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https://www.readbyqxmd.com/read/28934337/pyrene-conjugation-and-spectroscopic-analysis-of-hydroxypropyl-methylcellulose-compounds-successfully-demonstrated-a-local-dielectric-difference-associated-with-in-vivo-anti-prion-activity
#1
Kenta Teruya, Ayumi Oguma, Keiko Nishizawa, Hiroshi Kamitakahara, Katsumi Doh-Ura
Our previous study on prion-infected rodents revealed that hydroxypropyl methylcellulose compounds (HPMCs) with different molecular weights but similar composition and degree of substitution have different levels of long-lasting anti-prion activity. In this study, we searched these HPMCs for a parameter specifically associated with in vivo anti-prion activity by analyzing in vitro chemical properties and in vivo tissue distributions. Infrared spectroscopic and thermal analyses revealed no differences among HPMCs, whereas pyrene conjugation and spectroscopic analysis revealed that the fluorescence intensity ratio of peak III/peak I correlated with anti-prion activity...
2017: PloS One
https://www.readbyqxmd.com/read/28934062/manipulating-the-aggregation-activity-of-human-prion-like-proteins
#2
Sean M Cascarina, Kacy R Paul, Eric D Ross
Considerable advances in understanding the protein features favoring prion formation in yeast have facilitated the development of effective yeast prion prediction algorithms. Here we discuss a recent study in which we systematically explored the utility of the yeast prion prediction algorithm PAPA for designing mutations to modulate the aggregation activity of the human prion-like protein hnRNPA2B1. Mutations in hnRNPA2B1 cause multisystem proteinopathy in humans, and accelerate aggregation of the protein in vitro...
September 21, 2017: Prion
https://www.readbyqxmd.com/read/28932898/differential-effects-of-chaperones-on-yeast-prions-current-view
#3
REVIEW
Andrew G Matveenko, Yury A Barbitoff, Lina Manuela Jay-Garcia, Yury O Chernoff, Galina A Zhouravleva
Endogenous yeast amyloids that control heritable traits and are frequently used as models for human amyloid diseases are termed yeast prions. Yeast prions, including the best studied ones ([PSI (+)] and [URE3]), propagate via intimate interactions with molecular chaperones. Different yeast prions exhibit differential responses to changes in levels, functionality or localization of the components of chaperone machinery. Here, we provide additional data confirming differential effects of chaperones (and specifically, Hsp40s) on yeast prions and summarize current knowledge of the mechanisms underlying chaperone specificities...
September 20, 2017: Current Genetics
https://www.readbyqxmd.com/read/28931606/asparagine-and-glutamine-ladders-promote-cross-species-prion-conversion
#4
Timothy D Kurt, Patricia Aguilar-Calvo, Lin Jiang, José A Rodriguez, Nazilla Alderson, David S Eisenberg, Christina J Sigurdson
Prion transmission between species is governed in part by primary sequence similarity between the infectious prion aggregate, PrP(Sc), and the cellular prion protein of the host, PrP(C) A puzzling feature of prion formation is that certain PrP(C) sequences, such as that of bank vole, can be converted by a remarkably broad array of different mammalian prions, whereas others, such as rabbit, show robust resistance to cross-species prion conversion. To examine the structural determinants that confer susceptibility or resistance to prion conversion, we systematically tested over 40 PrP(C) variants of susceptible and resistant PrP(C) sequences in a prion conversion assay...
September 20, 2017: Journal of Biological Chemistry
https://www.readbyqxmd.com/read/28928456/enabling-stop-codon-read-through-translation-in-bacteria-as-a-probe-for-amyloid-aggregation
#5
Laura Molina-García, Rafael Giraldo
Amyloid aggregation of the eukaryotic translation terminator eRF3/Sup35p, the [PSI (+)] prion, empowers yeast ribosomes to read-through UGA stop codons. No similar functional prion, skipping a stop codon, has been found in Escherichia coli, a fact possibly due to the efficient back-up systems found in bacteria to rescue non-stop complexes. Here we report that engineering hydrophobic amyloidogenic repeats from a synthetic bacterial prion-like protein (RepA-WH1) into the E. coli releasing factor RF1 promotes its aggregation and enables ribosomes to continue with translation through a premature UAG stop codon located in a β-galactosidase reporter...
