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https://www.readbyqxmd.com/read/28421536/lysosomal-quality-control-in-prion-diseases
#1
REVIEW
Priyanka Majumder, Oishee Chakrabarti
Prion diseases are transmissible, familial or sporadic. The prion protein (PrP), a normal cell surface glycoprotein, is ubiquitously expressed throughout the body. While loss of function of PrP does not elicit apparent phenotypes, generation of misfolded forms of the protein or its aberrant metabolic isoforms has been implicated in a number of neurodegenerative disorders such as scrapie, kuru, Creutzfeldt-Jakob disease, fatal familial insomnia, Gerstmann-Sträussler-Scheinker and bovine spongiform encephalopathy...
April 18, 2017: Molecular Neurobiology
https://www.readbyqxmd.com/read/28407243/simulations-of-membrane-bound-diglycosylated-human-prion-protein-reveal-potential-protective-mechanisms-against-misfolding
#2
Chin Jung Cheng, Heidi Koldsø, Marc W Van der Kamp, Birgit Schiøtt, Valerie Daggett
Prion diseases are associated with the misfolding of the prion protein (PrP) from its normal cellular form (PrP(C) ) to its infectious scrapie form (PrP(S)(c) ). Posttranslational modifications of PrP in vivo can play an important role in modulating the process of misfolding. To gain more insight into the effects of posttranslational modifications on PrP structure and dynamics and to test the hypothesis that such modifications can interact with the protein, we have performed molecular dynamics simulations of diglycosylated human PrP(C) bound to a lipid bilayer via a glycophosphatidylinositol anchor...
April 13, 2017: Journal of Neurochemistry
https://www.readbyqxmd.com/read/28362554/detection-of-a-new-20-bp-insertion-deletion-indel-within-sheep-prnd-gene-using-mathematical-expectation-me-method
#3
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/28324664/limited-understanding-of-the-functional-diversity-of-n-linked-glycans-as-a-major-gap-of-prion-biology
#4
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/28268246/cerebellar-compartmentation-of-prion-pathogenesis
#5
Audrey Ragagnin, Juliette Ezpeleta, Aurélie Guillemain, François Boudet-Devaud, Anne-Marie Haeberlé, Valérie Demais, Catherine Vidal, Stanislas Demuth, Vincent Béringue, Odile Kellermann, Benoit Schneider, Nancy J Grant, Yannick Bailly
In prion diseases, the brain lesion profile is influenced by the prion "strain" properties, the invasion route to the brain, and still unknown host cell-specific parameters. To gain insight into those endogenous factors, we analyzed the histopathological alterations induced by distinct prion strains in the mouse cerebellum. We show that 22L and ME7 scrapie prion proteins (PrP(22L) , PrP(ME7) ), but not bovine spongiform encephalopathy PrP(6PB1) , accumulate in a reproducible parasagittal banding pattern in the cerebellar cortex of infected mice...
March 7, 2017: Brain Pathology
https://www.readbyqxmd.com/read/28236170/pathogen-safety-of-a-new-intravenous-immune-globulin-10-liquid
#6
Kai Uwe Radomski, Georg Lattner, Torben Schmidt, Jürgen Römisch
BACKGROUND: The manufacturing process of a new intravenous immune globulin (IVIG) 10% liquid product incorporates two dedicated pathogen safety steps: solvent/detergent (S/D) treatment and nanofiltration (20 nm). Ion-exchange chromatography (IEC) during protein purification also contributes to pathogen safety. The ability of these three process steps to inactivate/remove viruses and prions was evaluated. OBJECTIVES: The objective of this study was to evaluate the virus and prion safety of the new IVIG 10% liquid...
April 2017: BioDrugs: Clinical Immunotherapeutics, Biopharmaceuticals and Gene Therapy
https://www.readbyqxmd.com/read/28213529/case-control-study-on-the-use-of-pituitary-derived-hormones-from-sheep-as-a-potential-risk-factor-for-the-occurrence-of-atypical-scrapie-in-great-britain
#7
E Marier, M Dawson, M Simmons, J Hope, A Ortiz-Peláez
A case-control study was conducted in 2013 to investigate the use of pituitary-derived hormones from sheep as a potential risk factor for the presence of atypical scrapie in Great Britain sheep holdings. One hundred and sixty-five holdings were identified as cases. Two equal sets of controls were selected: no case of scrapie and cases of classical scrapie. A total of 495 holdings were selected for the questionnaire survey, 201 responses were received and 190 (38.3 per cent) were suitable for analysis. The variables 'use-of-heat-synchronisation/superovulation' and 'flock size' were significantly associated with the occurrence of atypical scrapie...
