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https://www.readbyqxmd.com/read/27921253/aberrant-alterations-of-mitochondrial-factors-drp1-and-opa1-in-the-brains-of-scrapie-experiment-rodents
#1
Xiao -Dong Yang, Qi Shi, Jing Sun, Yan Lv, Yue Ma, Cao Chen, Kang Xiao, Wei Zhou, Xiao-Ping Dong
The abnormal mitochondrial dynamics has been reported in the brains of some neurodegenerative diseases, such as Alzheimer's disease (AD) and Parkinson's disease (PD), but limitedly described in prion disease. Dynamin-related protein 1 (Drpl) and optic atrophy protein 1 (Opa1) are two essential elements for mitochondria fission and fusion. To evaluate possible changes of mitochondria dynamics during prion infection, the situations of brain Drp1 and Opa1 of scrapie strains 139A, ME7, and S15 mice, as well as 263K-infected hamsters, were analyzed...
December 6, 2016: Journal of Molecular Neuroscience: MN
https://www.readbyqxmd.com/read/27891596/in-this-issue-december-2016-chemical-immobilisation-of-free-range-cattle%C3%A2-%C3%A2-%C3%A2-dairy-cow-progesterone-assay%C3%A2-%C3%A2-%C3%A2-atypical-scrapie-in-sheep%C3%A2-%C3%A2-%C3%A2-antibiotic-resistance-in-healthy-dogs%C3%A2-%C3%A2-%C3%A2-contrast-ct-in-dermoid-sinuses%C3%A2-%C3%A2-pneumorrhachis-in-a-dog%C3%A2-%C3%A2-%C3%A2-dorsal-perineal
#2
EDITORIAL
https://www.readbyqxmd.com/read/27880822/isolation-of-a-defective-prion-mutant-from-natural-scrapie
#3
Ilaria Vanni, Sergio Migliore, Gian Mario Cosseddu, Michele Angelo Di Bari, Laura Pirisinu, Claudia D'Agostino, Geraldina Riccardi, Umberto Agrimi, Romolo Nonno
It is widely known that prion strains can mutate in response to modification of the replication environment and we have recently reported that prion mutations can occur in vitro during amplification of vole-adapted prions by Protein Misfolding Cyclic Amplification on bank vole substrate (bvPMCA). Here we exploited the high efficiency of prion replication by bvPMCA to study the in vitro propagation of natural scrapie isolates. Although in vitro vole-adapted PrPSc conformers were usually similar to the sheep counterpart, we repeatedly isolated a PrPSc mutant exclusively when starting from extremely diluted seeds of a single sheep isolate...
November 2016: PLoS Pathogens
https://www.readbyqxmd.com/read/27852982/regulation-of-human-cerebrospinal-fluid-malate-dehydrogenase-1-in-sporadic-creutzfeldt-jakob-disease-patients
#4
Matthias Schmitz, Franc Llorens, Alexander Pracht, Tobias Thom, Ângela Correia, Saima Zafar, Isidre Ferrer, Inga Zerr
The identification of reliable diagnostic biomarkers in differential diagnosis of neurodegenerative diseases is an ongoing topic. A previous two-dimensional proteomic study on cerebrospinal fluid (CSF) revealed an elevated level of an enzyme, mitochondrial malate dehydrogenase 1 (MDH1), in sporadic Creutzfeldt-Jakob disease (sCJD) patients. Here, we could demonstrate the expression of MDH1 in neurons as well as in the neuropil. Its levels are lower in sCJD brains than in control brains. An examination of CSF-MDH1 in sCJD patients by ELISA revealed a significant elevation of CSF-MDH1 levels in sCJD patients (independently from the PRNP codon 129 MV genotype or the prion protein scrapie (PrP(Sc)) type) in comparison to controls...