September 19, 2017: Scientific Reports
https://www.readbyqxmd.com/read/28927447/protecting-effect-of-prp-codons-m142-and-k222-in-goats-orally-challenged-with-bovine-spongiform-encephalopathy-prions
#6
C Fast, W Goldmann, P Berthon, K Tauscher, O Andréoletti, I Lantier, C Rossignol, A Bossers, J G Jacobs, N Hunter, M H Groschup, F Lantier, J P M Langeveld
Breeding towards genetic resistance to prion disease is effective in eliminating scrapie. In sheep, classical forms of scrapie have been eradicated almost completely in several countries by breeding programs using a prion protein (PrP) gene (PRNP) amino acid polymorphism. For goats, field and experimental studies have provided evidence for several amino acid polymorphisms that are associated with resistance to scrapie, but only limited data are available concerning the susceptibility of caprine PRNP genotypes to BSE...
September 19, 2017: Veterinary Research
https://www.readbyqxmd.com/read/28924012/inhibition-of-il-1%C3%AE-signaling-normalizes-nmda-dependent-neurotransmission-and-reduces-seizure-susceptibility-in-a-mouse-model-of-creutzfeldt-jakob-disease
#7
Ilaria Bertani, Valentina Iori, Massimo Trusel, Mattia Maroso, Claudia Foray, Susanna Mantovani, Raffaella Tonini, Annamaria Vezzani, Roberto Chiesa
Creutzfeldt-Jakob disease (CJD) is a neurodegenerative disorder caused by prion protein (PrP) misfolding, clinically recognized by cognitive and motor deficits, electroencephalographic (EEG) abnormalities and seizures. Its neurophysiological bases are not known. To assess the potential involvement of N-methyl-D-aspartate receptor (NMDAR) dysfunction, we analyzed NMDA-dependent synaptic plasticity in hippocampal slices from Tg(CJD) mice, which model a genetic form of CJD. Because PrP depletion may result in functional upregulation of NMDARs, we also analyzed PrP knockout (KO) mice...
September 18, 2017: Journal of Neuroscience: the Official Journal of the Society for Neuroscience
https://www.readbyqxmd.com/read/28923943/-psi-prion-propagation-is-controlled-by-inositol-polyphosphates
#8
Reed B Wickner, Amy C Kelly, Evgeny E Bezsonov, Herman K Edskes
The yeast prions [PSI+] and [URE3] are folded in-register parallel β-sheet amyloids of Sup35p and Ure2p, respectively. In a screen for antiprion systems curing [PSI+] without protein overproduction, we detected Siw14p as an antiprion element. An array of genetic tests confirmed that many variants of [PSI+] arising in the absence of Siw14p are cured by restoring normal levels of the protein. Siw14p is a pyrophosphatase specifically cleaving the β phosphate from 5-diphosphoinositol pentakisphosphate (5PP-IP5), suggesting that increased levels of this or some other inositol polyphosphate favors [PSI+] propagation...
September 18, 2017: Proceedings of the National Academy of Sciences of the United States of America
https://www.readbyqxmd.com/read/28922846/different-complicated-brain-pathologies-in-monozygotic-twins-with-gerstmann-str%C3%A3-ussler-scheinker-disease
#9
Hiroyuki Honda, Kensuke Sasaki, Hiroshi Takashima, Daisuke Mori, Sachiko Koyama, Satoshi O Suzuki, Toru Iwaki
Gerstmann-Sträussler-Scheinker disease (GSS) is an autosomal, dominantly inherited prion disease. In this study, we present different complicated brain pathologies determined postmortem of monozygotic GSS twin sisters. Case 1 showed cerebellar ataxia at the age of 58 years, and died at 66 years. Case 2 became symptomatic at the age of 75 years, and died at 79 years. There was a 17-year difference in the age of onset between the twins. Postmortem examination revealed numerous prion protein (PrP) plaques in the brains of both cases...
October 1, 2017: Journal of Neuropathology and Experimental Neurology
https://www.readbyqxmd.com/read/28922400/protein-folding-misfolding-and-aggregation-the-importance-of-two-electron-stabilizing-interactions
#10
Andrzej Stanisław Cieplak
Proteins associated with neurodegenerative diseases are highly pleiomorphic and may adopt an all-α-helical fold in one environment, assemble into all-β-sheet or collapse into a coil in another, and rapidly polymerize in yet another one via divergent aggregation pathways that yield broad diversity of aggregates' morphology. A thorough understanding of this behaviour may be necessary to develop a treatment for Alzheimer's and related disorders. Unfortunately, our present comprehension of folding and misfolding is limited for want of a physicochemical theory of protein secondary and tertiary structure...
