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https://www.readbyqxmd.com/read/29772786/tau-fibril-formation-in-cultured-cells-compatible-with-a-mouse-model-of-tauopathy
#1
Gen Matsumoto, Kazuki Matsumoto, Taeko Kimura, Tetsuya Suhara, Makoto Higuchi, Naruhiko Sahara, Nozomu Mori
Neurofibrillary tangles composed of hyperphosphorylated tau protein are primarily neuropathological features of a number of neurodegenerative diseases collectively termed tauopathy. To understand the mechanisms underlying the cause of tauopathy, precise cellular and animal models are required. Recent data suggest that the transient introduction of exogenous tau can accelerate the development of tauopathy in the brains of non-transgenic and transgenic mice expressing wild-type human tau. However, the transmission mechanism leading to tauopathy is not fully understood...
May 17, 2018: International Journal of Molecular Sciences
https://www.readbyqxmd.com/read/29769333/microglia-are-critical-in-host-defense-against-prion-disease
#2
James A Carroll, Brent Race, Katie Williams, James Striebel, Bruce Chesebro
Microglial cells in the central nervous system play important roles in neurodevelopment and resistance to infection, yet microglia can become neurotoxic under some conditions. An early event during prion infection is the activation of microglia and astrocytes in the brain prior to damage or death of neurons. Previous prion disease studies using two different strategies to manipulate signaling through the microglial receptor CSF-1R reported contrary effects on survival from prion disease. However, in these studies, reduction of microglial numbers and function were variable, thus confounding interpretation of the results...
May 16, 2018: Journal of Virology
https://www.readbyqxmd.com/read/29769283/-hermes-transposon-mutagenesis-shows-ure3-prion-pathology-prevented-by-a-ubiquitin-targeting-protein-evidence-for-carbon-nitrogen-assimilation-cross-talk-and-a-second-function-for-ure2p-in-saccharomyces-cerevisiae
#3
Herman K Edskes, Maryam Mukhamedova, Bouke K Edskes, Reed B Wickner
[URE3] is an amyloid-based prion of Ure2p, a regulator of nitrogen catabolism. While most variants of the [URE3] prion are toxic, mild variants that only slightly slow growth are more widely studied. The existence of several anti-prion systems suggests that some components may be protecting cells from potential detrimental effects of mild [URE3] variants. Our extensive Hermes transposon mutagenesis showed that disruption of YLR352W dramatically slows growth of [URE3-1] strains. Ylr352wp is an F-box protein, directing selection of substrates for ubiquitination by a cullin-containing E-3 ligase...
May 16, 2018: Genetics
https://www.readbyqxmd.com/read/29768194/extracellular-forms-of-a%C3%AE-and-tau-from-ipsc-models-of-alzheimer-s-disease-disrupt-synaptic-plasticity
#4
Neng-Wei Hu, Grant T Corbett, Steven Moore, Igor Klyubin, Tiernan T O'Malley, Dominic M Walsh, Frederick J Livesey, Michael J Rowan
The early stages of Alzheimer's disease are associated with synaptic dysfunction prior to overt loss of neurons. To identify extracellular molecules that impair synaptic plasticity in the brain, we studied the secretomes of human iPSC-derived neuronal models of Alzheimer's disease. When introduced into the rat brain, secretomes from human neurons with either a presenilin-1 mutation, amyloid precursor protein duplication, or trisomy of chromosome 21 all strongly inhibit hippocampal long-term potentiation. Synaptic dysfunction caused by presenilin-1 mutant and amyloid precusor protein duplication secretomes is mediated by Aβ peptides, whereas trisomy of chromosome 21 (trisomy 21) neuronal secretomes induce dysfunction through extracellular tau...
May 15, 2018: Cell Reports
https://www.readbyqxmd.com/read/29761205/cellular-mechanisms-responsible-for-cell-to-cell-spreading-of-prions
#5
REVIEW
Didier Vilette, Josquin Courte, Jean Michel Peyrin, Laurent Coudert, Laurent Schaeffer, Olivier Andréoletti, Pascal Leblanc
Prions are infectious agents that cause fatal neurodegenerative diseases. Current evidence indicates that they are essentially composed of an abnormally folded protein (PrPSc ). These abnormal aggregated PrPSc species multiply in infected cells by recruiting and converting the host PrPC protein into new PrPSc . How prions move from cell to cell and progressively spread across the infected tissue is of crucial importance and may provide experimental opportunity to delay the progression of the disease. In infected cells, different mechanisms have been identified, including release of infectious extracellular vesicles and intercellular transfer of PrPSc -containing organelles through tunneling nanotubes...