February 17, 2017: Veterinary Record
https://www.readbyqxmd.com/read/28194643/ultra-efficient-amplification-of-abnormal-prion-protein-by-modified-protein-misfolding-cyclic-amplification-with-electric-current
#8
Jeong-Ho Park, Yeong-Gon Choi, Seok-Joo Park, Hong-Seok Choi, Eun-Kyoung Choi, Yong-Sun Kim
Prion diseases are clinically diagnosed and confirmed upon post-mortem histopathological examination of brain tissue. The only reliable molecular marker for prion diseases is abnormal prion protein (PrPSc), a pathologically conformed prion protein that primarily accumulates in the central nervous system and to a lesser extent in lymphoreticular tissues. However, the use of PrPSc as a marker for preclinical diagnoses is limited because the concentration of PrPSc in easily accessible body fluids is extremely low...
February 13, 2017: Molecular Neurobiology
https://www.readbyqxmd.com/read/28193724/neurodegenerative-disease-transmission-and-transgenesis-in-mice
#9
Brittany N Dugger, Daniel P Perl, George A Carlson
Although the discovery of the prion protein (PrP) resulted from its co-purification with scrapie infectivity in Syrian hamsters, work with genetically defined and genetically modified mice proved crucial for understanding the fundamental processes involved not only in prion diseases caused by PrP misfolding, aggregation, and spread but also in other, much more common, neurodegenerative brain diseases. In this review, we focus on methodological and conceptual approaches used to study scrapie and related PrP misfolding diseases in mice and how these approaches have advanced our understanding of related disorders including Alzheimer's and Parkinson's disease...
February 13, 2017: Cold Spring Harbor Perspectives in Biology
https://www.readbyqxmd.com/read/28178353/cystatin-f-is-a-biomarker-of-prion-pathogenesis-in-mice
#10
Mario Nuvolone, Nicolas Schmid, Gino Miele, Silvia Sorce, Rita Moos, Christian Schori, Roger R Beerli, Monika Bauer, Philippe Saudan, Klaus Dietmeier, Ingolf Lachmann, Michael Linnebank, Roland Martin, Ulf Kallweit, Veronika Kana, Elisabeth J Rushing, Herbert Budka, Adriano Aguzzi
Misfolding of the cellular prion protein (PrPC) into the scrapie prion protein (PrPSc) results in progressive, fatal, transmissible neurodegenerative conditions termed prion diseases. Experimental and epidemiological evidence point toward a protracted, clinically silent phase in prion diseases, yet there is no diagnostic test capable of identifying asymptomatic individuals incubating prions. In an effort to identify early biomarkers of prion diseases, we have compared global transcriptional profiles in brains from pre-symptomatic prion-infected mice and controls...
2017: PloS One
https://www.readbyqxmd.com/read/28154522/metal-dyshomeostasis-and-their-pathological-role-in-prion-and-prion-like-diseases-the-basis-for-a-nutritional-approach
#11
REVIEW
Mattia Toni, Maria L Massimino, Agnese De Mario, Elisa Angiulli, Enzo Spisni
Metal ions are key elements in organisms' life acting like cofactors of many enzymes but they can also be potentially dangerous for the cell participating in redox reactions that lead to the formation of reactive oxygen species (ROS). Any factor inducing or limiting a metal dyshomeostasis, ROS production and cell injury may contribute to the onset of neurodegenerative diseases or play a neuroprotective action. Transmissible spongiform encephalopathies (TSEs), also known as prion diseases, are a group of fatal neurodegenerative disorders affecting the central nervous system (CNS) of human and other mammalian species...
