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https://www.readbyqxmd.com/read/28721847/classical-scrapie-transmission-in-arr-arr-genotype-sheep
#1
Caroline Lacroux, Hervé Cassard, Hugh Simmons, Jean Yves Douet, Fabien Corbière, Severine Lugan, Pierette Costes, Naima Aron, Alvina Huor, Cécile Tillier, Francois Schelcher, Olivier Andreoletti
The ARR allele is considered to provide a very strong resistance against classical scrapie infection in sheep. In this study, we report the occurrence of clinical transmissible spongiform encephalopathy in ARR/ARR sheep, following their inoculation by the intracerebral route with a classical scrapie isolate. On first passage, the disease displayed an incomplete attack rate transmission, with incubation periods exceeding 6 years. On second passage, the obtained prion did not display better abilities to propagate than the original isolate...
July 18, 2017: Journal of General Virology
https://www.readbyqxmd.com/read/28715601/methamphetamine-increases-prion-protein-and-induces-dopamine-dependent-expression-of-protease-resistant-prpsc
#2
M Ferrucci, L Ryskalin, F Biagioni, S Gambardella, C L Busceti, A Falleni, G Lazzeri, F Fornai
The cellular prion protein (PrPc) is physiologically expressed within selective brain areas of mammals. Alterations in the secondary structure of this protein lead to scrapie-like prion protein (PrPsc), which precipitates in the cell. PrPsc has been detected in infectious, inherited or sporadic neurodegenerative disorders. Prion protein metabolism is dependent on autophagy and ubiquitin proteasome. Despite not being fully elucidated, the physiological role of prion protein relates to chaperones which rescue cells under stressful conditions...
July 1, 2017: Archives Italiennes de Biologie
https://www.readbyqxmd.com/read/28714865/microglia-in-prion-diseases
#3
Adriano Aguzzi, Caihong Zhu
Prion diseases are a group of progressive and fatal neurodegenerative disorders characterized by deposition of scrapie prion protein (PrPSc) in the CNS. This deposition is accompanied by neuronal loss, spongiform change, astrogliosis, and conspicuous microglial activation. Here, we argue that microglia play an overall neuroprotective role in prion pathogenesis. Several microglia-related molecules, such as Toll-like receptors (TLRs), the complement system, cytokines, chemokines, inflammatory regulators, and phagocytosis mediators, are involved in prion pathogenesis...
July 17, 2017: Journal of Clinical Investigation
https://www.readbyqxmd.com/read/28691895/low-fraction-of-the-222k-prp-variant-in-the-protease-resistant-moiety-of-prpres-in-heterozygous-scrapie-positive-goats
#4
Maria Mazza, Chiara Guglielmetti, Francesco Ingravalle, Sonia Brusadore, Jan P M Langeveld, Loukia V Ekateriniadou, Olivier Andréoletti, Cristina Casalone, Pier Luigi Acutis
The presence of lysine (K) at codon 222 has been associated with resistance to classical scrapie in goats, but few scrapie cases have been identified in 222Q/K animals. To investigate the contribution of the 222K variant to PrPres formation in natural and experimental Q/K scrapie cases, we applied an immunoblotting method based on the use of two different monoclonal antibodies, F99/97.6.1 and SAF84, chosen for their different affinities to 222K and 222Q PrP variants. Our finding that PrPres seems to be formed nearly totally by the 222Q variant provides evidence that the 222K PrP variant confers resistance to conversion to PrPres formation and reinforces the view that this mutation has a protective role against classical scrapie in goats...
July 10, 2017: Journal of General Virology
https://www.readbyqxmd.com/read/28690540/the-role-of-microglia-in-prion-diseases-a-paradigm-of-functional-diversity
#5
REVIEW
Juliane Obst, Emilie Simon, Renzo Mancuso, Diego Gomez-Nicola
Inflammation is a major component of neurodegenerative diseases. Microglia are the innate immune cells in the central nervous system (CNS). In the healthy brain, microglia contribute to tissue homeostasis and regulation of synaptic plasticity. Under disease conditions, they play a key role in the development and maintenance of the neuroinflammatory response, by showing enhanced proliferation and activation. Prion diseases are progressive chronic neurodegenerative disorders associated with the accumulation of the scrapie prion protein PrP(Sc), a misfolded conformer of the cellular prion protein PrP(C)...
