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Prion

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https://www.readbyqxmd.com/read/28521568/prion-based-memory-of-heat-stress-in-yeast
#1
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 19, 2017: Prion
https://www.readbyqxmd.com/read/28509623/gastrostomy-in-patients-with-prion-disease
#2
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 three 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 three 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 16, 2017: Prion
https://www.readbyqxmd.com/read/28509609/amyloid-and-fdg-pet-in-sporadic-creutzfeldt-jakob-disease-correlation-with-pathological-prion-protein-in-neuropathology
#3
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 16, 2017: Prion
https://www.readbyqxmd.com/read/28486039/flow-cytometric-measurement-of-the-cellular-propagation-of-tdp-43-aggregation
#4
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 9, 2017: Prion
https://www.readbyqxmd.com/read/28402718/prions-on-the-run-how-extracellular-vesicles-serve-as-delivery-vehicles-for-self-templating-protein-aggregates
#5
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
#6
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
#7
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
#8
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
#9
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
#10
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
#11
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
https://www.readbyqxmd.com/read/28277942/hitchhiking-vesicular-transport-routes-to-the-vacuole-amyloid-recruitment-to-the-insoluble-protein-deposit-ipod
#12
Rajesh Kumar, Nicole Neuser, Jens Tyedmers
Sequestration of aggregates into specialized deposition sites occurs in many species across all kingdoms of life ranging from bacteria to mammals and is commonly believed to have a cytoprotective function. Yeast cells possess at least 3 different spatially separated deposition sites, one of which is termed "Insoluble Protein Deposit (IPOD)" and harbors amyloid aggregates. We have recently discovered that recruitment of amyloid aggregates to the IPOD uses an actin cable based recruitment machinery that also involves vesicular transport...
March 4, 2017: Prion
https://www.readbyqxmd.com/read/28281930/combined-trna-modification-defects-impair-protein-homeostasis-and-synthesis-of-the-yeast-prion-protein-rnq1
#13
Raffael Schaffrath, Roland Klassen
Modified nucleosides in tRNA anticodon loops such as 5-methoxy-carbonyl-methyl-2-thiouridine (mcm(5)s(2)U) and pseuduridine (Ψ) are thought to be required for an efficient decoding process. In Saccharomyces cerevisiae, the simultaneous presence of mcm(5)s(2)U and Ψ38 in tRNA(Gln)UUG was shown to mediate efficient synthesis of the Q/N rich [PIN(+)] prion forming protein Rnq1. (1) In the absence of these two tRNA modifications, higher than normal levels of hypomodified tRNA(Gln)UUG, but not its isoacceptor tRNA(Gln)CUG can restore Rnq1 synthesis...
January 2, 2017: Prion
https://www.readbyqxmd.com/read/28281929/in-vitro-amplification-of-h-type-atypical-bovine-spongiform-encephalopathy-by-protein-misfolding-cyclic-amplification
#14
Matthew J O'Connor, Keith Bishop, Robert G Workman, Ben C Maddison, Kevin C Gough
The in vitro amplification of prions by serial protein misfolding cyclic amplification has been shown to detect PrP(Sc) to levels at least as sensitive as rodent bioassay but in a fraction of the time. Bovine spongiform encephalopathy is a zoonotic prion disease in cattle and has been shown to occur in 3 distinct forms, classical BSE (C-BSE) and 2 atypical BSE forms (L-BSE and H-BSE). Atypical forms are usually detected in asymptomatic, older cattle and are suggested to be spontaneous forms of the disease. Here, we show the development of a serial protein misfolding cyclic amplification method for the detection of H-BSE...
