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Acta Neuropathologica Communications

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https://www.readbyqxmd.com/read/30231927/oligodendrogliomas-idh-mutant-and-1p-19q-codeleted-arising-during-teenage-years-often-lack-tert-promoter-mutation-that-is-typical-of-their-adult-counterparts
#1
LETTER
Julieann Lee, Angelica R Putnam, Samuel H Chesier, Anuradha Banerjee, Corey Raffel, Jessica Van Ziffle, Courtney Onodera, James P Grenert, Boris C Bastian, Arie Perry, David A Solomon
No abstract text is available yet for this article.
September 19, 2018: Acta Neuropathologica Communications
https://www.readbyqxmd.com/read/30231908/rapid-dissemination-of-alpha-synuclein-seeds-through-neural-circuits-in-an-in-vivo-prion-like-seeding-experiment
#2
Ayami Okuzumi, Masaru Kurosawa, Taku Hatano, Masashi Takanashi, Shuuko Nojiri, Takeshi Fukuhara, Tomoyuki Yamanaka, Haruko Miyazaki, Saki Yoshinaga, Yoshiaki Furukawa, Tomomi Shimogori, Nobutaka Hattori, Nobuyuki Nukina
Accumulating evidence suggests that the lesions of Parkinson's disease (PD) expand due to transneuronal spreading of fibrils composed of misfolded alpha-synuclein (a-syn), over the course of 5-10 years. However, the precise mechanisms and the processes underlying the spread of these fibril seeds have not been clarified in vivo. Here, we investigated the speed of a-syn transmission, which has not been a focus of previous a-syn transmission experiments, and whether a-syn pathologies spread in a neural circuit-dependent manner in the mouse brain...
September 19, 2018: Acta Neuropathologica Communications
https://www.readbyqxmd.com/read/30208948/core-rod-myopathy-due-to-a-novel-mutation-in-btb-poz-domain-of-kbtbd13-manifesting-as-late-onset-lgmd
#3
LETTER
Matteo Garibaldi, Fabiana Fattori, Carlo Augusto Bortolotti, Guy Brochier, Clemence Labasse, Margherita Verardo, Emilia Servian-Morilla, Lara Gibellini, Marcello Pinti, Giulia Di Rocco, Salvatore Raffa, Elena Maria Pennisi, Enrico Silvio Bertini, Carmen Paradas, Norma Beatriz Romero, Giovanni Antonini
No abstract text is available yet for this article.
September 13, 2018: Acta Neuropathologica Communications
https://www.readbyqxmd.com/read/30208966/preserving-prion-strain-identity-upon-replication-of-prions-in-vitro-using-recombinant-prion-protein
#4
Natallia Makarava, Regina Savtchenko, Peter Lasch, Michael Beekes, Ilia V Baskakov
Last decade witnessed an enormous progress in generating authentic infectious prions or PrPSc in vitro using recombinant prion protein (rPrP). Previous work established that rPrP that lacks posttranslational modification is able to support replication of highly infectious PrPSc with assistance of cofactors of polyanionic nature and/or lipids. Unexpectedly, previous studies also revealed that seeding of rPrP by brain-derived PrPSc gave rise to new prion strains with new disease phenotypes documenting loss of a strain identity upon replication in rPrP substrate...
September 12, 2018: Acta Neuropathologica Communications
https://www.readbyqxmd.com/read/30208955/some-dnm2-mutations-cause-extremely-severe-congenital-myopathy-and-phenocopy-myotubular-myopathy
#5
LETTER
Valérie Biancalana, Norma B Romero, Inger Johanne Thuestad, Jaakko Ignatius, Janne Kataja, Maria Gardberg, Delphine Héron, Edoardo Malfatti, Anders Oldfors, Jocelyn Laporte
No abstract text is available yet for this article.
September 12, 2018: Acta Neuropathologica Communications
https://www.readbyqxmd.com/read/30208949/the-complement-system-in-glioblastoma-multiforme
#6
REVIEW
T A M Bouwens van der Vlis, J M Kros, D A M Mustafa, R T A van Wijck, L Ackermans, P M van Hagen, P J van der Spek
The human complement system is represents the main effector arm of innate immunity and its ambivalent function in cancer has been subject of ongoing dispute. Glioma stem-like cells (GSC) residing in specific niches within glioblastomas (GBM) are capable of self-renewal and tumor proliferation. Recent data are indicative of the influence of the complement system on the maintenance of these cells. It appears that the role of the complement system in glial tumorigenesis, particularly its influence on GSC niches and GSC maintenance, is significant and warrants further exploration for therapeutic interventions...