2017: Frontiers in Neuroscience
https://www.readbyqxmd.com/read/28144628/de-novo-generation-of-a-unique-cervid-prion-strain-using-protein-misfolding-cyclic-amplification
#12
Crystal Meyerett-Reid, A Christy Wyckoff, Terry Spraker, Bruce Pulford, Heather Bender, Mark D Zabel
Substantial evidence supports the hypothesis that prions are misfolded, infectious, insoluble, and protease-resistant proteins (PrP(RES)) devoid of instructional nucleic acid that cause transmissible spongiform encephalopathies (TSEs). Protein misfolding cyclic amplification (PMCA) has provided additional evidence that PrPRes acts as a template that can convert the normal cellular prion protein (PrP(C)) present in uninfected normal brain homogenate (NBH) into the infectious misfolded PrP(RES) isoform. Human PrP(C) has been shown to spontaneously convert to a misfolded pathological state causing sporadic Creutzfeldt-Jakob disease (sCJD)...
January 2017: MSphere
https://www.readbyqxmd.com/read/28118747/in-silico-strategies-on-prion-pathogenic-conversion-and-inhibition-from-prp-c-prp-sc
#13
Nataraj S Pagadala, Khajamohiddin Syed, Rakesh Bhat
To date, various therapeutic strategies identified numerous anti-prion compounds and antibodies that stabilize PrP(C), block the conversion of PrP(C)-PrP(Sc) and increased effect on PrP(Sc) clearance. However, no suitable drug has been identified clinically so far due to the poor oral absorption, low blood-brain-barrier [BBB] penetration, and high toxicity. Although some of the drugs were proven to be effective in prion-infected cell culture and whole animal models, none of them increased the rate of survival compared to placebo...
February 2, 2017: Expert Opinion on Drug Discovery
https://www.readbyqxmd.com/read/28116677/a-heparin-purification-process-removes-spiked-transmissible-spongiform-encephalopathy-agent
#14
Cyrus Bett, Ksenija Grgac, Dianna Long, Michael Karfunkle, David A Keire, David M Asher, Luisa Gregori
In 2000, bovine heparin was withdrawn from the US market for fear of contamination with bovine spongiform encephalopathy (BSE) agent, the cause of variant Creutzfeldt-Jakob disease in humans. Thus, US heparin is currently sourced only from pig intestines. Availability of alternative sources of crude heparin, a life-saving drug, would benefit public health. Bovine heparin is an obvious option, but BSE clearance by the bovine heparin manufacturing process should be evaluated. To this end, using hamster 263K scrapie as a surrogate for BSE agent, we applied a four-step bench-scale heparin purification protocol resembling a typical heparin manufacturing process to investigate removal of the spiked scrapie agent...
January 23, 2017: AAPS Journal
https://www.readbyqxmd.com/read/28112164/divergent-prion-strain-evolution-driven-by-prp-c-expression-level-in-transgenic-mice
#15
Annick Le Dur, Thanh Lan Laï, Marie-George Stinnakre, Aude Laisné, Nathalie Chenais, Sabine Rakotobe, Bruno Passet, Fabienne Reine, Solange Soulier, Laetitia Herzog, Gaëlle Tilly, Human Rézaei, Vincent Béringue, Jean-Luc Vilotte, Hubert Laude
Prions induce a fatal neurodegenerative disease in infected host brain based on the refolding and aggregation of the host-encoded prion protein PrP(C) into PrP(Sc). Structurally distinct PrP(Sc) conformers can give rise to multiple prion strains. Constrained interactions between PrP(C) and different PrP(Sc) strains can in turn lead to certain PrP(Sc) (sub)populations being selected for cross-species transmission, or even produce mutation-like events. By contrast, prion strains are generally conserved when transmitted within the same species, or to transgenic mice expressing homologous PrP(C)...
January 23, 2017: Nature Communications
https://www.readbyqxmd.com/read/28109330/the-structure-of-mammalian-prions-and-their-aggregates
#16
E Vázquez-Fernández, H S Young, J R Requena, H Wille
Prion diseases, such as Creutzfeldt-Jakob disease in humans, bovine spongiform encephalopathy in cattle, chronic wasting disease in cervids (i.e., deer, elk, moose, and reindeer), and sheep scrapie, are caused by the misfolding of the cellular prion protein (PrP(C)) into a disease-causing conformer (PrP(Sc)). PrP(C) is a normal, GPI-anchored protein that is expressed on the surface of neurons and other cell types. The structure of PrP(C) is well understood, based on studies of recombinant PrP, which closely mimics the structure of native PrP(C)...
