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Prion

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https://www.readbyqxmd.com/read/28762865/chronic-wasting-disease-prion-infection-of-differentiated-neurospheres
#1
Yoshifumi Iwamaru, Candace K Mathiason, Glenn C Telling, Edward A Hoover
A possible strategy to develop more diverse cell culture systems permissive to infection with naturally occurring prions is to exploit culture of neurospheres from transgenic mice expressing the normal prion protein (PrP) of the native host species. Accordingly, we developed differentiated neurosphere cultures from the cervid PrP-expressing mice to investigate whether this in vitro system would support replication of non-adapted cervid-origin chronic wasting disease (CWD) prions. Here we report the successful amplification of disease-associated PrP in differentiated neurosphere cultures within 3 weeks after exposure to CWD prions from both white-tailed deer or elk...
July 4, 2017: Prion
https://www.readbyqxmd.com/read/28759332/the-cellular-prion-protein-prp-c-as-neuronal-receptor-for-%C3%AE-synuclein
#2
Laura Urrea, Isidro Ferrer, Rosalina Gavín, José Antonio Del Río
The term 'prion-like' is used to define some misfolded protein species that propagate intercellularly, triggering protein aggregation in recipient cells. For cell binding, both direct plasma membrane interaction and membrane receptors have been described for particular amyloids. In this respect, emerging evidence demonstrates that several β-sheet enriched proteins can bind to the cellular prion protein (PrP(C)). Among other interactions, the physiological relevance of the binding between β-amyloid and PrP(C) has been a relevant focus of numerous studies...
July 4, 2017: Prion
https://www.readbyqxmd.com/read/28759310/ultrastructure-and-pathology-of-prion-protein-amyloid-accumulation-and-cellular-damage-in-extraneural-tissues-of-scrapie-infected-transgenic-mice-expressing-anchorless-prion-protein
#3
Brent Race, Martin Jeffrey, Gillian McGovern, David Dorward, Bruce Chesebro
In most human and animal prion diseases the abnormal disease-associated prion protein (PrPSc) is deposited as non-amyloid aggregates in CNS, spleen and lymphoid organs. In contrast, in humans and transgenic mice with PrP mutations which cause expression of PrP lacking a glycosylphosphatidylinositol (GPI)-anchor, most PrPSc is in the amyloid form. In transgenic mice expressing only anchorless PrP (tg anchorless), PrPSc is deposited not only in CNS and lymphoid tissues, but also in extraneural tissues including heart, brown fat, white fat, and colon...
July 4, 2017: Prion
https://www.readbyqxmd.com/read/28749249/an-autopsy-verified-case-of-steroid-responsive-encephalopathy-with-convulsion-and-a-false-positive-result-from-the-real-time-quaking-induced-conversion-assay
#4
Yuichi Hayashi, Yasushi Iwasaki, Nobuaki Yoshikura, Takahiko Asano, Maya Mimuro, Akio Kimura, Katsuya Satoh, Tetsuyuki Kitamoto, Mari Yoshida, Takashi Inuzuka
We report an autopsy-verified case of steroid-responsive encephalopathy with convulsion and a false-positive result from the real-time quaking-induced conversion (RT-QUIC) assay. A 61-year-old Japanese man presented with acute onset of consciousness disturbance, and convulsions, but without a past medical or family history of progressive dementia, epilepsy, or prion disease. Brain diffusion and fluid-attenuated inverted recovery MR images revealed edematous cortical hyper-intensity, which diminished after the acute phase...
July 4, 2017: Prion
https://www.readbyqxmd.com/read/28665753/the-effects-of-glutamine-asparagine-content-on-aggregation-and-heterologous-prion-induction-by-yeast-prion-like-domains
#5
Jenifer E Shattuck, Aubrey C Waechter, Eric D Ross
Prion-like domains are low complexity, intrinsically disordered domains that compositionally resemble yeast prion domains. Many prion-like domains are involved in the formation of either functional or pathogenic protein aggregates. These aggregates range from highly dynamic liquid droplets to highly ordered detergent-insoluble amyloid-like aggregates. To better understand the amino acid sequence features that promote conversion to stable, detergent-insoluble aggregates, we used the prediction algorithm PAPA to identify predicted aggregation-prone prion-like domains with a range of compositions...