September 12, 2018: Acta Neuropathologica Communications
https://www.readbyqxmd.com/read/30201049/degeneration-of-human-photosensitive-retinal-ganglion-cells-may-explain-sleep-and-circadian-rhythms-disorders-in-parkinson-s-disease
#7
Isabel Ortuño-Lizarán, Gema Esquiva, Thomas G Beach, Geidy E Serrano, Charles H Adler, Pedro Lax, Nicolás Cuenca
Parkinson's disease (PD) patients often suffer from non-motor symptoms like sleep dysregulation, mood disturbances or circadian rhythms dysfunction. The melanopsin-containing retinal ganglion cells are involved in the control and regulation of these processes and may be affected in PD, as other retinal and visual implications have been described in the disease. Number and morphology of human melanopsin-containing retinal ganglion cells were evaluated by immunohistochemistry in eyes from donors with PD or control...
September 10, 2018: Acta Neuropathologica Communications
https://www.readbyqxmd.com/read/30193587/systemic-infection-modifies-the-neuroinflammatory-response-in-late-stage-alzheimer-s-disease
#8
Sonja Rakic, Yat M A Hung, Matthew Smith, Denise So, Hannah M Tayler, William Varney, Joe Wild, Scott Harris, Clive Holmes, Seth Love, William Stewart, James A R Nicoll, Delphine Boche
Clinical studies indicate that systemic infections accelerate cognitive decline in Alzheimer's disease. Animal models suggest that this may be due to enhanced pro-inflammatory changes in the brain. We have performed a post-mortem human study to determine whether systemic infection modifies the neuropathology and in particular, neuroinflammation, in the late-stage of the disease.Sections of cerebral cortex and underlying white matter from controls and Alzheimer's patients who died with or without a terminal systemic infection were immunolabelled and quantified for: (i) Αβ and phosphorylated-tau; (ii) the inflammation-related proteins Iba1, CD68, HLA-DR, FcγRs (CD64, CD32a, CD32b, CD16), CHIL3L1, IL4R and CCR2; and (iii) T-cell marker CD3...
September 7, 2018: Acta Neuropathologica Communications
https://www.readbyqxmd.com/read/30193580/somatostatin-receptor-2a-protein-expression-characterizes-anaplastic-oligodendrogliomas-with-favorable-outcome
#9
Romain Appay, Emeline Tabouret, Mehdi Touat, Catherine Carpentier, Carole Colin, François Ducray, Ahmed Idbaih, Karima Mokhtari, Emmanuelle Uro-Coste, Caroline Dehais, Dominique Figarella-Branger
Diffuse gliomas are classified according to the 2016 WHO Classification of Tumors of the Central Nervous System, which now defines entities by both histology and molecular features. Somatostatin receptor subtype 2A (SSTR2A) expression has been reported in various solid tumors as associated with favorable outcomes. Its expression has been reported in gliomas with uncertain results regarding its prognostic value. The objective of this study was to assess the prognostic impact of SSTR2A protein expression in a large cohort of grade III and IV gliomas classified according to the updated 2016 WHO classification...
September 7, 2018: Acta Neuropathologica Communications
https://www.readbyqxmd.com/read/30185219/unique-microglia-recovery-population-revealed-by-single-cell-rnaseq-following-neurodegeneration
#10
Tuan Leng Tay, Sagar, Jana Dautzenberg, Dominic Grün, Marco Prinz
Microglia are brain immune cells that constantly survey their environment to maintain homeostasis. Enhanced microglial reactivity and proliferation are typical hallmarks of neurodegenerative diseases. Whether specific disease-linked microglial subsets exist during the entire course of neurodegeneration, including the recovery phase, is currently unclear. Taking a single-cell RNA-sequencing approach in a susceptibility gene-free model of nerve injury, we identified a microglial subpopulation that upon acute neurodegeneration shares a conserved gene regulatory profile compared to previously reported chronic and destructive neurodegeneration transgenic mouse models...