May 14, 2018: Cellular and Molecular Life Sciences: CMLS
https://www.readbyqxmd.com/read/29760562/tau-a-common-denominator-and-therapeutic-target-for-neurodegenerative-disorders
#6
Alexander Maxan, Francesca Cicchetti
There is compelling evidence that a number of neurodegenerative diseases share common pathogenic mechanisms. Better understanding these mechanisms will allow us to develop new therapeutic strategies. This commentary follows up on our recent findings that tau pathology can be found in healthy fetal tissue transplanted into the brain of patients with either Huntington or Parkinson disease. We will examine how tau appears to be shared in a number of different conditions and how its expression relates to cognitive decline and disease progression...
2018: Journal of Experimental Neuroscience
https://www.readbyqxmd.com/read/29760292/-targeting-transcellular-transport-of-%C3%AE-synuclein-for-developing-disease-modifying-therapies-for-synucleinopathy
#7
Takafumi Hasegawa
Parkinson's disease (PD) is the second most common neurodegenerative disorder and it is characterized by progressive physical disability along with a variety of non-motor symptoms. Drugs that replenish dopamine can partly alleviate the motor symptoms; however, they do not cure the disease itself. Therefore, there is an urgent need for disease modifying therapies that would delay or prevent neurodegeneration. Increasing evidence suggests that α-synuclein, a key molecule in PD, is secreted into the extracellular environment and can be transported from cell-to-cell, thereby affecting the physiological state of the neighboring cells in a prion-like manner...
May 2018: Brain and Nerve, Shinkei Kenkyū No Shinpo
https://www.readbyqxmd.com/read/29757239/preparation-physicochemical-and-antioxidant-properties-of-acid-and-pepsin-soluble-collagens-from-the-swim-bladders-of-miiuy-croaker-miichthys-miiuy
#8
Wen-Hao Zhao, Chang-Feng Chi, Yu-Qin Zhao, Bin Wang
Collagen is one of the most useful biomaterials and widely applied in functional food and cosmetics. However, some consumers have paid close attention to the safety of mammalian collagens because of the outbreaks of bovine spongiform encephalopathy (BSE), foot-and-mouth disease (FMD), and other prion diseases. Therefore, there is a strong demand for developing alternative sources of collagen, with one promising source being from the process by-products of commercial fisheries. In this report, acid-soluble collagen (ASC-SB) and pepsin-soluble collagen (PSC-SB) from swim bladders of miiuy croaker ( Miichthys miiuy ) were isolated with yields of 1...
May 12, 2018: Marine Drugs
https://www.readbyqxmd.com/read/29755516/synaptic-paths-to-neurodegeneration-the-emerging-role-of-tdp-43-and-fus-in-synaptic-functions
#9
REVIEW
Shuo-Chien Ling
TAR DNA-binding protein-43 KDa (TDP-43) and fused in sarcoma (FUS) as the defining pathological hallmarks for amyotrophic lateral sclerosis (ALS) and frontotemporal dementia (FTD), coupled with ALS-FTD-causing mutations in both genes, indicate that their dysfunctions damage the motor system and cognition. On the molecular level, TDP-43 and FUS participate in the biogenesis and metabolism of coding and noncoding RNAs as well as in the transport and translation of mRNAs as part of cytoplasmic mRNA-ribonucleoprotein (mRNP) granules...
2018: Neural Plasticity
https://www.readbyqxmd.com/read/29755395/early-onset-creutzfeldt-jakob-disease-mimicking-immune-mediated-encephalitis
#10
Wietse A Wiels, Stephanie Du Four, Laura Seynaeve, Anja Flamez, Thomas Tousseyn, Dietmar Thal, Miguel D'Haeseleer
Objectives: The objective of this study is to explore the clinical, radiological, and pathological manifestations of a rare subtype of prion disease and their implication for differential diagnosis in case of an early onset neuropsychiatric deterioration. Methods: We discuss a patients' clinical history, as well as the string of investigations and symptomatological evolution that finally led to a pathological diagnosis. Results: Our patient had the extremely rare VV1 type sporadic Creutzfeldt-Jakob disease (sCJD)...