November 14, 2016: Aging
https://www.readbyqxmd.com/read/27849581/amyloid-fibrils-from-the-n-terminal-prion-protein-fragment-are-infectious
#5
Jin-Kyu Choi, Ignazio Cali, Krystyna Surewicz, Qingzhong Kong, Pierluigi Gambetti, Witold K Surewicz
Recombinant C-terminally truncated prion protein PrP23-144 (which corresponds to the Y145Stop PrP variant associated with a Gerstmann-Sträussler-Scheinker-like prion disease) spontaneously forms amyloid fibrils with a parallel in-register β-sheet architecture and β-sheet core mapping to residues ∼112-139. Here we report that mice (both tga20 and wild type) inoculated with a murine (moPrP23-144) version of these fibrils develop clinical prion disease with a 100% attack rate. Remarkably, even though fibrils in the inoculum lack the entire C-terminal domain of PrP, brains of clinically sick mice accumulate longer proteinase K-resistant (PrP(res)) fragments of ∼17-32 kDa, similar to those observed in classical scrapie strains...
November 29, 2016: Proceedings of the National Academy of Sciences of the United States of America
https://www.readbyqxmd.com/read/27847358/prp-knockout-cells-expressing-transmembrane-prp-resist-prion-infection
#6
Karen E Marshall, Andrew Hughson, Sarah Vascellari, Suzette A Priola, Akikazu Sakudo, Takashi Onodera, Gerald S Baron
: Glycosylphosphatidylinositol (GPI) anchoring of the prion protein (PrP(C)) influences PrP(C) misfolding into the disease-associated isoform, PrP(res), as well as prion propagation and infectivity. GPI proteins are found in cholesterol and sphingolipid-rich membrane regions called rafts. Exchanging the GPI anchor for a non-raft transmembrane sequence redirects PrP(C) away from rafts. Previous studies showed that non-raft transmembrane PrP(C) variants resist conversion to PrP(res) when transfected into scrapie-infected N2a neuroblastoma cells, likely due to segregation of transmembrane PrP(C) and GPI-anchored PrP(res) in distinct membrane environments...
November 9, 2016: Journal of Virology
https://www.readbyqxmd.com/read/27836542/anchorless-forms-of-prion-protein-impact-of-truncation-on-structure-destabilization-and-prion-protein-conversion
#7
Valerija Kovač, Iva Hafner-Bratkovič, Vladka Čurin Šerbec
Prion diseases are a group of fatal neurodegenerative diseases caused by scrapie form of prion protein, PrP(Sc). Prion protein (PrP) is bound to the cell via glycophosphatidylinositol (GPI) anchor. The role of GPI anchor in PrP(Sc) replication and propagation remains unclear. It has been shown that anchorless and truncated PrP accelerate the formation and propagation of prions in vivo and further increases the risk for transmission of prion diseases among species. To explain the role of anchorless forms of PrP in the development of prion diseases, we have prepared five C-terminal PrP truncated variants, determined their thermodynamic properties and analyzed the kinetics of conversion into amyloid fibrils...
December 2, 2016: Biochemical and Biophysical Research Communications
https://www.readbyqxmd.com/read/27826142/mirna-expression-profiles-in-the-brains-of-mice-infected-with-scrapie-agents-139a-me7-and-s15
#8
Chen Gao, Jing Wei, Bao-Yun Zhang, Qiang Shi, Cao Chen, Jing Wang, Qi Shi, Xiao-Ping Dong
MicroRNA (miRNA) is a class of non-coding endogenous small-molecule single-stranded RNA that regulates complementary mRNA through degradation or translation of the mRNA targets. Usually, miRNAs show remarkable cell and tissues specificity. Recently, alterations in a set of miRNAs in the brains of patients with certain neurodegenerative diseases, including prion diseases, have been reported. In this study, using deep sequencing technology, miRNA expression profiles in the brains of mice infected with scrapie agents 139A, ME7 and S15 at a terminal stage were comparatively analysed...
