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https://www.readbyqxmd.com/read/29147935/nucleotide-and-octapeptide-repeat-variations-of-the-prion-protein-coding-gene-prnp-in-anatolian-murrah-and-crossbred-water-buffaloes
#1
Yalçın Yaman, Cemal Ün
Resistance to bovine spongiform encephalopathy (BSE) that is significantly associated with insertion/deletion (indel) polymorphisms at two loci (putative promoter and intron 1) on the prion protein gene (PRNP) in cattle has been well documented. Studies suggest that the insertion alleles are related to BSE resistance. Until recently, BSE has never been reported in water buffaloes (unlike cattle). Previous studies have demonstrated that the PRNP gene in water buffalo consists mostly of insertion alleles at both loci; nevertheless, whether or not water buffaloes are genetically resistant to BSE and the role of indel polymorphisms in their resistance status is not clear...
November 16, 2017: Tropical Animal Health and Production
https://www.readbyqxmd.com/read/29133563/a-promising-anti-prion-trimethoxychalcone-binds-to-the-globular-domain-of-prp-c-and-changes-its-cellular-location
#2
N C Ferreira, L M Ascari, A G Hughson, G R Cavalheiro, C F Góes, P N Fernandes, J R Hollister, R A da Conceição, D S Silva, A M T Souza, M L C Barbosa, F A Lara, R A P Martins, B Caughey, Y Cordeiro
The search for anti-prion compounds has been encouraged by the fact that transmissible spongiform encephalopathies (TSEs) share molecular mechanisms with more prevalent neurodegenerative pathologies, such as Parkinson's and Alzheimer's diseases. Cellular prion protein conversion into protease-resistant forms (PrP(Res) or PrP(Sc)) is a critical step in the development of TSEs, thus being one of the main targets in the screening for anti-prion compounds. In this work, three trimethoxychalcones (J1, J8, J20) and one oxadiazole (Y17), previously identified in vitro as potential anti-prion compounds, were evaluated through different approaches in order to gain inferences about their mechanism of action...
November 13, 2017: Antimicrobial Agents and Chemotherapy
https://www.readbyqxmd.com/read/29122979/using-an-epidemiological-framework-and-bovine-spongiform-encephalopathy-investigation-questionnaire-to-investigate-suspect-bovine-spongiform-encephalopathy-cases-an-example-from-a-bovine-spongiform-encephalopathy-case-in-ireland-in-2015
#3
Jarlath T O'Connor, Justin P Byrne, Simon J More, Martin Blake, Guy McGrath, Jamie A Tratalos, Maire C Mcelroy, Paul Kiernan, Mary J Canty, Chris O'Brien-Lynch, John M Griffin
In several EU member states, bovine spongiform encephalopathy (BSE) cases have been identified in cattle born after the reinforced ban (BARB cases), for reasons that are not entirely clear. Epidemiological investigation of these cases has proved challenging. The European Food Safety Authority recently recommended the collection of a predefined set of epidemiological data from BSE suspects and confirmed BSE cases to aid future investigations. In this study, we present an epidemiological framework and BSE investigation questionnaire to aid the investigation of suspect BSE cases, and illustrate its application during the investigation of a BSE case in Ireland in 2015...
November 9, 2017: Veterinary Record
https://www.readbyqxmd.com/read/29116375/uncoupling-n-acetylaspartate-from-brain-pathology-implications-for-canavan-disease-gene-therapy
#4
Georg von Jonquieres, Ziggy H T Spencer, Benjamin D Rowlands, Claudia B Klugmann, Andre Bongers, Anne E Harasta, Kristina E Parley, Jennie Cederholm, Orla Teahan, Russell Pickford, Fabien Delerue, Lars M Ittner, Dominik Fröhlich, Catriona A McLean, Anthony S Don, Miriam Schneider, Gary D Housley, Caroline D Rae, Matthias Klugmann
N-Acetylaspartate (NAA) is the second most abundant organic metabolite in the brain, but its physiological significance remains enigmatic. Toxic NAA accumulation appears to be the key factor for neurological decline in Canavan disease-a fatal neurometabolic disorder caused by deficiency in the NAA-degrading enzyme aspartoacylase. To date clinical outcome of gene replacement therapy for this spongiform leukodystrophy has not met expectations. To identify the target tissue and cells for maximum anticipated treatment benefit, we employed comprehensive phenotyping of novel mouse models to assess cell type-specific consequences of NAA depletion or elevation...
