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Variant Creutzfeldt Jakob Disease and Bovine Spongiform Encephalopathy

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Chronic wasting disease associated with prion protein gene (PRNP) variation in Norwegian wild reindeer (Rangifer tarandus).

Mariella E Güere, Jørn Våge, Helene Tharaldsen, Sylvie L Benestad, Turid Vikøren, Knut Madslien, Petter Hopp, Christer M Rolandsen, Knut H Røed, Michael A Tranulis,

The emergence of CWD in Europe in 2016 and the first natural infection in wild reindeer warranted disease management. This led to the testing of 2424 hunted or culled reindeer during 2016-2018, from the infected subpopulation in the Nordfjella mountain range in Southern Norway. To identify any association between PRNP ... Read more >>

Prion (Prion)
[2020, 14(1):1-10]

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Predicting the spread-risk potential of chronic wasting disease to sympatric ungulate species.

Catherine I Cullingham, Rhiannon M Peery, Anh Dao, Debbie I McKenzie, David W Coltman,

Wildlife disease incidence is increasing, resulting in negative impacts on the economy, biodiversity, and potentially human health. Chronic wasting disease (CWD) is a fatal, transmissible spongiform encephalopathy of cervids (wild and captive) which continues to spread geographically resulting in exposure to potential new host species. The disease agent (PrPCWD) is ... Read more >>

Prion (Prion)
[2020, 14(1):56-66]

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Cerebrospinal fluid neurogranin as a new player in prion disease diagnosis and prognosis.

Anna Villar-Pique, Inga Zerr, Franc Llorens,

Neural Regen Res (Neural regeneration research)
[2020, 15(5):861-862]

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PMCA Applications for Prion Detection in Peripheral Tissues of Patients with Variant Creutzfeldt-Jakob Disease.

Giorgio Giaccone, Fabio Moda,

Prion diseases are neurodegenerative and invariably fatal conditions that affect humans and animals. In particular, Creutzfeldt-Jakob disease (CJD) and bovine spongiform encephalopathy (BSE) are paradigmatic forms of human and animal prion diseases, respectively. Human exposure to BSE through contaminated food caused the appearance of the new variant form of CJD ... Read more >>

Biomolecules (Biomolecules)
[2020, 10(3):]

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Immunotherapy against Prion Disease.

Yue Ma, Jiyan Ma,

The term "prion disease" encompasses a group of neurodegenerative diseases affecting both humans and animals. Currently, there is no effective therapy and all forms of prion disease are invariably fatal. Because of (a) the outbreak of bovine spongiform encephalopathy in cattle and variant Creutzfeldt-Jakob disease in humans; (b) the heated ... Read more >>

Pathogens (Pathogens (Basel, Switzerland))
[2020, 9(3):]

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Assessment of risk of variant creutzfeldt-Jakob disease (vCJD) from use of bovine heparin.

Yin Huang, Richard A Forshee, David Keire, Sau Lee, Luisa Gregori, David M Asher, Cyrus Bett, Brian Niland, Scott A Brubaker, Steven A Anderson, Hong Yang,

PURPOSE:In the late1990s, reacting to the outbreak of bovine spongiform encephalopathy (BSE) in the United Kingdom that caused a new variant of Creutzfeldt-Jakob disease (vCJD) in humans, manufacturers withdrew bovine heparin from the market in the United States. There have been growing concerns about the adequate supply and safety of ... Read more >>

Pharmacoepidemiol Drug Saf (Pharmacoepidemiology and drug safety)
[2020, :]

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A probable role of copper in the comorbidity in Wilson's and Creutzfeldt-Jakob's Diseases: a case report.

Effrosyni Koutsouraki, Dimitrios Michmizos, Olga Patsi, John Tzartos, Martha Spilioti, Marianthi Arnaoutoglou, Magda Tsolaki,

BACKGROUND:To the best of our knowledgedd, there is currently no case in the literature reporting the comorbidity of Wilson's and Creutzfeldt-Jakob disease (CJD), linked through copper. CASE PRESENTATION:A 44-year-old male with a history of inherited Wilson's disease (hepatolenticular degeneration), which manifested as mild liver injury and psychiatric symptoms, was admitted ... Read more >>

Virol. J. (Virology journal)
[2020, 17(1):35]

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Correlation between Bioassay and Protein Misfolding Cyclic Amplification for Variant Creutzfeldt-Jakob Disease Decontamination Studies.