2017: PloS One
https://www.readbyqxmd.com/read/28920852/experimental-transmission-to-a-calf-of-an-isolate-of-spanish-classical-scrapie
#11
Rosa Bolea, Carlos Hedman, Óscar López-Pérez, Belén Marín, Enríc Vidal, Martí Pumarola, Fabien Corbière, Antonio Romero, Bernardino Moreno, Inmaculada Martín-Burriel, Olivier Andréoletti, Juan José Badiola
Multiple theories exist regarding the origin of bovine spongiform encephalopathy (BSE). An early and prominent theory proposed that BSE was the result of the adaptation of sheep scrapie to cattle. The reports to date indicate that the distribution of the pathological prion protein (PrPSc) in experimental bovine scrapie is largely restricted to the central nervous system (CNS). Here, we describe pathological findings in a calf intracerebrally inoculated with a Spanish classical scrapie isolate. While clinical disease was observed 30 months after inoculation and PrPSc was detected in the CNS, the corresponding phenotype differed from that of BSE...
September 18, 2017: Journal of General Virology
https://www.readbyqxmd.com/read/28912682/extracellular-vesicles-in-brain-tumors-and-neurodegenerative-diseases
#12
REVIEW
Federica Ciregia, Andrea Urbani, Giuseppe Palmisano
Extracellular vesicles (EVs) can be classified into apoptotic bodies, microvesicles (MVs), and exosomes, based on their origin or size. Exosomes are the smallest and best characterized vesicles which derived from the endosomal system. These vesicles are released from many different cell types including neuronal cells and their functions in the nervous system are investigated. They have been proposed as novel means for intercellular communication, which takes part not only to the normal neuronal physiology but also to the transmission of pathogenic proteins...
2017: Frontiers in Molecular Neuroscience
https://www.readbyqxmd.com/read/28910422/a-prolonged-chronological-lifespan-is-an-unexpected-benefit-of-the-psi-prion-in-yeast
#13
Kai Wang, Ronald Melki, Mehdi Kabani
Self-replicating 'proteinaceous infectious particles' or prions are responsible for complex heritable traits in the yeast Saccharomyces cerevisiae. Our current understanding of the biology of yeast prions stems from studies mostly done in the context of actively dividing cells in optimal laboratory growth conditions. Evidence suggest that fungal prions exist in the wild where most cells are in a non-dividing quiescent state, because of imperfect growth conditions, scarcity of nutrients and competition. We know little about the faithful transmission of yeast prions in such conditions and their physiological consequences throughout the lifespan of yeast cells...
2017: PloS One
https://www.readbyqxmd.com/read/28910420/role-of-the-central-lysine-cluster-and-scrapie-templating-in-the-transmissibility-of-synthetic-prion-protein-aggregates
#14
Bradley R Groveman, Gregory J Raymond, Katrina J Campbell, Brent Race, Lynne D Raymond, Andrew G Hughson, Christina D Orrú, Allison Kraus, Katie Phillips, Byron Caughey
Mammalian prion structures and replication mechanisms are poorly understood. Most synthetic recombinant prion protein (rPrP) amyloids prepared without cofactors are non-infectious or much less infectious than bona fide tissue-derived PrPSc. This effect has been associated with differences in folding of the aggregates, manifested in part by reduced solvent exclusion and protease-resistance in rPrP amyloids, especially within residues ~90-160. Substitution of 4 lysines within residues 101-110 of rPrP (central lysine cluster) with alanines (K4A) or asparagines (K4N) allows formation of aggregates with extended proteinase K (PK) resistant cores reminiscent of PrPSc, particularly when seeded with PrPSc...
September 14, 2017: PLoS Pathogens
https://www.readbyqxmd.com/read/28910367/a-novel-panel-of-%C3%AE-synuclein-antibodies-reveal-distinctive-staining-profiles-in-synucleinopathies
#15
Jess-Karan S Dhillon, Cara Riffe, Brenda D Moore, Yong Ran, Paramita Chakrabarty, Todd E Golde, Benoit I Giasson
Synucleinopathies are a spectrum of neurodegenerative diseases characterized by the intracellular deposition of the protein α-synuclein leading to multiple outcomes, including dementia and Parkinsonism. Recent findings support the notion that across the spectrum of synucleinopathies there exist diverse but specific biochemical modifications and/or structural conformations of α-synuclein, which would give rise to protein strain specific prion-like intercellular transmission, a proposed model that could explain synucleinopathies disease progression...
