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  1. Content type: Research

    Amyloid plaques formed by abnormal prion protein (PrPSc) aggregates occur with low frequency in Creutzfeldt-Jakob disease, but represent a pathological hallmark of three relatively rare disease histotypes, namely...

    Authors: Marcello Rossi, Daniela Saverioni, Michele Di Bari, Simone Baiardi, Afina Willemina Lemstra, Laura Pirisinu, Sabina Capellari, Annemieke Rozemuller, Romolo Nonno and Piero Parchi

    Citation: Acta Neuropathologica Communications 2017 5:87

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  2. Content type: Research

    Missense mutations in the leucine-rich repeat kinase 2 (LRRK2) gene can cause late-onset Parkinson disease (PD). LRRK2 mutations increase LRRK2 kinase activities that may increase levels of LRRK2 autophosphorylat...

    Authors: Shijie Wang, Zhiyong Liu, Tao Ye, Omar S. Mabrouk, Tyler Maltbie, Jan Aasly and Andrew B. West

    Citation: Acta Neuropathologica Communications 2017 5:86

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  3. Content type: Methodology article

    The larger brain of the rat enables a much greater repertoire of complex behaviors than mice, likely making rats preferential for investigating neurodegeneration. Because molecular tools for specific expressio...

    Authors: T. Peter Lopez, Kurt Giles, Brittany N. Dugger, Abby Oehler, Carlo Condello, Zuzana Krejciova, Julian A. Castaneda, George A. Carlson and Stanley B. Prusiner

    Citation: Acta Neuropathologica Communications 2017 5:84

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  4. Content type: Research

    Genetic variation in a major histocompatibility complex II (MHCII)-encoding gene (HLA-DR) increases risk for Parkinson disease (PD), and the accumulation of MHCII-expressing immune cells in the brain correlates w...

    Authors: Ashley S. Harms, Vedad Delic, Aaron D. Thome, Nicole Bryant, Zhiyong Liu, Sidhanth Chandra, Asta Jurkuvenaite and Andrew B. West

    Citation: Acta Neuropathologica Communications 2017 5:85

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  5. Content type: Research

    Endothelin-converting enzyme-like 1 (ECEL1, also termed DINE in rodents), a membrane-bound metalloprotease, has been identified as a gene responsible for distal arthrogryposis (DA). ECEL1-mutated DA is generally ...

    Authors: Kenichi Nagata, Mika Takahashi, Sumiko Kiryu-Seo, Hiroshi Kiyama and Takaomi C. Saido

    Citation: Acta Neuropathologica Communications 2017 5:83

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  6. Content type: Research

    Blast-related traumatic brain injury (TBI) has been a common cause of injury in the recent conflicts in Iraq and Afghanistan. Blast waves can damage blood vessels, neurons, and glial cells within the brain. Ac...

    Authors: Miguel A. Gama Sosa, Rita De Gasperi, Georgina S. Perez Garcia, Heidi Sosa, Courtney Searcy, Danielle Vargas, Pierce L. Janssen, Gissel M. Perez, Anna E. Tschiffely, William G. Janssen, Richard M. McCarron, Patrick R. Hof, Fatemeh G. Haghighi, Stephen T. Ahlers and Gregory A. Elder

    Citation: Acta Neuropathologica Communications 2017 5:80

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  7. Content type: Research

    It is now widely accepted in the field that the normally secreted chaperone clusterin is redirected to the cytosol during endoplasmic reticulum (ER) stress, although the physiological function(s) of this physi...

    Authors: Jenna M. Gregory, Daniel R. Whiten, Rebecca A. Brown, Teresa P. Barros, Janet R. Kumita, Justin J. Yerbury, Sandeep Satapathy, Karina McDade, Colin Smith, Leila M. Luheshi, Christopher M. Dobson and Mark R. Wilson

    Citation: Acta Neuropathologica Communications 2017 5:81

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  8. Content type: Research

    Hypersynchronous neuronal excitation manifests clinically as seizure (ictogenesis), and may recur spontaneously and repetitively after a variable latency period (epileptogenesis). Despite tremendous research e...

    Authors: K. Kiese, J. Jablonski, J. Hackenbracht, J. K. Wrosch, T. W. Groemer, J. Kornhuber, I. Blümcke and K. Kobow

    Citation: Acta Neuropathologica Communications 2017 5:79

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  9. Content type: Research

    Pediatric high-grade gliomas (pHGGs) are aggressive neoplasms representing approximately 20% of brain tumors in children. Current therapies offer limited disease control, and patients have a poor prognosis. Em...