Maxime Bélondrade, Christelle Jas-Duval, Simon Nicot, Lilian Bruyère-Ostells, Charly Mayran, Laetitia Herzog, Fabienne Reine, Juan Maria Torres, Chantal Fournier-Wirth, Vincent Béringue, Sylvain Lehmann, Daisy Bougard,

To date, approximately 500 iatrogenic Creutzfeldt-Jakob disease cases have been reported worldwide, most of them resulting from cadaveric dura mater graft and from the administration of prion-contaminated human growth hormone. The unusual resistance of prions to decontamination processes, their large tissue distribution, and the uncertainty about the prevalence of variant ... Read more >>

mSphere (mSphere)
[2020, 5(1):]

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Cardiomyopathy associated with Ceutzfeld-Jakob disease: a diagnosis of exclusion: a case report.

Osnat Itzhaki Ben Zadok, Katia Orvin, Edna Inbar, Eldad Rechavia,

Background:Creutzfeldt-Jakob disease (CJD), the most common prion disease in humans, is primarily known for its adverse neurological impact and inevitable mortality. Data regarding myocardial involvement in CJD are scarce. Case summary:A 54-year-old female patient, presented with progressive effort dyspnoea, was diagnosed with unexplained non-ischaemic cardiomyopathy. An extensive cardiac work-up including ... Read more >>

Eur Heart J Case Rep (European heart journal. Case reports)
[2020, 4(1):1-5]

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Porcine Prion Protein as a Paradigm of Limited Susceptibility to Prion Strain Propagation.

Juan Carlos Espinosa, Alba Marín-Moreno, Patricia Aguilar-Calvo, Sylvie L Benestad, Olivier Andreoletti, Juan María Torres,

Although experimental transmission of bovine spongiform encephalopathy (BSE) to pigs and transgenic mice expressing pig cellular prion protein (PrPC) (porcine PrP [PoPrP]-Tg001) has been described, no natural cases of prion diseases in pig were reported. This study analyzed pig-PrPC susceptibility to different prion strains using PoPrP-Tg001 mice either as animal ... Read more >>

J. Infect. Dis. (The Journal of infectious diseases)
[2020, :]

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Preclinical Detection of Prions in Blood of Nonhuman Primates Infected with Variant Creutzfeldt-Jakob Disease.

Luis Concha-Marambio, Marcelo A Chacon, Claudio Soto,

Variant Creutzfeldt-Jakob disease (vCJD) is caused by prion infection with bovine spongiform encephalopathy and can be transmitted by blood transfusion. Protein misfolding cyclic amplification (PMCA) can detect prions in blood from vCJD patients with 100% sensitivity and specificity. To determine whether PMCA enables prion detection in blood during the preclinical ... Read more >>

Emerging Infect. Dis. (Emerging infectious diseases)
[2020, 26(1):34-43]

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What Are the Molecular Mechanisms by Which Functional Bacterial Amyloids Influence Amyloid Beta Deposition and Neuroinflammation in Neurodegenerative Disorders?

Robert P Friedland, Joseph D McMillan, Zimple Kurlawala,

Despite the enormous literature documenting the importance of amyloid beta (Ab) protein in Alzheimer's disease, we do not know how Ab aggregation is initiated and why it has its unique distribution in the brain. In vivo and in vitro evidence has been developed to suggest that functional microbial amyloid proteins ... Read more >>

Int J Mol Sci (International journal of molecular sciences)
[2020, 21(5):]

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Postmortem Quantitative Analysis of Prion Seeding Activity in the Digestive System.

Katsuya Satoh, Takayuki Fuse, Toshiaki Nonaka, Trong Dong, Masaki Takao, Takehiro Nakagaki, Daisuke Ishibashi, Yuzuru Taguchi, Ban Mihara, Yasushi Iwasaki, Mari Yoshida, Noriyuki Nishida,

Human prion diseases are neurodegenerative disorders caused by prion protein. Although infectivity was historically detected only in the central nervous system and lymphoreticular tissues of patients with sporadic Creutzfeldt-Jakob disease, recent reports suggest that the seeding activity of Creutzfeldt-Jakob disease prions accumulates in various non-neuronal organs including the liver, kidney, ... Read more >>

Molecules (Molecules (Basel, Switzerland))
[2019, 24(24):]

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Neutralizing Mutations Significantly Inhibit Amyloid Formation by Human Prion Protein and Decrease Its Cytotoxicity.