2018: Frontiers in Neurology
https://www.readbyqxmd.com/read/29754043/prion-protein-testis-specific-prnt-gene-polymorphisms-and-transcript-level-in-ovine-spermatozoa-implications-in-freezability-fertilization-and-embryo-production
#11
R M Pereira, P Mesquita, V M R Pires, M C Baptista, J P Barbas, J Pimenta, A E M Horta, J A M Prates, C C Marques
An essential role of prion protein testis specific (PRNT) and prion protein 2 dublet (PRND) genes in the male reproductive function has been highlighted, although a deeper knowledge for the mechanisms involved is still lacking. Our goal was to determine the importance of the PRNT haplotypic variants and mRNA expression levels in ovine spermatozoa freezability and ability for fertilization and embryo developmental processes. Their association with the PRND gene polymorphisms was also analyzed. DNA from rams belonging to three Portuguese sheep breeds (n = 28) was screened by single-strand conformation polymorphism (SSCP) analysis to identify the PRNT and PRND polymorphisms...
April 11, 2018: Theriogenology
https://www.readbyqxmd.com/read/29752551/tau-seeding-activity-begins-in-the-transentorhinal-entorhinal-regions-and-anticipates-phospho-tau-pathology-in-alzheimer-s-disease-and-part
#12
Sarah K Kaufman, Kelly Del Tredici, Talitha L Thomas, Heiko Braak, Marc I Diamond
Alzheimer's disease (AD) is characterized by accumulation of tau neurofibrillary tangles (NFTs) and, according to the prion model, transcellular propagation of pathological "seeds" may underlie its progression. Staging of NFT pathology with phospho-tau antibody is useful to classify AD and primary age-related tauopathy (PART) cases. The locus coeruleus (LC) shows the earliest phospho-tau signal, whereas other studies suggest that pathology begins in the transentorhinal/entorhinal cortices (TRE/EC)...
May 11, 2018: Acta Neuropathologica
https://www.readbyqxmd.com/read/29751084/improved-high-yield-expression-purification-and-refolding-of-recombinant-mammalian-prion-proteins-under-aerosol-free-elevated-biological-safety-conditions
#13
Peter Rehbein, Harald Schwalbe
Production of recombinant prion proteins is of crucial relevance in food technology (analytical standards, assay development) but also in basic research, most importantly structural biology (NMR, X-ray diffraction). Structural approaches conveniently allow for sophisticated investigation of prion disease pathogenesis, but usually require large amounts of sample material. Recently, working with recombinant prion proteins has been recategorized to biosafety levels > S1 as infectious prions may readily be generated de novo and become airborne via aerosols...
May 8, 2018: Protein Expression and Purification
https://www.readbyqxmd.com/read/29751083/the-amyloidogenicity-of-a-c-terminal-region-of-tdp-43-implicated-in-amyotrophic-lateral-sclerosis-can-be-affected-by-anions-acetylation-and-homodimerization
#14
Archana Prasad, Vishwanath Sivalingam, Vidhya Bharathi, Amandeep Girdhar, Basant K Patel
Amyotrophic Lateral Sclerosis (ALS) is a fatal neurodegenerative disease associated with accumulation of hyper-phosphorylated, and ubiquitinated TAR DNA-binding protein-43 (TDP-43) as inclusion deposits in neuronal cells. Recently, amyloid-like fibrillar aggregates of TDP-43 have been reported from several ALS patients. The C-terminal region of TDP-43 is central to TDP-43's pathological aggregation and most of the familial ALS mutations in the encoding TARDBP gene are located in this domain. Also, aberrant proteolytic cleavages of TDP-43 produce cytotoxic C-terminal fragments of ∼15-35 kDa...
May 8, 2018: Biochimie
https://www.readbyqxmd.com/read/29748616/prion-protein-is-essential-for-the-re1-silencing-transcription-factor-rest-dependent-developmental-switch-in-synaptic-nmda-receptors
#15
Zhiqi Song, Wei Yang, Guangyu Cheng, Xiangmei Zhou, Lifeng Yang, Deming Zhao
It is important that the correct amounts of GluN2 subunits are maintained, as they determine NMDAR functional properties, which are crucial to neuronal communication, synaptogenesis and cognitive function. The transcriptional repressor RE1 silencing transcription factor (REST) is critical for the postnatal developmental switch in NMDARs. However, the mechanisms triggering REST and the link between NMDARs and REST are unclear. Here we show a new physiological essential role for cellular prion protein (PrPC ) in REST-dependent homeostasis and the developmental switch of NMDARs...
