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https://www.readbyqxmd.com/read/29330304/efficient-prion-disease-transmission-through-common-environmental-materials
#1
Sandra Pritzkow, Rodrigo Morales, Adam Lyon, Luis Concha-Marambio, Akihiko Urayama, Claudio Soto
Prion diseases are a group of fatal neurodegenerative diseases associated with a protein-based infectious agent, termed prion. Compelling evidence suggests that natural transmission of prion diseases is mediated by environmental contamination with infectious prions. We hypothesized that several natural and man-made materials, commonly found in the environments of wild and captive animals, can bind prions and may act as vectors for disease transmission. To test our hypothesis, we exposed surfaces composed of various common environmental materials (i...
January 12, 2018: Journal of Biological Chemistry
https://www.readbyqxmd.com/read/29310723/iatrogenic-creutzfeldt-jakob-disease-with-amyloid-%C3%AE-pathology-an-international-study
#2
Ignazio Cali, Mark L Cohen, Stéphane Haїk, Piero Parchi, Giorgio Giaccone, Steven J Collins, Diane Kofskey, Han Wang, Catriona A McLean, Jean-Philippe Brandel, Nicolas Privat, Véronique Sazdovitch, Charles Duyckaerts, Tetsuyuki Kitamoto, Ermias D Belay, Ryan A Maddox, Fabrizio Tagliavini, Maurizio Pocchiari, Ellen Leschek, Brian S Appleby, Jiri G Safar, Lawrence B Schonberger, Pierluigi Gambetti
The presence of pathology related to the deposition of amyloid-β (Aβ) has been recently reported in iatrogenic Creutzfeldt-Jakob disease (iCJD) acquired from inoculation of growth hormone (GH) extracted from human cadaveric pituitary gland or use of cadaveric dura mater (DM) grafts.To investigate this phenomenon further, a cohort of 27 iCJD cases - 21 with adequate number of histopathological sections - originating from Australia, France, Italy, and the Unites States, were examined by immunohistochemistry, amyloid staining, and Western blot analysis of the scrapie prion protein (PrPSc), and compared with age-group matched cases of sporadic CJD (sCJD), Alzheimer disease (AD) or free of neurodegenerative diseases (non-ND)...
January 8, 2018: Acta Neuropathologica Communications
https://www.readbyqxmd.com/read/29310497/differential-effects-of-divalent-cations-on-elk-prion-protein-fibril-formation-and-stability
#3
Daniel Samorodnitsky, Eric M Nicholson
Misfolding of the normally folded prion protein of mammals (PrPC) into infectious fibrils causes a variety of diseases, from scrapie in sheep to chronic wasting disease (CWD) in cervids. The misfolded form of PrPC, termed PrPSc, or in this case PrPCWD, interacts with PrPC to create more PrPCWD. This process is not clearly defined but is affected by the presence and interactions of biotic and abiotic cofactors. These include nucleic acids, lipids, glycosylation, pH, and ionic character. PrPC has been shown to act as a copper-binding protein in vivo, though it also binds to other divalents as well...
January 9, 2018: Prion
https://www.readbyqxmd.com/read/29310343/cerebrospinal-fluid-real-time-quaking-induced-conversion-test-for-sporadic-creutzfeldt-jakob-disease-in-an-18-year-old-woman-a-case-report
#4
Yuan Yao, Xiaoping Dong, Hongzhi Guan, Qiang Lu
RATIONALE: Sporadic Creutzfeldt-Jakob disease (sCJD) mainly occurs in the elderly, with the peak age of onset ranging from 55 to 75 years. The symptoms of sCJD are not unique, and laboratory tests such as magnetic resonance imaging (MRI), electroencephalogram (EEG) and cerebrospinal fluid (CSF)14-3-3 protein have low sensitivity or specificity. Therefore, excluding treatable diseases and establishing a diagnosis could be difficult in young patients with suspected sCJD. Recently, real-time quaking-induced conversion (RT-QuIC) has been used in the diagnosis of sCJD, with more than 95% sensitivity and 100% specificity...