Jun-Jie Huang, Xiang-Ning Li, Wan-Li Liu, Han-Ye Yuan, Yuan Gao, Kan Wang, Bo Tang, Dai-Wen Pang, Jie Chen, Yi Liang,

Prion diseases, such as Creutzfeldt-Jakob disease and bovine spongiform encephalopathy, are fatal neurodegenerative diseases that affect many mammals including humans and are caused by the misfolding of prion protein (PrP). A naturally occurring protective polymorphism G127V in human PrP has recently been found to significantly attenuate prion diseases, but the mechanism ... Read more >>

J. Mol. Biol. (Journal of molecular biology)
[2020, 432(4):828-844]

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The First Report of Genetic and Structural Diversities in the SPRN Gene in the Horse, an Animal Resistant to Prion Disease.

Sae-Young Won, Yong-Chan Kim, Seon-Kwan Kim, Byung-Hoon Jeong,

Prion diseases are fatal neurodegenerative diseases and are characterized by the accumulation of abnormal prion protein (PrPSc) in the brain. During the outbreak of the bovine spongiform encephalopathy (BSE) epidemic in the United Kingdom, prion diseases in several species were reported; however, horse prion disease has not been reported thus ... Read more >>

Genes (Basel) (Genes)
[2019, 11(1):]

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Kinetics of α-synuclein prions preceding neuropathological inclusions in multiple system atrophy.

Amanda L Woerman, Smita Patel, Sabeen A Kazmi, Abby Oehler, Jisoo Lee, Daniel A Mordes, Steven H Olson, Stanley B Prusiner,

Multiple system atrophy (MSA), a progressive neurodegenerative disease characterized by autonomic dysfunction and motor impairment, is caused by the self-templated misfolding of the protein α-synuclein. With no treatment currently available, we sought to characterize the spread of α-synuclein in a transgenic mouse model of MSA prion propagation to support drug ... Read more >>

PLoS Pathog. (PLoS pathogens)
[2020, 16(2):e1008222]

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Development and structural determination of an anti-PrPC aptamer that blocks pathological conformational conversion of prion protein.

Tsukasa Mashima, Joon-Hwa Lee, Yuji O Kamatari, Tomohiko Hayashi, Takashi Nagata, Fumiko Nishikawa, Satoshi Nishikawa, Masahiro Kinoshita, Kazuo Kuwata, Masato Katahira,

Prion diseases comprise a fatal neuropathy caused by the conversion of prion protein from a cellular (PrPC) to a pathological (PrPSc) isoform. Previously, we obtained an RNA aptamer, r(GGAGGAGGAGGA) (R12), that folds into a unique G-quadruplex. The R12 homodimer binds to a PrPC molecule, inhibiting PrPC-to-PrPSc conversion. Here, we developed ... Read more >>

Sci Rep (Scientific reports)
[2020, 10(1):4934]

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Selective neuronal vulnerability is involved in cerebellar lesions of Guinea pigs infected with bovine spongiform encephalopathy (BSE) prions: Immunohistochemical and electron microscopic investigations.

Shoichi Sakaguchi, Sayo Shintani, Kyohei Kamio, Akio Sekiya, Satomi Kato, Yoshikage Muroi, Motohiro Horiuchi, Hidefumi Furuoka,

The cerebellar lesions of bovine spongiform encephalopathy (BSE)-infected guinea pigs were characterized as severe atrophy of the cerebellar cortex associated with the loss of granule cells, decrease in the width of the molecular layer, and intense protease-resistant prion protein (PrPSc ) accumulations that are similar to cerebellar lesions in kuru ... Read more >>

Neuropathology (Neuropathology : official journal of the Japanese Society of Neuropathology)
[2019, :]

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Study protocol for enhanced CJD surveillance in the 65+ years population group in Scotland: an observational neuropathological screening study of banked brain tissue donations for evidence of prion disease.

Alexander Howard Peden, Lovney Kanguru, Diane L Ritchie, Colin Smith, Anna M Molesworth,

INTRODUCTION:Creutzfeldt-Jakob disease (CJD) is a human prion disease that occurs in sporadic, genetic and acquired forms. Variant CJD (vCJD) is an acquired form first identified in 1996 in the UK. To date, 178 cases of vCJD have been reported in the UK, most of which have been associated with dietary ... Read more >>

BMJ Open (BMJ open)
[2019, 9(10):e033744]

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1413. Surveillance of Variant Creutzfeldt Jakob Disease, Based on the Non-confirmatory Diagnostic Criteria of the EU Case Definition, Greece, 2014–2018.