September 5, 2018: Acta Neuropathologica Communications
https://www.readbyqxmd.com/read/30176931/hypoxic-pre-conditioning-suppresses-experimental-autoimmune-encephalomyelitis-by-modifying-multiple-properties-of-blood-vessels
#11
Sebok K Halder, Ravi Kant, Richard Milner
While hypoxic pre-conditioning protects against neurological disease the underlying mechanisms have yet to be fully defined. As chronic mild hypoxia (CMH, 10% O2 ) triggers profound vascular remodeling in the central nervous system (CNS), the goal of this study was to examine the protective potential of hypoxic pre-conditioning in the experimental autoimmune encephalomyelitis (EAE) model of multiple sclerosis (MS) and then determine how CMH influences vascular integrity and the underlying cellular and molecular mechanisms during EAE...
September 3, 2018: Acta Neuropathologica Communications
https://www.readbyqxmd.com/read/30170631/analysis-of-cerebrospinal-fluid-metabolites-in-patients-with-primary-or-metastatic-central-nervous-system-tumors
#12
Leomar Y Ballester, Guangrong Lu, Soheil Zorofchian, Venkatrao Vantaku, Vasanta Putluri, Yuanqing Yan, Octavio Arevalo, Ping Zhu, Roy F Riascos, Arun Sreekumar, Yoshua Esquenazi, Nagireddy Putluri, Jay-Jiguang Zhu
Cancer cells have altered cellular metabolism. Mutations in genes associated with key metabolic pathways (e.g., isocitrate dehydrogenase 1 and 2, IDH1/IDH2) are important drivers of cancer, including central nervous system (CNS) tumors. Therefore, we hypothesized that the abnormal metabolic state of CNS cancer cells leads to abnormal levels of metabolites in the CSF, and different CNS cancer types are associated with specific changes in the levels of CSF metabolites. To test this hypothesis, we used mass spectrometry to analyze 129 distinct metabolites in CSF samples from patients without a history of cancer (n = 8) and with a variety of CNS tumor types (n = 23) (i...
August 31, 2018: Acta Neuropathologica Communications
https://www.readbyqxmd.com/read/30157964/a-novel-speg-mutation-causes-non-compaction-cardiomyopathy-and-neuropathy-in-a-floppy-infant-with-centronuclear-myopathy
#13
LETTER
Haicui Wang, Anne Schänzer, Birgit Kampschulte, Hülya-Sevcan Daimagüler, Thushiha Logeswaran, Hannah Schlierbach, Jutta Petzinger, Harald Ehrhardt, Andreas Hahn, Sebahattin Cirak
No abstract text is available yet for this article.
August 29, 2018: Acta Neuropathologica Communications
https://www.readbyqxmd.com/read/30157956/nuclear-poly-adp-ribose-activity-is-a-therapeutic-target-in-amyotrophic-lateral-sclerosis
#14
L McGurk, J Mojsilovic-Petrovic, V M Van Deerlin, J Shorter, R G Kalb, V M Lee, J Q Trojanowski, E B Lee, N M Bonini
Amyotrophic lateral sclerosis (ALS) is a devastating and fatal motor neuron disease. Diagnosis typically occurs in the fifth decade of life and the disease progresses rapidly leading to death within ~ 2-5 years of symptomatic onset. There is no cure, and the few available treatments offer only a modest extension in patient survival. A protein central to ALS is the nuclear RNA/DNA-binding protein, TDP-43. In > 95% of ALS patients, TDP-43 is cleared from the nucleus and forms phosphorylated protein aggregates in the cytoplasm of affected neurons and glia...
August 29, 2018: Acta Neuropathologica Communications
https://www.readbyqxmd.com/read/30134961/anti-tau-conformational-scfv-mc1-antibody-efficiently-reduces-pathological-tau-species-in-adult-jnpl3-mice
#15
Francesca Vitale, Luca Giliberto, Santiago Ruiz, Kristen Steslow, Philippe Marambaud, Cristina d'Abramo
Tau, the main component of the neurofibrillary tangles (NFTs), is an attractive target for immunotherapy in Alzheimer's disease (AD) and other tauopathies. MC1/Alz50 are currently the only antibodies targeting a disease-specific conformational modification of tau. Passive immunization experiments using intra-peritoneal injections have previously shown that MC1 is effective at reducing tau pathology in the forebrain of tau transgenic JNPL3 mice. In order to reach a long-term and sustained brain delivery, and avoid multiple injection protocols, we tested the efficacy of the single-chain variable fragment of MC1 (scFv-MC1) to reduce tau pathology in the same animal model, with focus on brain regional differences...