November 7, 2017: Acta Neuropathologica
https://www.readbyqxmd.com/read/29104136/nucleic-acid-aptamers-for-neurodegenerative-diseases
#5
REVIEW
Alix Bouvier-Müller, Frédéric Ducongé
The increased incidence of neurodegenerative diseases represents a huge challenge for societies. These diseases are characterized by neuronal death and include several different pathologies, such as Alzheimer's disease, Parkinson's disease, multiple sclerosis, Huntington's disease and transmissible spongiform encephalopathies. Most of these pathologies are often associated with the aggregation of misfolded proteins, such as amyloid-ß, tau, α-synuclein, huntingtin and prion proteins. However, the precise mechanisms that lead to neuronal dysfunction and death in these diseases remain poorly understood...
November 2, 2017: Biochimie
https://www.readbyqxmd.com/read/29098931/ante-mortem-detection-of-chronic-wasting-disease-in-recto-anal-mucosa-associated-lymphoid-tissues-from-elk-cervus-elaphus-nelsoni-using-real-time-quaking-induced-conversion-rt-quic-assay-a-blinded-collaborative-study
#6
Sireesha Manne, Naveen Kondru, Tracy Nichols, Aaron Lehmkuhl, Bruce Thomsen, Rodger Main, Patrick Halbur, Somak Dutta, Anumantha G Kanthasamy
Prion diseases are transmissible spongiform encephalopathies (TSEs) characterized by fatal, progressive neurologic diseases with prolonged incubation periods and an accumulation of infectious misfolded prion proteins. Antemortem diagnosis is often difficult due to a long asymptomatic incubation period, differences in the pathogenesis of different prions, and the presence of very low levels of infectious prion in easily accessible samples. Chronic wasting disease (CWD) is a TSE affecting both wild and captive populations of cervids, including mule deer, white-tailed deer, elk, moose, muntjac, and most recently, wild reindeer...
November 3, 2017: Prion
https://www.readbyqxmd.com/read/29098930/biological-and-biochemical-characterization-of-m2b-cells-classical-bse-prion-is-conserved-in-transgenic-mice-overexpressing-bovine-prion-protein-gene
#7
Tae-Young Suh, In Soon Roh, Hyo-Jin Kim, Peter C Griffiths, Kyung Je Park, Hoo Chang Park, James Hope, Hae Eun Kang, Dae-Yong Kim, Hyun Joo Sohn
M2B cells with persistent classical bovine spongiform encephalopathy (C-BSE) have been established previously. In this study, we performed strain characterization of the M2B cell line in bovine PrP(C) overexpressing mice (Tg 1896). Mice intracranially inoculated with M2B cells and C-BSE survived for 451 ± 7 and 465 ± 31 d post inoculation, respectively. Although biochemical properties, including deglycosylation and conformational stability, differed between M2B cells and C-BSE, inoculation with M2B cell lysate and C-BSE resulted in comparable phenotypes...
November 3, 2017: Prion
https://www.readbyqxmd.com/read/29097653/experimental-transfusion-of-variant-cjd-infected-blood-reveals-previously-uncharacterised-prion-disorder-in-mice-and-macaque
#8
Emmanuel E Comoy, Jacqueline Mikol, Nina Jaffré, Vincent Lebon, Etienne Levavasseur, Nathalie Streichenberger, Chryslain Sumian, Armand Perret-Liaudet, Marc Eloit, Olivier Andreoletti, Stéphane Haïk, Philippe Hantraye, Jean-Philippe Deslys
Exposure of human populations to bovine spongiform encephalopathy through contaminated food has resulted in <250 cases of variant Creutzfeldt-Jakob disease (vCJD). However, more than 99% of vCJD infections could have remained silent suggesting a long-term risk of secondary transmission particularly through blood. Here, we present experimental evidence that transfusion in mice and non-human primates of blood products from symptomatic and non-symptomatic infected donors induces not only vCJD, but also a different class of neurological impairments...