January 2, 2017: Prion
https://www.readbyqxmd.com/read/28281928/amyloid-cores-in-prion-domains-key-regulators-for-prion-conformational-conversion
#15
María Rosario Fernández, Cristina Batlle, Marcos Gil-García, Salvador Ventura
Despite the significant efforts devoted to decipher the particular protein features that encode for a prion or prion-like behavior, they are still poorly understood. The well-characterized yeast prions constitute an ideal model system to address this question, because, in these proteins, the prion activity can be univocally assigned to a specific region of their sequence, known as the prion forming domain (PFD). These PFDs are intrinsically disordered, relatively long and, in many cases, of low complexity, being enriched in glutamine/asparagine residues...
January 2, 2017: Prion
https://www.readbyqxmd.com/read/28281927/are-brazilian-cervids-at-risk-of-prion-diseases
#16
Caio Bruno Ribeiro Falcão, Isabel Luiza de Melo Nunes Freire Lima, José Maurício Barbanti Duarte, João Ricardo Mendes de Oliveira, Rodrigo Augusto Torres, Artur Maia Wanderley, José Eriton Gomes da Cunha, José Eduardo Garcia
Prion diseases are neurodegenerative fatal disorders that affect human and non-human mammals. Chronic Wasting Disease (CWD) is a prion disease of cervids regarded as a public health problem in North America, and polymorphisms at specific codons in the PRNP gene are associated with this disease. To assess the potential CWD susceptibility of South American free-ranging deer, the presence of these polymorphisms was examined in Mazama gouazoubira, Ozotoceros bezoarticus and Blastocerus dichotomus. Despite the lack of CWD reports in Brazil, the examined codons (95, 96, 116, 132, 225, and 226) of the PRNP gene showed potential CWD susceptibility in Brazilian deer...
January 2, 2017: Prion
https://www.readbyqxmd.com/read/28281926/allelic-variants-of-hereditary-prions-the-bimodularity-principle
#17
Oleg N Tikhodeyev, Oleg V Tarasov, Stanislav A Bondarev
Modern biology requires modern genetic concepts equally valid for all discovered mechanisms of inheritance, either "canonical" (mediated by DNA sequences) or epigenetic. Applying basic genetic terms such as "gene" and "allele" to protein hereditary factors is one of the necessary steps toward these concepts. The basic idea that different variants of the same prion protein can be considered as alleles has been previously proposed by Chernoff and Tuite. In this paper, the notion of prion allele is further developed...
January 2, 2017: Prion
https://www.readbyqxmd.com/read/28281925/infectious-prions-and-proteinopathies
#18
Rona M Barron
Transmissible spongiform encephalopathies (TSEs) are caused by an infectious agent that is thought to consist of only misfolded and aggregated prion protein (PrP). Unlike conventional micro-organisms, the agent spreads and propagates by binding to and converting normal host PrP into the abnormal conformer, increasing the infectious titre. Synthetic prions, composed of refolded fibrillar forms of recombinant PrP (rec-PrP) have been generated to address whether PrP aggregates alone are indeed infectious prions...
January 2, 2017: Prion
https://www.readbyqxmd.com/read/28281924/a-stretch-of-residues-within-the-protease-resistant-core-is-not-necessary-for-prion-structure-and-infectivity
#19
Carola Munoz-Montesino, Christina Sizun, Mohammed Moudjou, Laetitia Herzog, Fabienne Reine, Angelique Igel-Egalon, Clément Barbereau, Jérôme Chapuis, Danica Ciric, Hubert Laude, Vincent Béringue, Human Rezaei, Michel Dron
Mapping out regions of PrP influencing prion conversion remains a challenging issue complicated by the lack of prion structure. The portion of PrP associated with infectivity contains the α-helical domain of the correctly folded protein and turns into a β-sheet-rich insoluble core in prions. Deletions performed so far inside this segment essentially prevented the conversion. Recently we found that deletion of the last C-terminal residues of the helix H2 was fully compatible with prion conversion in the RK13-ovPrP cell culture model, using 3 different infecting strains...
January 2, 2017: Prion
https://www.readbyqxmd.com/read/28281923/in-memory-of-susan-lindquist-1949-2016
#20
Yury O Chernoff
No abstract text is available yet for this article.
January 2, 2017: Prion
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