2017: Frontiers in Aging Neuroscience
https://www.readbyqxmd.com/read/28674232/a-cross-sectional-study-of-prnp-gene-in-two-native-sicilian-goat-populations-in-italy-a-relation-between-prion-gene-polymorphisms-and-scrapie-incidence
#6
Sergio Migliore, Stefano Agnello, Salvatore D'Avola, Wilfred Goldmann, Vincenzo Di Marco Lo Presti, Maria Vitale
Transmissible spongiform encephalopathies (TSEs) are a group of neurodegenerative diseases affecting humans and animals, and scrapie in small ruminants is considered the archetype of TSEs. Derivata di Siria is a native dairy goat of Sicily (south Italy), which is related to Syrian goat breeds. Scrapie disease is considered endemic in Sicily since 1997, following the administration of an infected vaccine.Derivata di Siria goatswere involved in six of 66 scrapie-infected flocks in Sicily. Prion protein gene (PRNP) analysis revealed that none of the scrapie cases carried the p...
June 2017: Journal of Genetics
https://www.readbyqxmd.com/read/28674229/prion-protein-gene-polymorphisms-in-turkish-native-goat-breeds
#7
Hasan Meydan, Erkan Pehlivan, Mustafa Muhip Özkan, Mehmet Ali Yildiz, Wilfred Goldmann
Susceptibility to 'scrapie' disease in goats is influenced by polymorphisms of the prion protein (PRNP) gene. The aim of this study was to identify PRNP gene polymorphisms in a total of 356 scrapie disease-free goats from 10 Turkish native breeds. Eighteen single-nucleotide polymorphisms were detected in the caprine PRNP open-reading frame. Ten previously described amino acid substitutions (I142M, H143R, N146S, N146D, R151H, R154H, P168Q, R211Q, Q222K and P240S) and two novel dimorphisms (G134E and Q163P) were identified...
June 2017: Journal of Genetics
https://www.readbyqxmd.com/read/28669732/the-retention-of-prion-protein-in-the-endoplasmic-reticulum-prevents-n2a-cells-from-proteasome-inhibition-induced-cytotoxicity
#8
Shuping Fang, Ruixue Wang, Honghao Liu, Weiliang Zhuang, Zhen Wang, Jianjun Zhang, Lili Pei, Yumei Liu, Yunpeng Su
Prion disease is a fatal neurodegenerative disease that may result from the conversion of normal cellular prion protein (PrP(C)) to the pathogenic scrapie PrP isoform (PrP(Sc)), however, how proliferation of prion leads to neuronal apoptosis is still not clear. In this study, to explore the role of the endoplasmic reticulum (ER) in prion diseases, we engineered the KDEL ER-retention motif to the C-terminus of PrP(C) and studied its effect on N2A cell toxicity. The KDEL retention signal led to the accumulation of PrP in the ER, and KDEL signal could effectively deplete PrP from the cell surface and trap PrP in the ER/Cis-Golgi compartment...
June 29, 2017: Biochemical and Biophysical Research Communications
https://www.readbyqxmd.com/read/28665745/ovine-recombinant-prp-as-an-inhibitor-of-ruminant-prion-propagation-in-vitro
#9
Rob G Workman, Ben C Maddison, Kevin C Gough
Prion diseases are fatal and incurable neurodegenerative diseases of humans and animals. Despite years of research, no therapeutic agents have been developed that can effectively manage or reverse disease progression. Recently it has been identified that recombinant prion proteins (rPrP) expressed in bacteria can act as inhibitors of prion replication within the in vitro prion replication system Protein Misfolding Cyclic Amplification (PMCA). Here, within PMCA reactions amplifying a range of ruminant prions including distinct Prnp genotypes/host species and distinct prion strains, recombinant ovine VRQ PrP displayed consistent inhibition of prion replication and produced IC50 values of 122 and 171 nM for ovine scrapie and bovine BSE replication, respectively...
June 30, 2017: Prion
https://www.readbyqxmd.com/read/28636656/selective-propagation-of-mouse-passaged-scrapie-prions-with-long-incubation-period-from-a-mixed-prion-population-using-gt1-7-cells
#10
Kohtaro Miyazawa, Kentaro Masujin, Hiroyuki Okada, Yuko Ushiki-Kaku, Yuichi Matsuura, Takashi Yokoyama
In our previous study, we demonstrated the propagation of mouse-passaged scrapie isolates with long incubation periods (L-type) derived from natural Japanese sheep scrapie cases in murine hypothalamic GT1-7 cells, along with disease-associated prion protein (PrPSc) accumulation. We here analyzed the susceptibility of GT1-7 cells to scrapie prions by exposure to infected mouse brains at different passages, following interspecies transmission. Wild-type mice challenged with a natural sheep scrapie case (Kanagawa) exhibited heterogeneity of transmitted scrapie prions in early passages, and this mixed population converged upon one with a short incubation period (S-type) following subsequent passages...