    Authors: Ralph Salloum, Melissa K. McConechy, Leonie G. Mikael, Christine Fuller, Rachid Drissi, Mariko DeWire, Hamid Nikbakht, Nicolas De Jay, Xiaodan Yang, Daniel Boue, Lionel M. L. Chow, Jonathan L. Finlay, Tenzin Gayden, Jason Karamchandani, Trent R. Hummel, Randal Olshefski…

    Citation: Acta Neuropathologica Communications 2017 5:78

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  10. Content type: Research

    Basal forebrain cholinergic neurons (BFCNs) are believed to be one of the first cell types to be affected in all forms of AD, and their dysfunction is clinically correlated with impaired short-term memory form...

    Authors: Maitane Ortiz-Virumbrales, Cesar L. Moreno, Ilya Kruglikov, Paula Marazuela, Andrew Sproul, Samson Jacob, Matthew Zimmer, Daniel Paull, Bin Zhang, Eric E. Schadt, Michelle E. Ehrlich, Rudolph E. Tanzi, Ottavio Arancio, Scott Noggle and Sam Gandy

    Citation: Acta Neuropathologica Communications 2017 5:77

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  11. Content type: Research

    The identification of the TAR DNA-binding protein 43 (TDP-43) as the ubiquitinated cytoplasmic inclusions in frontotemporal lobar degeneration (FTLD) and amyotrophic lateral sclerosis (ALS) confirmed that thes...

    Authors: Rachel H. Tan, Yue Yang, Woojin S. Kim, Carol Dobson-Stone, John B. Kwok, Matthew C. Kiernan and Glenda M. Halliday

    Citation: Acta Neuropathologica Communications 2017 5:76

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  12. Content type: Research

    [F-18]-AV-1451 is a novel positron emission tomography (PET) tracer with high affinity to neurofibrillary tau pathology in Alzheimer’s disease (AD). PET studies have shown increased tracer retention in patient...

    Authors: Marta Marquié, Eline E. Verwer, Avery C. Meltzer, Sally Ji Who Kim, Cinthya Agüero, Jose Gonzalez, Sara J. Makaretz, Michael Siao Tick Chong, Prianca Ramanan, Ana C. Amaral, Marc D. Normandin, Charles R. Vanderburg, Stephen N. Gomperts, Keith A. Johnson, Matthew P. Frosch and Teresa Gómez-Isla

    Citation: Acta Neuropathologica Communications 2017 5:75

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  13. Content type: Research

    The neuronal ceroid lipofuscinoses (NCLs or Batten disease) are a group of inherited, fatal neurodegenerative disorders of childhood. In these disorders, glial (microglial and astrocyte) activation typically o...

    Authors: Lotta Parviainen, Sybille Dihanich, Greg W. Anderson, Andrew M. Wong, Helen R. Brooks, Rosella Abeti, Payam Rezaie, Giovanna Lalli, Simon Pope, Simon J. Heales, Hannah M. Mitchison, Brenda P. Williams and Jonathan D. Cooper

    Citation: Acta Neuropathologica Communications 2017 5:74

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  14. Content type: Research

    Amyloid β (Aβ) deposition in the brain is an early and invariable feature of Alzheimer’s disease (AD). The Aβ peptides are composed of about 40 amino acids and are generated from amyloid precursor proteins (AP...

    Authors: Nobuto Kakuda, Tomohiro Miyasaka, Noriyuki Iwasaki, Takashi Nirasawa, Satoko Wada-Kakuda, Junko Takahashi-Fujigasaki, Shigeo Murayama, Yasuo Ihara and Masaya Ikegawa

    Citation: Acta Neuropathologica Communications 2017 5:73

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  15. Content type: Research

    Myopathies encompass a wide variety of acquired and hereditary disorders. The pathomechanisms include structural and functional changes affecting, e.g., myofiber metabolism and contractile properties. In this ...

    Authors: Andreas Unger, Lisa Beckendorf, Pierre Böhme, Rudolf Kley, Marion von Frieling-Salewsky, Hanns Lochmüller, Rolf Schröder, Dieter O. Fürst, Matthias Vorgerd and Wolfgang A. Linke

    Citation: Acta Neuropathologica Communications 2017 5:72

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  16. Content type: Research

    Parkinson’s disease (PD) pathophysiology develops in part from the formation, transmission, and aggregation of toxic species of the protein α-synuclein (α-syn). Recent evidence suggests that extracellular vesi...

    Authors: Junichi Matsumoto, Tessandra Stewart, Lifu Sheng, Na Li, Kristin Bullock, Ning Song, Min Shi, William A Banks and Jing Zhang

    Citation: Acta Neuropathologica Communications 2017 5:71

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  17. Content type: Research

    Systemic lupus erythematosus (SLE) is a potentially fatal autoimmune disease that is often accompanied by brain atrophy and diverse neuropsychiatric manifestations of unknown origin. More recently, it was obse...