August 22, 2018: Acta Neuropathologica Communications
https://www.readbyqxmd.com/read/30129426/akt-and-mtorc1-signaling-as-predictive-biomarkers-for-the-egfr-antibody-nimotuzumab-in-glioblastoma
#16
Michael W Ronellenfitsch, Pia S Zeiner, Michel Mittelbronn, Hans Urban, Torsten Pietsch, Dirk Reuter, Christian Senft, Joachim P Steinbach, Manfred Westphal, Patrick N Harter
Glioblastoma (GB) is the most frequent primary brain tumor in adults with a dismal prognosis despite aggressive treatment including surgical resection, radiotherapy and chemotherapy with the alkylating agent temozolomide. Thus far, the successful implementation of the concept of targeted therapy where a drug targets a selective alteration in cancer cells was mainly limited to model diseases with identified genetic drivers. One of the most commonly altered oncogenic drivers of GB and therefore plausible therapeutic target is the epidermal growth factor receptor (EGFR)...
August 21, 2018: Acta Neuropathologica Communications
https://www.readbyqxmd.com/read/30111355/csf-h3f3a-k27m-circulating-tumor-dna-copy-number-quantifies-tumor-growth-and-in-vitro-treatment-response
#17
LETTER
Stefanie Stallard, Masha G Savelieff, Kyle Wierzbicki, Brendan Mullan, Zachary Miklja, Amy Bruzek, Taylor Garcia, Ruby Siada, Bailey Anderson, Benjamin H Singer, Rintaro Hashizume, Angel M Carcaboso, Kaitlin Q McMurray, Jason Heth, Karin Muraszko, Patricia L Robertson, Rajen Mody, Sriram Venneti, Hugh Garton, Carl Koschmann
No abstract text is available yet for this article.
August 15, 2018: Acta Neuropathologica Communications
https://www.readbyqxmd.com/read/30107856/membrane-binding-internalization-and-sorting-of-alpha-synuclein-in-the-cell
#18
Caterina Masaracchia, Marilena Hnida, Ellen Gerhardt, Tomás Lopes da Fonseca, Anna Villar-Pique, Tiago Branco, Markus A Stahlberg, Camin Dean, Claudio O Fernández, Ira Milosevic, Tiago F Outeiro
Alpha-synuclein (aSyn) plays a crucial role in Parkinson's disease (PD) and other synucleinopathies, since it misfolds and accumulates in typical proteinaceous inclusions. While the function of aSyn is thought to be related to vesicle binding and trafficking, the precise molecular mechanisms linking aSyn with synucleinopathies are still obscure. aSyn can spread in a prion-like manner between interconnected neurons, contributing to the propagation of the pathology and to the progressive nature of synucleinopathies...
August 14, 2018: Acta Neuropathologica Communications
https://www.readbyqxmd.com/read/30097059/e90-subunit-vaccine-protects-mice-from-zika-virus-infection-and-microcephaly
#19
Xingliang Zhu, Chunfeng Li, Shabbir Khan Afridi, Shulong Zu, Jesse W Xu, Natalie Quanquin, Heng Yang, Genhong Cheng, Zhiheng Xu
Zika virus (ZIKV) became a global threat due to its unprecedented outbreak and its association with congenital malformations such as microcephaly in developing fetuses and neonates. There are currently no effective vaccines or drugs available for the prevention or treatment of ZIKV infection. Although multiple vaccine platforms have been established, their effectiveness in preventing congenital microcephaly has not been addressed. Herein, we tested a subunit vaccine containing the 450 amino acids at the N-terminus of the ZIKV envelope protein (E90) in mouse models for either in utero or neonatal ZIKV infection...
August 10, 2018: Acta Neuropathologica Communications
https://www.readbyqxmd.com/read/30097055/prp-res-deposition-in-the-retina-is-a-common-finding-of-sporadic-familial-and-iatrogenic-creutzfeldt-jakob-diseases-cjd
#20
LETTER
Masaki Takao, Hiroaki Kimura, Tetsuyuki Kitamoto, Ban Mihara
No abstract text is available yet for this article.
August 10, 2018: Acta Neuropathologica Communications
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