November 2, 2017: Nature Communications
https://www.readbyqxmd.com/read/29069734/mgr1-antigen-37-kda-laminin-receptor-precursor-promotes-cellular-prion-protein-induced-multi-drug-resistance-of-gastric-cancer
#9
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/29049389/endemic-chronic-wasting-disease-causes-mule-deer-population-decline-in-wyoming
#10
Melia T DeVivo, David R Edmunds, Matthew J Kauffman, Brant A Schumaker, Justin Binfet, Terry J Kreeger, Bryan J Richards, Hermann M Schätzl, Todd E Cornish
Chronic wasting disease (CWD) is a fatal transmissible spongiform encephalopathy affecting white-tailed deer (Odocoileus virginianus), mule deer (Odocoileus hemionus), Rocky Mountain elk (Cervus elaphus nelsoni), and moose (Alces alces shirasi) in North America. In southeastern Wyoming average annual CWD prevalence in mule deer exceeds 20% and appears to contribute to regional population declines. We determined the effect of CWD on mule deer demography using age-specific, female-only, CWD transition matrix models to estimate the population growth rate (λ)...
2017: PloS One
https://www.readbyqxmd.com/read/29046443/prion-protein-devoid-of-the-octapeptide-repeat-region-delays-bse-pathogenesis-in-mice
#11
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/29038292/proteolysis-suppresses-spontaneous-prion-generation-in-yeast
#12
Atsushi Okamoto, Nao Hosoda, Anri Tanaka, Gary P Newnam, Yury O Chernoff, Shin-Ichi Hoshino
Prions are infectious proteins that cause fatal neurodegenerative disorders including Creutzfeldt-Jacob and bovine spongiform encephalopathy (mad cow) diseases. The yeast [PSI+] prion is formed by the translation-termination factor Sup35, is the best-studied prion, and provides a useful model system for studying such diseases. However, despite recent progress in the understanding of prion diseases, the cellular defense mechanism against prions has not been elucidated. Here, we report that proteolytic cleavage of Sup35 suppresses spontaneous de novo generation of the [PSI+] prion...
October 16, 2017: Journal of Biological Chemistry
https://www.readbyqxmd.com/read/28992347/common-molecular-pathogenesis-of-disease-related-intrinsically-disordered-proteins-revealed-by-nmr-analysis
#13
Yoshiki Shigemitsu, Hidekazu Hiroaki
Intrinsically disordered proteins (IDPs) are either completely unstructured or contain large disordered regions in their native state; they have drawn much attention in the field of molecular pathology. Some of them substantially tend to form protein self-assemblies, such as toxic or non-toxic aggregates and fibrils, and have been postulated to relate to diseases. These disease-related IDPs include Aβ(1-42) [Alzheimer's disease (AD)], Tau (AD and tauopathy), α-synuclein (Parkinson's disease), and p53 (cancer)...
September 11, 2017: Journal of Biochemistry
https://www.readbyqxmd.com/read/28989689/semisynthetic-prion-protein-prp-variants-carrying-glycan-mimics-at-position-181-and-197-do-not-form-fibrils
#14
Can Araman, Robert E Thompson, Siyao Wang, Stefanie Hackl, Richard J Payne, Christian F W Becker
The prion protein (PrP) is an N-glycosylated protein attached to the outer leaflet of eukaryotic cell membranes via a glycosylphosphatidylinositol (GPI) anchor. Different prion strains have distinct glycosylation patterns and the extent of glycosylation of potentially pathogenic misfolded prion protein (PrP(Sc)) has a major impact on several prion-related diseases (transmissible spongiform encephalopathies, TSEs). Based on these findings it is hypothesized that posttranslational modifications (PTMs) of PrP influence conversion of cellular prion protein (PrP(C)) into PrP(Sc) and, as such, modified PrP variants are critical tools needed to investigate the impact of PTMs on the pathogenesis of TSEs...