Georgios Dougas, Kassiani Mellou, Konstantinos Mitrou, Flora Kontopidou, Theano Georgakopoulou, Sotirios Tsiodras,

AbstractBackgroundVariant Creutzfeldt–Jakob disease (vCJD), is a form of CJD associated with consumption of tissues from cattle infected with bovine spongiform encephalopathy. Confirmation requires cerebral histopathologic examination, ascertaining spongiform change and extensive prion protein deposition with florid plaques. In Greece, the disease has been included in the Mandatory Notification System since ... Read more >>

Open Forum Infect Dis (Open forum infectious diseases)
[2019, 6(Suppl 2):S515-S515]

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Goats naturally devoid of PrPC are resistant to scrapie.

Øyvind Salvesen, Arild Espenes, Malin R Reiten, Tram T Vuong, Giulia Malachin, Linh Tran, Olivier Andréoletti, Ingrid Olsaker, Sylvie L Benestad, Michael A Tranulis, Cecilie Ersdal,

Prion diseases are progressive and fatal, neurodegenerative disorders described in humans and animals. According to the "protein-only" hypothesis, the normal host-encoded prion protein (PrPC) is converted into a pathological and infectious form (PrPSc) in these diseases. Transgenic knockout models have shown that PrPC is a prerequisite for the development of ... Read more >>

Vet. Res. (Veterinary research)
[2020, 51(1):1]

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Scrapie susceptibility-associated indel polymorphism of shadow of prion protein gene (SPRN) in Korean native black goats.

Yong-Chan Kim, Seon-Kwan Kim, Byung-Hoon Jeong,

Prion diseases in sheep and goats are called scrapie and belong to a group of transmissible spongiform encephalopathies (TSEs) caused by the abnormal misfolding of the prion protein encoded by the prion protein gene (PRNP). The shadow of the prion protein gene (SPRN) is the only prion gene family member ... Read more >>

Sci Rep (Scientific reports)
[2019, 9(1):15261]

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Novel Strain of the Chronic Wasting Disease Agent Isolated From Experimentally Inoculated Elk With LL132 Prion Protein.

Jo Moore, Trudy Tatum, Soyoun Hwang, Catherine Vrentas, M Heather West Greenlee, Qingzhong Kong, Eric Nicholson, Justin Greenlee,

Chronic wasting disease (CWD) is a fatal, progressive disease that affects cervid species, including Rocky mountain elk (Cervus elaphus nelsoni). There are 2 allelic variants in the elk prion protein gene: L132 (leucine) and M132 (methionine). Following experimental oral challenge with the CWD agent incubation periods are longest in LL132 ... Read more >>

Sci Rep (Scientific reports)
[2020, 10(1):3148]

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Role of donor genotype in RT-QuIC seeding activity of chronic wasting disease prions using human and bank vole substrates.

Soyoun Hwang, Justin J Greenlee, Eric M Nicholson,

Chronic wasting disease is a transmissible spongiform encephalopathy of cervids. This fatal neurodegenerative disease is caused by misfolding of the cellular prion protein (PrPC) to pathogenic conformers (PrPSc), and the pathogenic forms accumulate in the brain and other tissues. Real-time Quaking Induced Conversion (RT-QuIC) can be used for the detection ... Read more >>

PLoS ONE (PloS one)
[2020, 15(1):e0227487]

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Sheep Are Susceptible to the Bovine Adapted Transmissible Mink Encephalopathy Agent by Intracranial Inoculation and Have Evidence of Infectivity in Lymphoid Tissues.

Eric D Cassmann, S Jo Moore, Jodi D Smith, Justin J Greenlee,

Transmissible mink encephalopathy (TME) is a food borne prion disease. Epidemiological and experimental evidence suggests similarities between the agents of TME and L-BSE. This experiment demonstrates the susceptibility of four different genotypes of sheep to the bovine adapted TME agent by intracranial inoculation. The four genotypes of sheep used in ... Read more >>

Front Vet Sci (Frontiers in veterinary science)
[2019, 6:430]

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