November 9, 2016: Emerging Microbes & Infections
https://www.readbyqxmd.com/read/27825366/l-bse-experimentally-transmitted-to-sheep-presents-as-a-unique-disease-phenotype
#9
Marion M Simmons, Melanie J Chaplin, Timm Konold, Cristina Casalone, Katy E Beck, Leigh Thorne, Sharon Everitt, Tobias Floyd, Derek Clifford, John Spiropoulos
Apart from prion protein genotype, the factors determining the host range and susceptiblity for specific transmissible spongiform encephalopathy agents remain unclear. It is known that bovine atypical L-BSE can transmit to a range of species including primates and humanised transgenic mice. It is important, therefore, that there is as broad an understanding as possible of how such isolates might present in food animal species and how robust they are on inter- and intra-species transmission to inform surveillance sytems and risk assessments...
November 8, 2016: Veterinary Research
https://www.readbyqxmd.com/read/27818203/cytotoxicity-of-prion-protein-derived-cell-penetrating-peptides-is-modulated-by-ph-but-independent-of-amyloid-formation
#10
Vineeth Mukundan, Christy Maksoudian, Maria C Vogel, Ibrahim Chehade, Marios S Katsiotis, Saeed M Alhassan, Mazin Magzoub
Prion diseases are associated with conversion of cellular prion protein (PrP(C)) into an abnormally folded and infectious scrapie isoform (PrP(Sc)). We previously showed that peptides derived from the unprocessed N-termini of mouse and bovine prion proteins, mPrP1-28 and bPrP1-30, function as cell-penetrating peptides (CPPs), and destabilize model membrane systems, which could explain the infectivity and toxicity of prion diseases. However, subsequent studies revealed that treatment with mPrP1-28 or bPrP1-30 significantly reduce PrP(Sc) levels in prion-infected cells...
November 3, 2016: Archives of Biochemistry and Biophysics
https://www.readbyqxmd.com/read/27810202/detecting-animal-by-product-intake-using-stable-isotope-ratio-mass-spectrometry-irms
#11
D A F da Silva, N P Biscola, L D Dos Santos, M M P Sartori, J C Denadai, E T da Silva, C Ducatti, S D Bicudo, B Barraviera, R S Ferreira
Sheep are used in many countries as food and for manufacturing bioproducts. However, when these animals consume animal by-products (ABP), which is widely prohibited, there is a risk of transmitting scrapie - a fatal prion disease in human beings. Therefore, it is essential to develop sensitive methods to detect previous ABP intake to select safe animals for producing biopharmaceuticals. We used stable isotope ratio mass spectrometry (IRMS) for (13)C and (15)N to trace animal proteins in the serum of three groups of sheep: 1 - received only vegetable protein (VP) for 89 days; 2 - received animal and vegetable protein (AVP); and 3 - received animal and vegetable protein with animal protein subsequently removed (AVPR)...
November 2016: Veterinary Journal
https://www.readbyqxmd.com/read/27807855/atypical-scrapie-in-australia
#12
R W Cook, J Bingham, A S Besier, C L Bayley, M Hawes, P L Shearer, M Yamada, J Bergfeld, D T Williams, D J Middleton
BACKGROUND: Since its initial detection in Norway in 1998, atypical scrapie ('atypical/Nor98 scrapie') has been reported in sheep in the majority of European countries (including in regions free of classical scrapie) and in the Falkland Islands, the USA, Canada, New Zealand and Australia. CASE SERIES: The diagnosis in Australia of atypical scrapie in four Merino and one Merino-cross sheep showing clinical signs of neurological disease was based on the detection of grey matter neuropil vacuolation (spongiform change) in the brain (particularly in the molecular layer of the cerebellar cortex) and associated abnormal prion protein (PrP(Sc) ) deposition in both grey and white matter...