September 1, 2017: Chemical Science
https://www.readbyqxmd.com/read/28978705/an-in-vitro-approach-to-identify-the-key-amino-acids-in-the-low-susceptibility-of-rabbit-prp-to-misfolding
#15
Hasier Eraña, Natalia Fernández-Borges, Saioa R Elezgarai, Chafik Harrathi, Jorge M Charco, Francesca Chianini, Mark P Dagleish, Gabriel Ortega, Óscar Millet, Joaquín Castilla
Prion diseases or transmissible spongiform encephalopathies (TSEs) are a group of rare progressive neurodegenerative disorders caused by an abnormally folded prion protein (PrP(Sc)). This is capable of transforming the normal cellular prion protein (PrP(C)) in to new infectious PrP(Sc) Interspecies prion transmissibility studies performed by experimental challenge and the outbreak of bovine spongiform encephalopathy that occurred in the late 1980s-1990's showed that while some species were readily susceptible to TSEs (sheep, mice and cats), others were apparently resistant (rabbits, dogs and horses) when exposed to the same agent...
October 4, 2017: Journal of Virology
https://www.readbyqxmd.com/read/28961066/toward-therapy-of-human-prion-diseases
#16
Adriano Aguzzi, Asvin K K Lakkaraju, Karl Frontzek
Three decades after the discovery of prions as the cause of Creutzfeldt-Jakob disease and other transmissible spongiform encephalopathies, we are still nowhere close to finding an effective therapy. Numerous pharmacological interventions have attempted to target various stages of disease progression, yet none has significantly ameliorated the course of disease. We still lack a mechanistic understanding of how the prions damage the brain, and this situation results in a dearth of validated pharmacological targets...
September 27, 2017: Annual Review of Pharmacology and Toxicology
https://www.readbyqxmd.com/read/28936596/age-related-behavioral-morphological-and-physiological-changes-in-the-hippocampus-of-zitter-rats
#17
Ayuka Ehara, Masao Maekawa, Yuuichi Hori, Kazuhiko Nakadate, Shiuchi Ueda
Convulsive seizure is known to be associated with hippocampal abnormalities, such as hilar cell degeneration, abnormal mossy fiber sprouting in the dentate gyrus (DG) and abnormal expression of immediate early genes. However, whether these morphological changes are a cause or consequence of convulsive seizures has remained contentious. Zitter (zi/zi) rats carry a mutation of the attractin gene and display spongiform degeneration of the brain. Spontaneous convulsive seizures in zi/zi rats over 8 months (M) old were demonstrated using 24-h video monitoring...
September 21, 2017: Anatomical Science International
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/28926025/animal-health-and-price-transmission-along-livestock-supply-chains
#19
M Aragrande, M Canali
Animal health diseases can severely affect the food supply chain by causing variations in prices and market demand. Price transmission analysis reveals in what ways price variations are transmitted along the supply chain, and how supply chains of substitute products and different regional markets are also affected. In perfect markets, a price variation would be completely and instantaneously transmitted across the different levels of the supply chain: producers, the processing industry, retailers and consumers...
April 2017: Revue Scientifique et Technique
https://www.readbyqxmd.com/read/28922846/different-complicated-brain-pathologies-in-monozygotic-twins-with-gerstmann-str%C3%A3-ussler-scheinker-disease
#20
Hiroyuki Honda, Kensuke Sasaki, Hiroshi Takashima, Daisuke Mori, Sachiko Koyama, Satoshi O Suzuki, Toru Iwaki
Gerstmann-Sträussler-Scheinker disease (GSS) is an autosomal, dominantly inherited prion disease. In this study, we present different complicated brain pathologies determined postmortem of monozygotic GSS twin sisters. Case 1 showed cerebellar ataxia at the age of 58 years, and died at 66 years. Case 2 became symptomatic at the age of 75 years, and died at 79 years. There was a 17-year difference in the age of onset between the twins. Postmortem examination revealed numerous prion protein (PrP) plaques in the brains of both cases...
October 1, 2017: Journal of Neuropathology and Experimental Neurology
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