December 2017: Medicine (Baltimore)
https://www.readbyqxmd.com/read/29240410/determining-the-relative-susceptibility-of-four-prp-genotypes-to-atypical-scrapie
#5
Christopher J Silva, Melissa L Erickson-Beltran, Inmaculada Martin-Burriel, Juan José Badiola, Jesus Rodriguez Requena, Rosa Bolea
Atypical scrapie is a sheep prion (PrPSc) disease whose epidemiology is consistent with a sporadic origin and is associated with specific polymorphisms of the normal cellular prion protein (PrPC). To determine the relative amounts of PrP polymorphisms present in scrapie, total PrP was digested with chymotrypsin to generate characteristic peptides spanning relevant polymorphisms at positions 136, 141, 154, 171 and 172 of sheep PrPC. A multiple reaction monitoring method (MRM), employing 15N-labeled internal standards, was used to detect and quantify these polymorphisms present in both the PrPSc and PrPC from heterozygous (ALRRY and ALHQY or ALRQD or AFRQY) atypical scrapie-infected or uninfected control sheep...
December 14, 2017: Analytical Chemistry
https://www.readbyqxmd.com/read/29220360/substitutions-of-prp-n-terminal-histidine-residues-modulate-scrapie-disease-pathogenesis-and-incubation-time-in-transgenic-mice
#6
Sabina Eigenbrod, Petra Frick, Uwe Bertsch, Gerda Mitteregger-Kretzschmar, Janina Mielke, Marko Maringer, Niklas Piening, Alexander Hepp, Nathalie Daude, Otto Windl, Johannes Levin, Armin Giese, Vignesh Sakthivelu, Jörg Tatzelt, Hans Kretzschmar, David Westaway
Prion diseases have been linked to impaired copper homeostasis and copper induced-oxidative damage to the brain. Divalent metal ions, such as Cu2+ and Zn2+, bind to cellular prion protein (PrPC) at octapeptide repeat (OR) and non-OR sites within the N-terminal half of the protein but information on the impact of such binding on conversion to the misfolded isoform often derives from studies using either OR and non-OR peptides or bacterially-expressed recombinant PrP. Here we created new transgenic mouse lines expressing PrP with disrupted copper binding sites within all four histidine-containing OR's (sites 1-4, H60G, H68G, H76G, H84G, "TetraH>G" allele) or at site 5 (composed of residues His-95 and His-110; "H95G" allele) and monitored the formation of misfolded PrP in vivo...
2017: PloS One
https://www.readbyqxmd.com/read/29202042/relative-impact-of-complement-receptors-cd21-35-cr2-1-on-scrapie-pathogenesis-in-mice
#7
Sarah J Kane, Eric Swanson, Elizabeth O Gordon, Savannah Rocha, Heather R Bender, Luke R Donius, Adriano Aguzzi, Jonathan P Hannan, Mark D Zabel
Complement receptors 1 and 2 (CR1/2 or CD35/CD21) recognize complement-opsonized antigens to initiate innate and adaptive immunity, respectively. CD35 stimulates phagocytosis on macrophages and antigen presentation on follicular dendritic cells (FDCs). CD21 helps activate B cells as part of the B cell coreceptor with CD19 and CD81. Differential splicing of transcripts from the mouse Cr2 gene generates isoforms with both shared and unique complement binding capacities and cell-type expression. In mouse models, genetic depletion of Cr2 causes either a delay or complete prevention of prion disease, but the relative importance of CD35 versus CD21 in promoting prion disease remains unknown...
November 2017: MSphere
https://www.readbyqxmd.com/read/29161325/the-mechanisms-of-humic-substances-self-assembly-with-biological-molecules-the-case-study-of-the-prion-protein
#8
Gabriele Giachin, Ridvan Nepravishta, Walter Mandaliti, Sonia Melino, Alja Margon, Denis Scaini, Pierluigi Mazzei, Alessandro Piccolo, Giuseppe Legname, Maurizio Paci, Liviana Leita
Humic substances (HS) are the largest constituent of soil organic matter and are considered as a key component of the terrestrial ecosystem. HS may facilitate the transport of organic and inorganic molecules, as well as the sorption interactions with environmentally relevant proteins such as prions. Prions enter the environment through shedding from live hosts, facilitating a sustained incidence of animal prion diseases such as Chronic Wasting Disease and scrapie in cervid and ovine populations, respectively...
2017: PloS One
https://www.readbyqxmd.com/read/29157304/a-novel-vector-for-transgenesis-in-the-rat-cns
#9
T Peter Lopez, Kurt Giles, Brittany N Dugger, Abby Oehler, Carlo Condello, Zuzana Krejciova, Julian A Castaneda, George A Carlson, Stanley B Prusiner
The larger brain of the rat enables a much greater repertoire of complex behaviors than mice, likely making rats preferential for investigating neurodegeneration. Because molecular tools for specific expression of transgenes in the rat brain are sparse, we chose Prnp encoding the prion protein (PrP) to develop a novel vector to drive transgene expression in the rat brain. We compared the rat Prnp sequence with mouse and Syrian hamster Prnp sequences, identifying conserved genetic elements and hypothesizing that these elements would be able to drive neuronal transgene expression...