    Authors: Minesh Kapadia, Dunja Bijelić, Hui Zhao, Donglai Ma, Ljudmila Stojanovich, Milena Milošević, Pavle Andjus and Boris Šakić

    Citation: Acta Neuropathologica Communications 2017 5:70

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  18. Content type: Research

    The pathology of Alzheimer’s disease (AD) is characterized by dystrophic neurites (DNs) surrounding extracellular Aβ-plaques, microgliosis, astrogliosis, intraneuronal tau hyperphosphorylation and aggregation....

    Authors: Jonas Elias Schweig, Hailan Yao, David Beaulieu-Abdelahad, Ghania Ait-Ghezala, Benoit Mouzon, Fiona Crawford, Michael Mullan and Daniel Paris

    Citation: Acta Neuropathologica Communications 2017 5:69

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  19. Content type: Research

    Frontotemporal lobar degeneration with TDP-43 inclusions (FTLD-TDP) is associated with the accumulation of pathological neuronal and glial intracytoplasmic inclusions as well as accompanying neuron loss. We ex...

    Authors: Ahmed Yousef, John L. Robinson, David J. Irwin, Matthew D. Byrne, Linda K. Kwong, Edward B. Lee, Yan Xu, Sharon X. Xie, Lior Rennert, EunRan Suh, Vivianna M. Van Deerlin, Murray Grossman, Virginia M.-Y. Lee and John Q. Trojanowski

    Citation: Acta Neuropathologica Communications 2017 5:68

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  20. Content type: Research

    The aggregation mechanism of phosphorylated tau is an important therapeutic target for tauopathies, including Alzheimer’s disease, although the mechanism by which aggregation occurs is still unknown. Because t...

    Authors: Tetsuya Kimura, Mamiko Suzuki and Takumi Akagi

    Citation: Acta Neuropathologica Communications 2017 5:67

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  21. Content type: Research

    Pompe disease is a lysosomal storage disorder caused by acid-α-glucosidase (GAA) deficiency, leading to glycogen storage. The disease manifests as a fatal cardiomyopathy in infantile form. Enzyme replacement t...

    Authors: J. Hordeaux, L. Dubreil, C. Robveille, J. Deniaud, Q. Pascal, B. Dequéant, J. Pailloux, L. Lagalice, M. Ledevin, C. Babarit, P. Costiou, F. Jamme, M. Fusellier, Y. Mallem, C. Ciron, C. Huchet…

    Citation: Acta Neuropathologica Communications 2017 5:66

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  22. Content type: Research

    A dysfunctional endosomal pathway and abnormally enlarged early endosomes in neurons are an early characteristic of Down syndrome (DS) and Alzheimer’s disease (AD). We have hypothesized that endosomal material...

    Authors: Sébastien A. Gauthier, Rocío Pérez-González, Ajay Sharma, Fang-Ke Huang, Melissa J. Alldred, Monika Pawlik, Gurjinder Kaur, Stephen D. Ginsberg, Thomas A. Neubert and Efrat Levy

    Citation: Acta Neuropathologica Communications 2017 5:65

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  23. Content type: Research

    Mutations in the telomerase reverse transcriptase gene promoter (TERTp) are common in glioblastomas (GBMs) and oligodendrogliomas (ODGs), and therefore, have a key role in tumorigenesis and may be of prognostic v...

    Authors: Yujin Lee, Jaemoon Koh, Seong-Ik Kim, Jae Kyung Won, Chul-Kee Park, Seung Hong Choi and Sung-Hye Park

    Citation: Acta Neuropathologica Communications 2017 5:62

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  24. Content type: Research

    Spatiotemporal tau pathology progression is regarded as highly stereotyped within each type of degenerative condition. For instance, AD has a progression of tau pathology consistently beginning in the entorhin...

    Authors: Chris Mezias, Eve LoCastro, Chuying Xia and Ashish Raj

    Citation: Acta Neuropathologica Communications 2017 5:61

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  25. Content type: Research

    Tauopathies are a group of neurodegenerative disorders, including Alzheimer’s disease, defined by the presence of brain pathological inclusions comprised of abnormally aggregated and highly phosphorylated tau ...

    Authors: Kevin H. Strang, Marshall S. Goodwin, Cara Riffe, Brenda D. Moore, Paramita Chakrabarty, Yona Levites, Todd E. Golde and Benoit I. Giasson

    Citation: Acta Neuropathologica Communications 2017 5:58

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  26. Content type: Research

    The E693Δ (Osaka) mutation in APP is linked to familial Alzheimer’s disease. While this mutation accelerates amyloid β (Aβ) oligomerization, only patient homozygotes suffer from dementia, implying that this mutat...