July 4, 2017: Prion
https://www.readbyqxmd.com/read/28665745/ovine-recombinant-prp-as-an-inhibitor-of-ruminant-prion-propagation-in-vitro
#6
Rob G Workman, Ben C Maddison, Kevin C Gough
Prion diseases are fatal and incurable neurodegenerative diseases of humans and animals. Despite years of research, no therapeutic agents have been developed that can effectively manage or reverse disease progression. Recently it has been identified that recombinant prion proteins (rPrP) expressed in bacteria can act as inhibitors of prion replication within the in vitro prion replication system protein misfolding cyclic amplification (PMCA). Here, within PMCA reactions amplifying a range of ruminant prions including distinct Prnp genotypes/host species and distinct prion strains, recombinant ovine VRQ PrP displayed consistent inhibition of prion replication and produced IC50 values of 122 and 171 nM for ovine scrapie and bovine BSE replication, respectively...
July 4, 2017: Prion
https://www.readbyqxmd.com/read/28657440/a%C3%AE-seeds-and-prions-how-close-the-fit
#7
Jay Rasmussen, Mathias Jucker, Lary C Walker
The prion paradigm is increasingly invoked to explain the molecular pathogenesis of neurodegenerative diseases involving the misfolding and aggregation of proteins other than the prion protein (PrP). Extensive evidence from in vitro and in vivo studies indicates that misfolded and aggregated Aβ peptide, which is the probable molecular trigger for Alzheimer's disease, manifests all of the key characteristics of canonical mammalian prions. These features include a β-sheet rich architecture, tendency to polymerize into amyloid, templated corruption of like protein molecules, ability to form structurally and functionally variant strains, systematic spread by neuronal transport, and resistance to inactivation by heat and formaldehyde...
July 4, 2017: Prion
https://www.readbyqxmd.com/read/28574745/prion-specific-hsp40-function-the-role-of-the-auxilin-homolog-swa2
#8
Emily E Oliver, Elizabeth M Troisi, Justin K Hines
Yeast prions are protein-based genetic elements that propagate through cell populations via cytosolic transfer from mother to daughter cell. Molecular chaperone proteins including Hsp70, the Hsp40/J-protein Sis1, and Hsp104 are required for continued prion propagation, however the specific requirements of chaperone proteins differ for various prions. We recently reported that Swa2, the yeast homolog of the mammalian protein auxilin, is specifically required for the propagation of the prion [URE3]. (1) [URE3] propagation requires both a functional J-domain and the tetratricopeptide repeat (TPR) domain of Swa2, but does not require Swa2 clathrin binding...
May 4, 2017: Prion
https://www.readbyqxmd.com/read/28574744/spatial-regulation-of-coalesced-protein-assemblies-lessons-from-yeast-to-diseases
#9
Juha Saarikangas, Fabrice Caudron
Organisms rely on correctly folded proteins to carry out essential functions. Protein quality control factors guard proteostasis and prevent protein misfolding. When quality control fails and in response to diverse stresses, many proteins start to accumulate at specific deposit sites that maintain cellular organization and protect the functionality of coalescing proteins. These transitions involve dedicated proteins that promote coalescence and are facilitated by endo-membranes and cytoskeletal platforms...
May 4, 2017: Prion
https://www.readbyqxmd.com/read/28521568/prion-based-memory-of-heat-stress-in-yeast
#10
Tatiana A Chernova, Yury O Chernoff, Keith D Wilkinson
Amyloids and amyloid-based prions are self-perpetuating protein aggregates which can spread by converting a normal protein of the same sequence into a prion form. They are associated with diseases in humans and mammals, and control heritable traits in yeast and other fungi. Some amyloids are implicated in biologically beneficial processes. As prion formation generates reproducible memory of a conformational change, prions can be considered as molecular memory devices.  We have demonstrated that in yeast, stress-inducible cytoskeleton-associated protein Lsb2 forms a metastable prion in response to high temperature...