2017: PloS One
https://www.readbyqxmd.com/read/28906181/a-decade-of-using-small-to-medium-throughput-allele-discrimination-assay-to-determine-prion-protein-gene-prnp-genotypes-in-sheep-in-slovenia
#16
Jelka Zabavnik, Marko Cotman, Polona Juntes, Ivan Ambrozic
Sheep with valine (V) at codon 136 and glutamine (Q) at codon 171 of the prion protein gene ( Prnp) are highly susceptible to classical scrapie, whereas phenylalanine (F) at codon 141 and histidine (H) at codon 154 play a major role in the susceptibility to atypical scrapie. A TaqMan real-time PCR assay was developed to determine Prnp alleles at codons 136, 141, 154, and 171 and used in classical scrapie eradication and breeding programs adopted in Slovenia. The frequency of the most resistant genotypes ARR/ARR and ARR/ARQ increased significantly in tested animals ( n = 35,138) from 6...
September 1, 2017: Journal of Veterinary Diagnostic Investigation
https://www.readbyqxmd.com/read/28903310/characterization-of-physiochemical-properties-of-caveolin-1-from-normal-and-prion-infected-human-brains
#17
Xiangzhu Xiao, Pingping Shen, Zerui Wang, Johnny Dang, Alise Adornato, Lewis S Zou, Zhiqian Dong, Jue Yuan, Jiachun Feng, Li Cui, Wen-Quan Zou
Caveolin-1 is a major component protein of the caveolae-a type of flask shaped, 50-100 nm, nonclathrin-coated, microdomain present in the plasma membrane of most mammalian cells. Caveolin-1 functions as a scaffolding protein to organize and concentrate signaling molecules within the caveolae, which may be associated with its unique physicochemical properties including oligomerization, acquisition of detergent insolubility, and association with cholesterol. Here we demonstrate that caveolin-1 is detected in all brain areas examined and recovered in both detergent-soluble and -insoluble fractions...
August 15, 2017: Oncotarget
https://www.readbyqxmd.com/read/28903070/role-of-the-ampk-pathway-in-promoting-autophagic-flux-via-modulating-mitochondrial-dynamics-in-neurodegenerative-diseases-insight-into-prion-diseases
#18
REVIEW
Syed Zahid Ali Shah, Deming Zhao, Tariq Hussain, Lifeng Yang
Neurons are highly energy demanding cells dependent on the mitochondrial oxidative phosphorylation system. Mitochondria generate energy via respiratory complexes that constitute the electron transport chain. Adenosine triphosphate depletion or glucose starvation act as a trigger for the activation of adenosine monophosphate-activated protein kinase (AMPK). AMPK is an evolutionarily conserved protein that plays an important role in cell survival and organismal longevity through modulation of energy homeostasis and autophagy...
September 10, 2017: Ageing Research Reviews
https://www.readbyqxmd.com/read/28902452/mgrn1-mediated-ubiquitination-of-%C3%AE-tubulin-regulates-microtubule-dynamics-and-intracellular-transport
#19
Rukmini Mukherjee, Priyanka Majumder, Oishee Chakrabarti
MGRN1 mediated ubiquitination of α-tubulin regulates microtubule stability and mitotic spindle positioning in mitotic cells. This study elucidates the effect of MGRN1 mediated ubiquitination of α-tubulin in interphase cells. Here, we show that MGRN1 mediated ubiquitination regulates dynamics of EB1 labelled plus ends of microtubules. Intracellular transport of mitochondria and endosomes are affected in cultured cells where functional MGRN1 is depleted. Defects in microtubule-dependent organellar transport are evident in cells where noncanonical K6 mediated ubiquitination of α-tubulin by MGRN1 is compromised...
September 13, 2017: Traffic
https://www.readbyqxmd.com/read/28901450/role-of-hypoxia%C3%A2-mediated-cellular-prion-protein-functional-change-in-stem-cells-and-potential-application-in-angiogenesis-review
#20
Seung Pil Yun, Yong-Seok Han, Jun Hee Lee, Yeo Min Yoon, Chul Won Yun, Peter Rhee, Sang Hun Lee
Cellular prion protein (PrPC) can replace other pivotal molecules due to its interaction with several partners in performing a variety of important biological functions that may differ between embryonic and mature stem cells. Recent studies have revealed major advances in elucidating the putative role of PrPC in the regulation of stem cells and its application in stem cell therapy. What is special about PrPC is that its expression may be regulated by hypoxia‑inducible factor (HIF)‑1α, which is the transcriptional factor of cellular response to hypoxia...
August 29, 2017: Molecular Medicine Reports
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