November 21, 2017: Acta Neuropathologica Communications
https://www.readbyqxmd.com/read/29155968/novel-spiroplasma-spp-cultured-from-brains-and-lymph-nodes-from-ruminants-affected-with-transmissible-spongiform-encephalopathy
#10
Frank O Bastian, James Lynch, Sue Hagius, Xiaochu Wu, Greg McCormick, Donald G Luther, Philip H Elzer
Spiroplasma spp., tiny filterable wall-less bacteria, are consistently associated with the transmissible spongiform encephalopathies (TSE). Spiral forms have been transiently isolated from TSE-affected brain tissues in SP4 growth media designed for isolation of Spiroplasma spp., but the isolate could not be propagated in SP4 media. A bacterium must grow in vitro in cell-free cultures to allow full characterization of a suspect pathogen. Here, a novel Spiroplasma sp. was isolated from scrapie- and chronic wasting disease (CWD)-affected brains and lymph nodes...
November 15, 2017: Journal of Neuropathology and Experimental Neurology
https://www.readbyqxmd.com/read/29107182/identification-of-the-internal-ribosome-entry-sites-ires-of-prion-protein-gene
#11
Xiao-Nuan Luo, Qin-Qin Song, Jie Yu, Juan Song, Xin-Ling Wang, Dong Xia, Peng Sun, Jun Han
Many studies demonstrated that there are several type bands of prion protein in cells. However, the formation of different prion protein bands is elusive. After several low molecular weight bands of prion protein appeared in SMB-S15 cells infected with scrapie agent Chandler, we think that IRES-dependent translation mechanism induced by prion is involved in the formation of prion protein bands. Then we designed a series of pPrP-GFP fusing plasmids and bicistronic plasmids to identify the IRES sites of prion protein gene and found 3 IRES sites inside of PrP mRNA...
October 26, 2017: International Journal of Biochemistry & Cell Biology
https://www.readbyqxmd.com/read/29069734/mgr1-antigen-37-kda-laminin-receptor-precursor-promotes-cellular-prion-protein-induced-multi-drug-resistance-of-gastric-cancer
#12
Guanhong Luo, Weijie Wang, Qiong Wu, Yuanyuan Lu, Tao Su, Nan Gu, Kai Li, Jingbo Wang, Rui Du, Xiaodi Zhao, Xiaohua Li, Rui Fan, Hongbo Zhang, Yongzhan Nie, Xinmin Zhou, Yongquan Shi, Jie Liang, Xin Wang, Daiming Fan
Cellular prion protein (PrP(C)), the infective agent of transmissible spongiform encephalopathies, is thought to be related to several cellular physiological and physiopathological processes. We have previously reported that PrP(C) participates in multi-drug-resistance of gastric cancer. As the salient ligand molecule of PrP for participating in internalization and propagation of the scrapie form of prion protein (PrP(Sc)), 37 kDa laminin receptor precursor protein (37LRP) shared the same gene coding sequence of MGr1-Ag, another protein previously found to be involved in multi-drug-resistance of gastric cancer in our lab...
September 22, 2017: Oncotarget
https://www.readbyqxmd.com/read/29058651/estimating-chronic-wasting-disease-susceptibility-in-cervids-using-real-time-quaking-induced-conversion
#13
Nicholas J Haley, Rachel Rielinger, Kristen A Davenport, Katherine O'Rourke, Gordon Mitchell, Jürgen A Richt
In mammals, susceptibility to prion infection is primarily modulated by the host's cellular prion protein (PrP(C)) sequence. In the sheep scrapie model, a graded scale of susceptibility has been established both in vivo and in vitro based on PrP(C) amino acids 136, 154 and 171, leading to global breeding programmes to reduce the prevalence of scrapie in sheep. Chronic wasting disease (CWD) resistance in cervids is often characterized as decreased prevalence and/or protracted disease progression in individuals with specific alleles; at present, no PrP(C) allele conferring absolute resistance in cervids has been identified...