May 4, 2017: Prion
https://www.readbyqxmd.com/read/28509623/gastrostomy-in-patients-with-prion-disease
#11
Yasushi Iwasaki, Keiko Mori, Masumi Ito, Yoshinari Kawai, Ken-Ichiro Hoshino, Yuko Kawabata, Maya Mimuro, Mari Yoshida
Patients with prion diseases can live for long periods of time in a state of akinetic mutism given appropriate management of their symptoms. To study symptom support in these cases, we performed gastrostomies on 3 patients with V180I genetic Creutzfeldt-Jakob disease (CJD) who had become akinetic and mute, and compared them to 14 other similar patients being fed by tube. In the 3 gastrostomy cases, there were no direct complications due to the gastrostomy or tube feeding, nor were there episodes of discontinuation of tube feeding or initiation of continuous drip infusion due to severe complications...
May 4, 2017: Prion
https://www.readbyqxmd.com/read/28509609/amyloid-and-fdg-pet-in-sporadic-creutzfeldt-jakob-disease-correlation-with-pathological-prion-protein-in-neuropathology
#12
Jordi A Matías-Guiu, Carmen Guerrero-Márquez, María Nieves Cabrera-Martín, Ulises Gómez-Pinedo, María Romeral, Diego Mayo, Jesús Porta-Etessam, Teresa Moreno-Ramos, José Luis Carreras, Jorge Matías-Guiu
INTRODUCTION: The role of positron emission tomography (PET) in Creutzfeldt-Jakob disease is less defined than in other neurodegenerative diseases. We studied the correlation between the uptake of (18)F-florbetaben and (18)F-fluorodeoxyglucose with pathological prion protein deposition in histopathology in a case. METHODS: A patient with 80 y old with a rapid neurological deterioration with a confirmed diagnosis of CJD was studied. PET and MRI studies were performed between 13-20 d before the death...
May 4, 2017: Prion
https://www.readbyqxmd.com/read/28486039/flow-cytometric-measurement-of-the-cellular-propagation-of-tdp-43-aggregation
#13
Rafaa Zeineddine, Daniel R Whiten, Natalie E Farrawell, Luke McAlary, Maya A Hanspal, Janet R Kumita, Mark R Wilson, Justin J Yerbury
Amyotrophic lateral sclerosis is a devastating neuromuscular degenerative disease characterized by a focal onset of motor neuron loss, followed by contiguous outward spreading of pathology including TAR DNA-binding protein of 43 kDa (TDP-43) aggregates. Previous work suggests that TDP-43 can move between cells. Here we used a novel flow cytometry technique (FloIT) to analyze TDP-43 inclusions and propagation. When cells were transfected to express either mutant G294A TDP-43 fused to GFP or wild type TDP-43fused to tomato red and then co-cultured, flow cytometry detected intact cells containing both fusion proteins and using FloIT detected an increase in the numbers of inclusions in lysates from cells expressing wild type TDP-43-tomato...
May 4, 2017: Prion
https://www.readbyqxmd.com/read/28402718/prions-on-the-run-how-extracellular-vesicles-serve-as-delivery-vehicles-for-self-templating-protein-aggregates
#14
REVIEW
Shu Liu, André Hossinger, Sarah Göbbels, Ina M Vorberg
Extracellular vesicles (EVs) are actively secreted, membrane-bound communication vehicles that exchange biomolecules between cells. EVs also serve as dissemination vehicles for pathogens, including prions, proteinaceous infectious agents that cause transmissible spongiform encephalopathies (TSEs) in mammals. Increasing evidence accumulates that diverse protein aggregates associated with common neurodegenerative diseases are packaged into EVs as well. Vesicle-mediated intercellular transmission of protein aggregates can induce aggregation of homotypic proteins in acceptor cells and might thereby contribute to disease progression...
March 4, 2017: Prion
https://www.readbyqxmd.com/read/28362554/detection-of-a-new-20-bp-insertion-deletion-indel-within-sheep-prnd-gene-using-mathematical-expectation-me-method
#15
Jie Li, Xichun Zhu, Lin Ma, Hongwei Xu, Xin Cao, Renyun Luo, Hong Chen, Xiuzhu Sun, Yong Cai, Xianyong Lan
Prion-related protein doppel gene (PRND), as an essential member of the mammalian prion gene family, is associated with the scrapie susceptibility as well as phenotype traits, so the genetic variation of the PRND has been highly concerned recently, including the single nucleiotide polymorphism (SNP) and insertion/deletion (indel). Therefore, the objective of present study was to examine the possible indel variants by mathematical expectation (ME) detection method as well as explore its associations with phenotype traits...