    Authors: Tomohiro Umeda, Tetsuya Kimura, Kayo Yoshida, Keizo Takao, Yuki Fujita, Shogo Matsuyama, Ayumi Sakai, Minato Yamashita, Yuki Yamashita, Kiyouhisa Ohnishi, Mamiko Suzuki, Hiroshi Takuma, Tsuyoshi Miyakawa, Akihiko Takashima, Takashi Morita, Hiroshi Mori…

    Citation: Acta Neuropathologica Communications 2017 5:59

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  27. Content type: Research

    Alzheimer’s disease (AD) is histopathologically characterized by the build-up of fibrillar amyloid beta (Aβ) in the form of amyloid plaques and the development of intraneuronal neurofibrillary tangles consisti...

    Authors: Juan Pablo Palavicini, Chunyan Wang, Linyuan Chen, Kristen Hosang, Jianing Wang, Takami Tomiyama, Hiroshi Mori and Xianlin Han

    Citation: Acta Neuropathologica Communications 2017 5:56

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  28. Content type: Research

    Pathogenesis in aquaporin-4 immunoglobulin G (AQP4-IgG) seropositive neuromyelitis optica spectrum disorders (herein called NMO) involves complement-dependent cytotoxicity initiated by AQP4-IgG binding to astr...

    Authors: Xiaoming Yao and Alan S. Verkman

    Citation: Acta Neuropathologica Communications 2017 5:57

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  29. Content type: Research

    Neurofilament heavy chain (NEFH) gene was recently identified to cause autosomal dominant axonal Charcot-Marie-Tooth disease (CMT2cc). However, the clinical spectrum of this condition and the physio-pathological ...

    Authors: Arnaud Jacquier, Cécile Delorme, Edwige Belotti, Raoul Juntas-Morales, Guilhem Solé, Odile Dubourg, Marianne Giroux, Claude-Alain Maurage, Valérie Castellani, Adriana Rebelo, Alexander Abrams, Stephan Züchner, Tanya Stojkovic, Laurent Schaeffer and Philippe Latour

    Citation: Acta Neuropathologica Communications 2017 5:55

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  30. Content type: Research

    Frontotemporal Lobar Degeneration (FTLD) encompasses certain related neurodegenerative disorders which alter personality and cognition. Heterogeneous ribonuclear proteins (hnRNPs) maintain RNA metabolism and c...

    Authors: Yvonne S. Davidson, Andrew C. Robinson, Louis Flood, Sara Rollinson, Bridget C. Benson, Yasmine T. Asi, Anna Richardson, Matthew Jones, Julie S. Snowden, Stuart Pickering-Brown, Tammaryn Lashley and David M. A. Mann

    Citation: Acta Neuropathologica Communications 2017 5:54

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  31. Content type: Research

    Elevated iron in the SNpc may play a key role in Parkinson’s disease (PD) neurodegeneration since drug candidates with high iron affinity rescue PD animal models, and one candidate, deferirpone, has shown effi...

    Authors: David I. Finkelstein, Jessica L. Billings, Paul A. Adlard, Scott Ayton, Amelia Sedjahtera, Colin L. Masters, Simon Wilkins, David M. Shackleford, Susan A. Charman, Wojciech Bal, Izabela A Zawisza, Ewa Kurowska, Andrew L. Gundlach, Sheri Ma, Ashley I. Bush, Dominic J. Hare…

    Citation: Acta Neuropathologica Communications 2017 5:53

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  32. Content type: Research

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

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

    Citation: Acta Neuropathologica Communications 2017 5:52

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  33. Content type: Research

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

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

    Citation: Acta Neuropathologica Communications 2017 5:50

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  34. Content type: Research

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

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

    Citation: Acta Neuropathologica Communications 2017 5:51

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  35. Content type: Research

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

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

    Citation: Acta Neuropathologica Communications 2017 5:49

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  36. Content type: Research

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

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

    Citation: Acta Neuropathologica Communications 2017 5:47

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  37. Content type: Research

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

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

    Citation: Acta Neuropathologica Communications 2017 5:48

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  38. Content type: Research

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

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

    Citation: Acta Neuropathologica Communications 2017 5:43

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  39. Content type: Research

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

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

    Citation: Acta Neuropathologica Communications 2017 5:46

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  40. Content type: Methodology article

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

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

    Citation: Acta Neuropathologica Communications 2017 5:41

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  41. Content type: Research

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

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

    Citation: Acta Neuropathologica Communications 2017 5:44

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  42. Content type: Research

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

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

    Citation: Acta Neuropathologica Communications 2017 5:45

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  43. Content type: Research

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

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

    Citation: Acta Neuropathologica Communications 2017 5:42

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  44. Content type: Research

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

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

    Citation: Acta Neuropathologica Communications 2017 5:40

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  45. Content type: Research

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

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

    Citation: Acta Neuropathologica Communications 2017 5:39

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