2017: PloS One
https://www.readbyqxmd.com/read/28619160/protein-misfolding-cyclic-amplification-corroborates-the-absence-of-prp-sc-accumulation-in-placenta-from-foetuses-with-the-arr-arq-genotype-in-natural-scrapie
#11
María Carmen Garza, Hasier Eraña, Joaquín Castilla, Cristina Acín, Antonia Vargas, Juan José Badiola, Eva Monleón
Ovine scrapie is a worldwide spread prion disease that is transmitted horizontally under field conditions. Placenta from scrapie-infected ewes is an important source of infection, since this tissue can accumulate high amounts of PrP(Sc) depending on the foetal genotype. Therefore, placentas carrying susceptible foetuses can accumulate PrP(Sc) but there is not PrP(Sc) accumulation in presence of foetuses with at least one ARR haplotype. In scrapie eradication programs, ARR/ARR males are used for breeding to increase the resistant progeny and reduce the horizontal transmission of the disease through the placenta...
May 2017: Veterinary Microbiology
https://www.readbyqxmd.com/read/28613153/precision-in-the-design-of-an-experimental-study-deflects-the-significance-of-proteinase-activated-receptor-2-expression-in-scrapie-inoculated-mice
#12
Zdenka Hanusova, Tibor Mosko, Radoslav Matej, Karel Holada
Proteinase-activated receptor 2 (PAR2) is suspected to modulate the pathogenesis of various neurodegenerative conditions. We previously described delayed onset of clinical symptoms and prolonged survival of PAR2-deficient mice after intracerebral inoculation with prions. Here we report the results from a refined blinded study that aimed to investigate the effects of PAR2 deletion on scrapie pathogenesis after peripheral infection. This study failed to confirm that PAR2 deficiency impacts on the length of the incubation period, with PAR2-/- and PAR2+/+ littermates developing scrapie at the same time...
June 2017: Journal of General Virology
https://www.readbyqxmd.com/read/28605958/the-first-report-of-prion-related-protein-gene-prnt-polymorphisms-in-goat
#13
Yong-Chan Kim, Byung-Hoon Jeong
Prion protein is encoded by the prion protein gene (PRNP). Polymorphisms of several members of the prion gene family have shown association with prion diseases in several species. Recent studies on a novel member of the prion gene family in rams have shown that prion-related protein gene (PRNT) has a linkage with codon 26 of prion-like protein (PRND). In a previous study, codon 26 polymorphism of PRND has shown connection with PRNP haplotype which is strongly associated with scrapie vulnerability. In addition, the genotype of a single nucleotide polymorphism (SNP) at codon 26 of PRND is related to fertilisation capacity...
June 2017: Acta Veterinaria Hungarica
https://www.readbyqxmd.com/read/28596963/prion-diagnosis-application-of-real-time-quaking-induced-conversion
#14
REVIEW
Hae-Eun Kang, Youngwon Mo, Raihah Abd Rahim, Hye-Mi Lee, Chongsuk Ryou
Prions composed of pathogenic scrapie prion protein (PrP(Sc)) are infectious pathogens that cause progressive neurological conditions known as prion diseases or transmissible spongiform encephalopathies. Although these diseases pose considerable risk to public health, procedures for early diagnosis have not been established. One of the most recent attempts at sensitive and specific detection of prions is the real-time quaking-induced conversion (RT-QuIC) method, which measures the activity of PrP(Sc) aggregates or amyloid formation triggered by PrP(Sc) seeds in the presence of recombinant PrP...
2017: BioMed Research International
https://www.readbyqxmd.com/read/28549342/mgr1-antigen-37-kda-laminin-receptor-precursor-promotes-cellular-prion-protein-induced-multi-drug-resistance-of-gastric-cancer
#15
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 (PrPC), the infective agent of transmissible spongiform encephalopathies, is thought to be related to several cellular physiological and physiopathological processes. We have previously reported that PrPC 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 (PrPSc), 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...
