den Bosch, Philip Van Damme
Essential roles of mitochondrial biogenesis regulator Nrf1 in retinal development and homeostasis
Takae Kiyama, Ching-Kang Chen, Steven W Wang, Ping Pan, Zhenlin Ju, Jing Wang, Shinako Takada, William H Klein, Chai-An Mao
Blood-brain barrier-associated pericytes internalize and clear aggregated amyloid-β42 by LRP1-dependent apolipoprotein E isoform-specific mechanism
Qingyi Ma, Zhen Zhao, Abhay P Sagare, Yingxi Wu, Min Wang, Nelly Chuqui Owens, Philip B Verghese, Joachim Herz, David M Holtzman, Berislav V Zlokovic
Correction to: Peripheral immune system in aging and Alzheimer’s disease
Wei Cao, Hui Zheng
A brain-penetrant triazolopyrimidine enhances microtubule-stability, reduces axonal dysfunction and decreases tau pathology in a mouse tauopathy model
Bin Zhang, Yuemang Yao, Anne-Sophie Cornec, Killian Oukoloff, Michael J. James, Pyry Koivula, John Q. Trojanowski, Amos B. Smith III, Virginia M.-Y. Lee, Carlo Ballatore, Kurt R. Brunden
Tissue-enhanced plasma proteomic analysis for disease stratification in amyotrophic lateral sclerosis
Irene Zubiri, Vittoria Lombardi, Michael Bremang, Vikram Mitra, Giovanni Nardo, Rocco Adiutori, Ching-Hua Lu, Emanuela Leoni, Ping Yip, Ozlem Yildiz, Malcolm Ward, Linda Greensmith, Caterina Bendotti, Ian Pike, Andrea Malaspina
Functional alterations of myeloid cells during the course of Alzheimer’s disease
Aaron D. Thome, Alireza Faridar, David R. Beers, Jason R. Thonhoff, Weihua Zhao, Shixiang Wen, Belen Pascual, Joseph C. Masdeu, Stanley H. Appel
GSK3β-mediated tau hyperphosphorylation triggers diabetic retinal neurodegeneration by disrupting synaptic and mitochondrial functions
Huazhang Zhu, Weizhen Zhang, Yingying Zhao, Xingsheng Shu, Wencong Wang, Dandan Wang, Yangfan Yang, Zhijun He, Xiaomei Wang, Ying Ying
Disease-modifying effects of metabolic perturbations in ALS/FTLD
Ali Jawaid, Romesa Khan, Magdalini Polymenidou, Paul E. Schulz
Advances in developing novel therapeutic strategies for Alzheimer’s disease
Jiqing Cao, Jianwei Hou, Jing Ping, Dongming Cai
Genome-wide RNAseq study of the molecular mechanisms underlying microglia activation in response to pathological tau perturbation in the rTg4510 tau transgenic animal model
Hong Wang, Yupeng Li, John W. Ryder, Justin T. Hole, Philip J. Ebert, David C. Airey, Hui-Rong Qian, Benjamin Logsdon, Alice Fisher, Zeshan Ahmed, Tracey K. Murray, Annalisa Cavallini, Suchira Bose, Brian J. Eastwood, David A. Collier, Jeffrey L. Dage, Bradley B. Miller, Kalpana M. Merchant, Michael J. O’Neill, Ronald B. Demattos
New insights into the role of TREM2 in Alzheimer’s disease
Maud Gratuze, Cheryl E. G. Leyns, David M. Holtzman
Development and validation of a simplified method to generate human microglia from pluripotent stem cells
Amanda McQuade, Morgan Coburn, Christina H. Tu, Jonathan Hasselmann, Hayk Davtyan, Mathew Blurton-Jones
Proteolytic cleavage of Beclin 1 exacerbates neurodegeneration
Gregor Bieri, Kurt M. Lucin, Caitlin E. O’Brien, Hui Zhang, Saul A. Villeda, Tony Wyss-Coray