November 3, 2016: Australian Veterinary Journal
https://www.readbyqxmd.com/read/27803163/identification-of-anti-prion-compounds-using-a-novel-cellular-assay
#13
Thibaut Imberdis, James T Heeres, Han Yueh, Cheng Fang, Jessie Zhen, Celeste B Rich, Marcie Glicksman, Aaron Beeler, David A Harris
Prion diseases are devastating neurodegenerative disorders with no known cure. One strategy for developing therapies for these diseases is to identify compounds that block conversion of the cellular form of the prion protein (PrPC) into the infectious isoform (PrPSc). Most previous efforts to discover such molecules by high-throughput screening methods have utilized, as a read-out, a single kind of cellular assay system: neuroblastoma cells that are persistently infected with scrapie prions. Here, we describe the use of an alternative cellular assay based on suppressing the spontaneous cytotoxicity of a mutant form of PrP (Δ105-125)...
November 1, 2016: Journal of Biological Chemistry
https://www.readbyqxmd.com/read/27751198/goats-with-aspartic-acid-or-serine-at-codon-146-of-the-prnp-gene-remain-scrapie-negative-after-lifetime-exposure-in-affected-herds-in-cyprus
#14
S Georgiadou, A Ortiz-Pelaez, M M Simmons, O Windl, M Dawson, P Neocleous, P Papasavva-Stylianou
The results of the study reported here are part of an ongoing integrated research programme aimed at producing additional, robust, evidence on the genetic resistance to classical scrapie in goats, with particular reference to codon 146. The study targeted animals aged ⩾6 years, which were born and raised in infected herds and were being culled for management reasons. A total of 556 animals were tested, and all positive animals (n = 117) were of the susceptible NN genotype. A total of 246 goats heterozygous or homozygous for putatively resistant alleles (S146 and D146) were screened with no positive results...
October 18, 2016: Epidemiology and Infection
https://www.readbyqxmd.com/read/27735933/the-real-time-quaking-induced-conversion-assay-for-detection-of-human-prion-disease-and-study-of-other-protein-misfolding-diseases
#15
Matthias Schmitz, Maria Cramm, Franc Llorens, Dominik Müller-Cramm, Steven Collins, Ryuichiro Atarashi, Katsuya Satoh, Christina D Orrù, Bradley R Groveman, Saima Zafar, Walter J Schulz-Schaeffer, Byron Caughey, Inga Zerr
The development and adaption of in vitro misfolded protein amplification systems has been a major innovation in the detection of abnormally folded prion protein scrapie (PrP(Sc)) in human brain and cerebrospinal fluid (CSF) samples. Herein, we describe a fast and efficient protein amplification technique, real-time quaking-induced conversion (RT-QuIC), for the detection of a PrP(Sc) seed in human brain and CSF. In contrast to other in vitro misfolded protein amplification assays-such as protein misfolding cyclic amplification (PMCA)-which are based on sonication, the RT-QuIC technique is based on prion seed-induced misfolding and aggregation of recombinant prion protein substrate, accelerated by alternating cycles of shaking and rest in fluorescence plate readers...
November 2016: Nature Protocols
https://www.readbyqxmd.com/read/27733649/bioassay-of-prion-infected-blood-plasma-in-prp-transgenic-drosophila
#16
Alana M Thackray, Olivier Andreoletti, Raymond Bujdoso
In pursuit of a tractable bioassay to assess blood prion infectivity we have generated PrP transgenic Drosophila , which show a neurotoxic phenotype in adulthood after exposure to exogenous prions at the larval stage. Here we determined the sensitivity of ovine PrP transgenic Drosophila to ovine prion infectivity by exposure of these flies to a dilution series of scrapie-infected sheep brain homogenate. Ovine PrP transgenic Drosophila showed a significant neurotoxic response to dilutions of 10-2 through to 10-10 of the original scrapie-infected sheep brain homogenate...