May 11, 2017: Oncotarget
https://www.readbyqxmd.com/read/28545141/prion-pathogenesis-is-unaltered-in-the-absence-of-sirp%C3%AE-mediated-don-t-eat-me-signaling
#16
Mario Nuvolone, Marta Paolucci, Silvia Sorce, Veronika Kana, Rita Moos, Takashi Matozaki, Adriano Aguzzi
Prion diseases are neurodegenerative conditions caused by misfolding of the prion protein, leading to conspicuous neuronal loss and intense microgliosis. Recent experimental evidence point towards a protective role of microglia against prion-induced neurodegeneration, possibly through elimination of prion-containing apoptotic bodies. The molecular mechanisms by which microglia recognize and eliminate apoptotic cells in the context of prion diseases are poorly defined. Here we investigated the possible involvement of signal regulatory protein α (SIRPα), a key modulator of host cell phagocytosis; SIRPα is encoded by the Sirpa gene that is genetically linked to the prion gene Prnp...
2017: PloS One
https://www.readbyqxmd.com/read/28540665/different-molecular-mechanisms-mediate-direct-or-glia-dependent-prion-protein-fragment-90-231-neurotoxic-effects-in-cerebellar-granule-neurons
#17
Stefano Thellung, Elena Gatta, Francesca Pellistri, Valentina Villa, Alessandro Corsaro, Mario Nizzari, Mauro Robello, Tullio Florio
Glia over-stimulation associates with amyloid deposition contributing to the progression of central nervous system neurodegenerative disorders. Here we analyze the molecular mechanisms mediating microglia-dependent neurotoxicity induced by prion protein (PrP)90-231, an amyloidogenic polypeptide corresponding to the protease-resistant portion of the pathological prion protein scrapie (PrP(Sc)). PrP90-231 neurotoxicity is enhanced by the presence of microglia within neuronal culture, and associated to a rapid neuronal [Ca(++)] i increase...
May 25, 2017: Neurotoxicity Research
https://www.readbyqxmd.com/read/28507517/the-role-of-unfolded-protein-response-and-mitogen-activated-protein-kinase-signaling-in-neurodegenerative-diseases-with-special-focus-on-prion-diseases
#18
REVIEW
Syed Zahid Ali Shah, Deming Zhao, Tariq Hussain, Lifeng Yang
Prion diseases are neurodegenerative pathologies characterized by the accumulation of a protease-resistant form of the cellular prion protein named prion protein scrapie (PrP(Sc)) in the brain. PrP(Sc) accumulation in the endoplasmic reticulum (ER) result in a dysregulated calcium (Ca(2+)) homeostasis and subsequent initiation of unfolded protein response (UPR) leading to neuronal dysfunction and apoptosis. The molecular mechanisms for the transition between adaptation to ER stress and ER stress-induced apoptosis are still unclear...
2017: Frontiers in Aging Neuroscience
https://www.readbyqxmd.com/read/28472956/codon-141-polymorphisms-of-the-ovine-prion-protein-gene-affect-the-phenotype-of-classical-scrapie-transmitted-from-goats-to-sheep
#19
Timm Konold, Laura J Phelan, Ben R Donnachie, Melanie J Chaplin, Saira Cawthraw, Lorenzo González
BACKGROUND: A study to investigate transmission of classical scrapie via goat milk was carried out in sheep: firstly, lambs were challenged orally with goat scrapie brain homogenate to confirm transmission of scrapie from goats to sheep. In the second study phase, milk from scrapie-infected goats was fed to lambs. Lambs were selected according to their prion protein gene (PRNP) genotype, which was either VRQ/VRQ or ARQ/ARQ, with or without additional polymorphisms at codon 141 (FF141, LF141 or LL141) of the ovine PRNP...
May 4, 2017: BMC Veterinary Research
https://www.readbyqxmd.com/read/28470584/myelin-basic-protein-citrullination-a-hallmark-of-central-nervous-system-demyelination-assessed-by-novel-monoclonal-antibodies-in-prion-diseases
#20
Byungki Jang, Yong-Chul Jeon, Hae-Young Shin, Yun-Jung Lee, Hyunji Kim, Yoshitaka Kondo, Akihito Ishigami, Yong-Sun Kim, Eun-Kyoung Choi
Myelin basic protein (MBP) citrullination by peptidylarginine deiminase (PAD) enzymes leads to incomplete protein-lipid bilayer interactions and vulnerability to proteolytic enzymes, resulting in disorganization of the myelin sheath in the central nervous system. Therefore, citrullinated MBP (citMBP) has been suggested as a hallmark of demyelination, but how citMBP is implicated in prion diseases remains unknown. For the first time, we developed mouse monoclonal anti-citMBP IgG1 (clones 1B8, 1H1, and 3C6) and IgM (clone 3G5) antibodies that recognize human citMBP at its R25, R122, and R130 residues and at its C-terminal region (or the corresponding sites in mouse MBP), respectively...
May 3, 2017: Molecular Neurobiology
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