March 4, 2017: Prion
https://www.readbyqxmd.com/read/28362551/the-double-life-of-the-ribosome-when-its-protein-folding-activity-supports-prion-propagation
#16
REVIEW
Cécile Voisset, Marc Blondel, Gary W Jones, Gaëlle Friocourt, Guillaume Stahl, Stéphane Chédin, Vincent Béringue, Reynald Gillet
It is no longer necessary to demonstrate that ribosome is the central machinery of protein synthesis. But it is less known that it is also key player of the protein folding process through another conserved function: the protein folding activity of the ribosome (PFAR). This ribozyme activity, discovered more than 2 decades ago, depends upon the domain V of the large rRNA within the large subunit of the ribosome. Surprisingly, we discovered that anti-prion compounds are also potent PFAR inhibitors, highlighting an unexpected link between PFAR and prion propagation...
March 4, 2017: Prion
https://www.readbyqxmd.com/read/28358272/evaluation-of-rapid-post-mortem-test-kits-for-bovine-spongiform-encephalopathy-bse-screening-in-japan-their-analytical-sensitivity-to-atypical-bse-prions
#17
Ken'ichi Hagiwara, Yoshifumi Iwamaru, Naoko Tabeta, Takashi Yokoyama, Minoru Tobiume
A classical type of bovine spongiform encephalopathy (C-BSE), recognized in 1987, had a large impact on public health due to its zoonotic link to variant Creutzfeldt-Jakob disease by the human consumption of dietary products contaminated with the C-BSE prion. Thus, a number of countries implemented BSE surveillance using rapid post-mortem test kits that were approved for detection of the C-BSE prion in the cattle brain. However, as atypical BSE (L- and H-BSE) cases emerged in subsequent years, the efficacy of the kits for the detection of atypical BSE prions became a matter of concern...
March 4, 2017: Prion
https://www.readbyqxmd.com/read/28350512/high-prevalence-of-prion-protein-genotype-associated-with-resistance-to-chronic-wasting-disease-in-one-alberta-woodland-caribou-population
#18
Yo Ching Cheng, Marco Musiani, Maria Cavedon, Sabine Gilch
Chronic wasting disease (CWD) is a prion disease found in deer, elk and moose in North America and since recently, wild reindeer in Norway. Caribou are at-risk to encounter CWD in areas such as Alberta, Canada, where the disease spreads toward caribou habitats. CWD susceptibility is modulated by species-specific polymorphisms in the prion protein gene (Prnp). We sequenced Prnp of woodland caribou from 9 Albertan populations. In one population (Chinchaga) a significantly higher frequency of the 138N allele linked to reduced CWD susceptibility was observed...
March 4, 2017: Prion
https://www.readbyqxmd.com/read/28324664/limited-understanding-of-the-functional-diversity-of-n-linked-glycans-as-a-major-gap-of-prion-biology
#19
Ilia V Baskakov
Among a broad range of hypotheses on the molecular nature of transmissible spongiform encephalopathy or scrapie agents discussed in 1960s was a hypothesis of self-replicating polysaccharides. While the studies of the past 40 years provided unambiguous proof that this is not the case, emerging evidence suggests that carbohydrates in the form of sialylated N-linked glycans, which are a constitutive part of mammalian prions or PrP(Sc), are essential in determining prion fate in an organism. The current extra-view article discusses recent advancements on the role of N-linked glycans and specifically their sialylation status in controlling prion fate...
March 4, 2017: Prion
https://www.readbyqxmd.com/read/28278113/clinical-and-laboratory-features-of-14-young-chinese-probable-scjd-patients
#20
Qi Shi, Kang Xiao, Cao Chen, Wei Zhou, Chen Gao, Jing Wang, Bao-Yun Zhang, Yuan Wang, Xiao-Ping Dong
Sporadic Creutzfeldt-Jakob disease (sCJD) occurs frequently in the relatively older population, mainly in the groups of 60-69 and 70-79 year-old. Since 2006 when China performed national CJD surveillance, 14 young probable sCJD patients below 40 year-old were identified, counting for 1.93% of all probable sCJD cases. The clinical features of young probable sCJD cases, including the onset feature, the presence of sCJD-associated signs and the clinical duration, are indistinguishable from those of older patients...
March 4, 2017: Prion
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