2017: International Review of Cell and Molecular Biology
https://www.readbyqxmd.com/read/28102071/temperature-induced-misfolding-in-prion-protein-evidence-of-multiple-partially-disordered-states-stabilized-by-non-native-hydrogen-bonds
#17
Neharika G Chamachi, Suman Chakrabarty
The structural basis of pathways of misfolding of a cellular prion (PrP(C)) into the toxic scrapie form (PrP(SC)) and identification of possible intermediates (e.g., PrP*) still eludes us. In this work, we have used a cumulative ∼65 μs of replica exchange molecular dynamics simulation data to construct the conformational free energy landscapes and capture the structural and thermodynamic characteristics associated with various stages of the thermal denaturation process in human prion protein. The temperature-dependent free energy surfaces consist of multiple metastable states stabilized by non-native contacts and hydrogen bonds, thus rendering the protein prone to misfolding...
February 2, 2017: Biochemistry
https://www.readbyqxmd.com/read/28091514/novel-strain-properties-distinguishing-sporadic-prion-diseases-sharing-prion-protein-genotype-and-prion-type
#18
Laura Cracco, Silvio Notari, Ignazio Cali, Man-Sun Sy, Shu G Chen, Mark L Cohen, Bernardino Ghetti, Brian S Appleby, Wen-Quan Zou, Byron Caughey, Jiri G Safar, Pierluigi Gambetti
In most human sporadic prion diseases the phenotype is consistently associated with specific pairings of the genotype at codon 129 of the prion protein gene and conformational properties of the scrapie PrP (PrP(Sc)) grossly identified types 1 and 2. This association suggests that the 129 genotype favours the selection of a distinct strain that in turn determines the phenotype. However, this mechanism cannot play a role in the phenotype determination of sporadic fatal insomnia (sFI) and a subtype of sporadic Creutzfeldt-Jakob disease (sCJD) identified as sCJDMM2, which share 129 MM genotype and PrP(Sc) type 2 but are associated with quite distinct phenotypes...
January 16, 2017: Scientific Reports
https://www.readbyqxmd.com/read/28072756/rapid-testing-for-creutzfeldt-jakob-disease-in-donors-of-cornea
#19
Luisa Gregori, Arthur R Serer, Kristy L McDowell, Juraj Cervenak, David M Asher
BACKGROUND: Creutzfeldt-Jakob disease (CJD) has been accidentally transmitted by contaminated corneal transplants. Eye donors are not ordinarily tested for CJD, in part because an easy test is not available. We propose a relatively simple postmortem procedure to collect brain samples without performing full autopsy and show that a test currently marketed for veterinary diagnosis would offer an effective screening test. METHODS: We selected 6 brains from confirmed cases of human sporadic CJD and sampled each in triplicate (18 specimens), 28 control brains of individuals with non-CJD neurodegenerative diseases and 10 normal brains...
April 2017: Transplantation
https://www.readbyqxmd.com/read/28068881/eu-approved-rapid-tests-might-underestimate-bovine-spongiform-encephalopathy-infection-in-goats
#20
Daniela Meloni, Elena Bozzetta, Jan P M Langeveld, Martin H Groschup, Wilfred Goldmann, Olivier Andrèoletti, Isabelle Lantier, Lucien Van Keulen, Alex Bossers, Danilo Pitardi, Romolo Nonno, Theodoros Sklaviadis, Francesco Ingravalle, Simone Peletto, Silvia Colussi, Pier Luigi Acutis
We report the diagnostic sensitivity of 3 EU-approved rapid tests (ELISAs; 1 from IDEXX and 2 from Bio-Rad) for the detection of transmissible spongiform encephalopathy diseases in goats. Ninety-eight goat brainstem samples were tested. All the rapid tests had 100% specificity and ≥80% sensitivity, with the IDEXX test significantly more sensitive than the 2 Bio-Rad tests. All tests detected 100% of samples from goats with clinical scrapie, but missed 8% (IDEXX) to 33% (Bio-Rad SG) of samples from preclinical goats...
January 1, 2017: Journal of Veterinary Diagnostic Investigation
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