October 12, 2016: Biochemical Journal
https://www.readbyqxmd.com/read/27716411/genetic-histochemical-and-biochemical-studies-on-goat-tse-cases-from-cyprus
#17
Susanne Niedermeyer, Martin Eiden, Pavlos Toumazos, Penelope Papasavva-Stylianou, Ioannis Ioannou, Theodoros Sklaviadis, Cynthia Panagiotidis, Jan Langeveld, Alex Bossers, Thorsten Kuczius, Martin Kaatz, Martin H Groschup, Christine Fast
Scrapie and bovine spongiform encephalopathy (BSE) are transmissible spongiform encephalopathies (TSE's) affecting sheep and goats. Susceptibility of goats to scrapie is influenced by polymorphisms of the prion protein gene (PRNP) of the host. Five polymorphisms are associated with reduced susceptibility to TSE's. In the study presented here caprine samples from a scrapie eradication program on Cyprus were genotyped and further characterized using BioRad TeSeE rapid test, histological, immunohistochemical and biochemical methods...
October 6, 2016: Veterinary Research
https://www.readbyqxmd.com/read/27685252/inactivation-of-prions-and-amyloid-seeds-with-hypochlorous-acid
#18
Andrew G Hughson, Brent Race, Allison Kraus, Laura R Sangaré, Lori Robins, Bradley R Groveman, Eri Saijo, Katie Phillips, Luis Contreras, Virkamal Dhaliwal, Matteo Manca, Gianluigi Zanusso, Daniel Terry, Jeffrey F Williams, Byron Caughey
Hypochlorous acid (HOCl) is produced naturally by neutrophils and other cells to kill conventional microbes in vivo. Synthetic preparations containing HOCl can also be effective as microbial disinfectants. Here we have tested whether HOCl can also inactivate prions and other self-propagating protein amyloid seeds. Prions are deadly pathogens that are notoriously difficult to inactivate, and standard microbial disinfection protocols are often inadequate. Recommended treatments for prion decontamination include strongly basic (pH ≥~12) sodium hypochlorite bleach, ≥1 N sodium hydroxide, and/or prolonged autoclaving...
September 2016: PLoS Pathogens
https://www.readbyqxmd.com/read/27659200/goat-k222-prp-c-polymorphic-variant-does-not-provide-resistance-to-atypical-scrapie-in-transgenic-mice
#19
Patricia Aguilar-Calvo, Juan-Carlos Espinosa, Olivier Andréoletti, Lorenzo González, Leonor Orge, Ramón Juste, Juan-María Torres
Host prion (PrP(C)) genotype is a major determinant for the susceptibility to prion diseases. The Q/K222-PrP(C) polymorphic variant provides goats and mice with high resistance against classical scrapie and bovine spongiform encephalopathy (BSE); yet its effect against atypical scrapie is unknown. Here, transgenic mice expressing the goat wild-type (wt) or the K222-PrP(C) variant were intracerebrally inoculated with several natural cases of atypical scrapie from sheep and goat and their susceptibility to the prion disease was determined...
2016: Veterinary Research
https://www.readbyqxmd.com/read/27640200/evidence-of-scrapie-transmission-to-sheep-via-goat-milk
#20
Timm Konold, Leigh Thorne, Hugh A Simmons, Steve A C Hawkins, Marion M Simmons, Lorenzo González
BACKGROUND: Previous studies confirmed that classical scrapie can be transmitted via milk in sheep. The current study aimed to investigate whether scrapie can also be transmitted via goat milk using in vivo (new-born lambs fed milk from scrapie-affected goats due to the unavailability of goat kids from guaranteed scrapie-free herds) and in vitro methods (serial protein misfolding cyclic amplification [sPMCA] on milk samples). RESULTS: In an initial pilot study, new-born lambs of two different prion protein gene (PRNP) genotypes (six VRQ/VRQ and five ARQ/ARQ) were orally challenged with 5 g brain homogenate from two scrapie-affected goats to determine susceptibility of sheep to goat scrapie...
September 17, 2016: BMC Veterinary Research
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