May 10, 2018: Cell Death & Disease
https://www.readbyqxmd.com/read/29744847/discriminating-strains-of-self-propagating-protein-aggregates-using-a-conformational-stability-assay
#16
Heather H C Lau, Angus Lau, Joel C Watts
Prions and other self-propagating protein aggregates can exist as distinct strains, which are thought to represent different conformations of aggregates. There is growing evidence that protein aggregate strains may be important for understanding the biology of common neurodegenerative disorders, such as Alzheimer's disease and Parkinson's disease. While methodology for discriminating prion strains is in widespread use, there is a paucity of tools for comparing the conformational properties of aggregates composed of β-amyloid (Aβ) peptide or α-synuclein protein, particularly when present in complex samples such as brain extracts...
2018: Methods in Molecular Biology
https://www.readbyqxmd.com/read/29744836/synthesis-of-mikto-arm-star-peptide-conjugates
#17
Jin Mo Koo, Hao Su, Yi-An Lin, Honggang Cui
Mikto-arm star peptide conjugates are an emerging class of self-assembling peptide-based structural units that contain three or more auxiliary segments of different chemical compositions and/or functionalities. This group of molecules exhibit interesting self-assembly behavior in solution due to their chemically asymmetric topology. Here we describe the detailed procedure for synthesis of an ABC Mikto-arm star peptide conjugate in which two immiscible entities (a saturated hydrocarbon and a hydrophobic and lipophobic fluorocarbon) are conjugated onto a short β-sheet forming peptide sequence, GNNQQNY, derived from the Sup35 prion, through a lysine junction...
2018: Methods in Molecular Biology
https://www.readbyqxmd.com/read/29740275/synaptic-tau-seeding-precedes-tau-pathology-in-human-alzheimer-s-disease-brain
#18
Sarah L DeVos, Bianca T Corjuc, Derek H Oakley, Chloe K Nobuhara, Riley N Bannon, Alison Chase, Caitlin Commins, Jose A Gonzalez, Patrick M Dooley, Matthew P Frosch, Bradley T Hyman
Alzheimer's disease (AD) is defined by the presence of intraneuronal neurofibrillary tangles (NFTs) composed of hyperphosphorylated tau aggregates as well as extracellular amyloid-beta plaques. The presence and spread of tau pathology through the brain is classified by Braak stages and thought to correlate with the progression of AD. Several in vitro and in vivo studies have examined the ability of tau pathology to move from one neuron to the next, suggesting a "prion-like" spread of tau aggregates may be an underlying cause of Braak tau staging in AD...
2018: Frontiers in Neuroscience
https://www.readbyqxmd.com/read/29738705/phosphorylation-leads-the-way-for-protein-aggregate-disassembly
#19
Steven Boeynaems, Aaron D Gitler
Protein aggregation can be beneficial, with important biological functions, but must be somehow controlled. In this issue of Developmental Cell, Carpenter et al. (2018) uncover how a solid-like supermolecular protein assembly that regulates yeast meiosis is disassembled through phosphorylation of a disordered prion-like domain to control the timing of meiotic progression.
May 7, 2018: Developmental Cell
https://www.readbyqxmd.com/read/29735445/-effect-of-intrahippocampal-injection-of-anti-cellular-prion-protein-monoclonal-antibody-on-cognitive-deficits-in-appswe-psen1-de9-transgenic-mice
#20
Hai-Ying Zhang, Yi-Heng Liu, Yuan Fu, Peng-Cheng Chen, Rui Lu, Jian-Xing Li, Ming-Hui Chen, Hao-Chi Yang, Yu-Sheng Zhang
OBJECTIVE: To study the effects of intrahippocampal injection of cellular prion protein (PrPC ) antibody on cognitive deficits of APPswe/PSEN1dE9 transgenic mice. METHODS: Eight-month-old male APPswe/PSEN1dE9 transgenic mice were subjected to bilateral intrahippocampal injection of a single dose (2 µL) of anti-PrPC monoclonal antibody (EP1802Y) or PBS, with wild-type C57Bl/6J mice serving as the control group. After two months, the mice were tested for cognitive behaviors using open filed (OF) test, Morris water maze (MWM) test, fear conditioning (FC) test, and novel object recognition (NOR) test, and immunohistochemistry was used to examine the changes in hippocampal expression of Aβ1-42 ...
April 20, 2018: Nan Fang Yi Ke da Xue Xue Bao, Journal of Southern Medical University
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