November 2017: Journal of General Virology
https://www.readbyqxmd.com/read/29046443/prion-protein-devoid-of-the-octapeptide-repeat-region-delays-bse-pathogenesis-in-mice
#14
Hideyuki Hara, Hironori Miyata, Nandita Rani Das, Junji Chida, Tatenobu Yoshimochi, Keiji Uchiyama, Hitomi Watanabe, Gen Kondoh, Takashi Yokoyama, Suehiro Sakaguchi
Conformational conversion of the cellular isoform of prion protein PrP(C), into the abnormally folded, amyloidogenic isoform, PrP(Sc), is a key pathogenic event in prion diseases including Creutzfeldt-Jakob disease in humans and scrapie and bovine spongiform encephalopathy (BSE) in animals. We previously reported that the octapeptide repeat (OR) region could be dispensable for converting PrP(C) into PrP(Sc) after infection with RML prions. We demonstrated that mice transgenically expressing mouse PrP with deletion of the OR region on the PrP-knockout background, designated Tg(PrPΔOR)/Prnp(0/0) mice, did not reduce susceptibility to RML scrapie prions, with abundant accumulation of PrP(Sc)ΔOR in their brains...
October 18, 2017: Journal of Virology
https://www.readbyqxmd.com/read/28968141/aberrant-alterations-of-the-expressions-and-s-nitrosylation-of-calmodulin-and-the-downstream-factors-in-the-brains-of-the-rodents-during-scrapie-infection
#15
Ren-Qing Zhang, Cao Chen, Li-Jie Xiao, Jing Sun, Yue Ma, Xiao-Dong Yang, Xiao-Feng Xu, Kang Xiao, Qi Shi, Zhi-Bao Chen, Xiao-Ping Dong
The aberrant alterations of calmodulin (CaM) and its downstream substrates have been reported in some neurodegenerative diseases, but rarely described in prion disease. In this study, the potential changes of Ca(2+)/CaM and its associated agents in the brains of scrapie agent 263K-infected hamsters and the prion infected cell line SMB-S15 were evaluated by various methodologies. We found that the level of CaM in the brains of 263K-infected hamsters started to increase at early stage and maintained at high level till terminal stage...
September 3, 2017: Prion
https://www.readbyqxmd.com/read/28956708/remarkable-increases-of-%C3%AE-1-antichymotrypsin-in-brain-tissues-of-rodents-during-prion-infection
#16
Cao Chen, Xiao-Feng Xu, Ren-Qing Zhang, Yue Ma, Yan Lv, Jian-Le Li, Qiang Shi, Kang Xiao, Jing Sun, Xiao-Dong Yang, Qi Shi, Xiao-Ping Dong
α1-Antichymotrypsin (α1-ACT) belongs to a kind of acute-phase inflammatory protein. Recently, such protein has been proved exist in the amyloid deposits which is the hallmark of Alzheimer's disease, but limitedly reported in prion disease. To estimate the change of α1-ACT during prion infection, the levels of α1-ACT in the brain tissues of scrapie agents 263K-, 139A- and ME7-infected rodents were analyzed, respectively. Results shown that α1-ACT levels were significantly increased in the brain tissues of the three kinds of scrapie-infected rodents, displaying a time-dependent manner during prion infection...
September 3, 2017: Prion
https://www.readbyqxmd.com/read/28942337/a-novel-approach-for-scrapie-associated-prion-prp-sc-detection-in-blood-using-the-competitive-affinity-of-an-aggregate-specific-antibody-and-streptavidin-to-prp-sc
#17
Andrei Soutyrine, Hongsheng Huang, Olga Andrievskaia, Ines Walther, Gordon Mitchell
Scrapie is a fatal neurodegenerative disorder affecting sheep and goats, originating from exposure to disease-associated prions (PrP(Sc)). An ante-mortem screening test that can detect native PrP(Sc) in body fluids remains unavailable due to insufficient sensitivity of current detection methods that involve proteinase or denaturation treatments. We adopted an approach to detect PrP(Sc) in whole blood using a simple proteinase- and denaturation-independent immunoassay, based on the competitive affinity of an aggregate-specific monoclonal antibody and streptavidin to PrP(Sc)...
September 9, 2017: Research in Veterinary Science
https://www.readbyqxmd.com/read/28927447/protecting-effect-of-prp-codons-m142-and-k222-in-goats-orally-challenged-with-bovine-spongiform-encephalopathy-prions
#18
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/28920852/experimental-transmission-to-a-calf-of-an-isolate-of-spanish-classical-scrapie
#19
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/28910420/role-of-the-central-lysine-cluster-and-scrapie-templating-in-the-transmissibility-of-synthetic-prion-protein-aggregates
#20
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 2017: PLoS Pathogens
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