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https://www.readbyqxmd.com/read/29167394/prion-seeding-activity-and-infectivity-in-skin-samples-from-patients-with-sporadic-creutzfeldt-jakob-disease
#1
Christina D Orrú, Jue Yuan, Brian S Appleby, Baiya Li, Yu Li, Dane Winner, Zerui Wang, Yi-An Zhan, Mark Rodgers, Jason Rarick, Robert E Wyza, Tripti Joshi, Gong-Xian Wang, Mark L Cohen, Shulin Zhang, Bradley R Groveman, Robert B Petersen, James W Ironside, Miguel E Quiñones-Mateu, Jiri G Safar, Qingzhong Kong, Byron Caughey, Wen-Quan Zou
Sporadic Creutzfeldt-Jakob disease (sCJD), the most common human prion disease, is transmissible through iatrogenic routes due to abundant infectious prions [misfolded forms of the prion protein (PrPSc)] in the central nervous system (CNS). Some epidemiological studies have associated sCJD risk with non-CNS surgeries. We explored the potential prion seeding activity and infectivity of skin from sCJD patients. Autopsy or biopsy skin samples from 38 patients [21 sCJD, 2 variant CJD (vCJD), and 15 non-CJD] were analyzed by Western blotting and real-time quaking-induced conversion (RT-QuIC) for PrPSc Skin samples from two patients were further examined for prion infectivity by bioassay using two lines of humanized transgenic mice...
November 22, 2017: Science Translational Medicine
https://www.readbyqxmd.com/read/29142239/differential-overexpression-of-serpina3-in-human-prion-diseases
#2
S Vanni, F Moda, M Zattoni, E Bistaffa, E De Cecco, M Rossi, G Giaccone, F Tagliavini, S Haïk, J P Deslys, G Zanusso, J W Ironside, I Ferrer, G G Kovacs, G Legname
Prion diseases are fatal neurodegenerative disorders with sporadic, genetic or acquired etiologies. The molecular alterations leading to the onset and the spreading of these diseases are still unknown. In a previous work we identified a five-gene signature able to distinguish intracranially BSE-infected macaques from healthy ones, with SERPINA3 showing the most prominent dysregulation. We analyzed 128 suitable frontal cortex samples, from prion-affected patients (variant Creutzfeldt-Jakob disease (vCJD) n = 20, iatrogenic CJD (iCJD) n = 11, sporadic CJD (sCJD) n = 23, familial CJD (gCJD) n = 17, fatal familial insomnia (FFI) n = 9, Gerstmann-Sträussler-Scheinker syndrome (GSS)) n = 4), patients with Alzheimer disease (AD, n = 14) and age-matched controls (n = 30)...
November 15, 2017: Scientific Reports
https://www.readbyqxmd.com/read/29141869/human-stem-cell-derived-astrocytes-replicate-human-prions-in-a-prnp-genotype-dependent-manner
#3
Zuzana Krejciova, James Alibhai, Chen Zhao, Robert Krencik, Nina M Rzechorzek, Erik M Ullian, Jean Manson, James W Ironside, Mark W Head, Siddharthan Chandran
Prions are infectious agents that cause neurodegenerative diseases such as Creutzfeldt-Jakob disease (CJD). The absence of a human cell culture model that replicates human prions has hampered prion disease research for decades. In this paper, we show that astrocytes derived from human induced pluripotent stem cells (iPSCs) support the replication of prions from brain samples of CJD patients. For experimental exposure of astrocytes to variant CJD (vCJD), the kinetics of prion replication occur in a prion protein codon 129 genotype-dependent manner, reflecting the genotype-dependent susceptibility to clinical vCJD found in patients...
November 15, 2017: Journal of Experimental Medicine
https://www.readbyqxmd.com/read/29097653/experimental-transfusion-of-variant-cjd-infected-blood-reveals-previously-uncharacterised-prion-disorder-in-mice-and-macaque
#4
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/28838665/infectious-and-sporadic-prion-diseases
#5
Richard Knight
Prion diseases are progressive fatal encephalopathies characterized by a neurodegenerative pathology, the tissue deposition of abnormally folded prion protein and, in general, potential transmissibility. Creutzfeldt-Jakob disease (CJD) is the commonest human prion disease and occurs in three principal forms: sporadic (idiopathic), acquired (infectious), and inherited (genetic). This chapter concerns the sporadic and acquired forms. Sporadic CJD occurs worldwide and affects mainly the middle aged and elderly...
2017: Progress in Molecular Biology and Translational Science
https://www.readbyqxmd.com/read/28838661/transmission-and-replication-of-prions
#6
Alba Marín-Moreno, Natalia Fernández-Borges, Juan C Espinosa, Olivier Andréoletti, Juan M Torres
Transmissible spongiform encephalopathies (TSEs) are a group of progressive, invariably fatal diseases that affect the nervous system of many mammals including humans. The key molecular event in the pathogenesis of TSEs is the conversion of the cellular prion protein PrP(C) into a disease-associated isoform PrP(Sc). The "protein-only hypothesis" argues that PrP(Sc) itself is the infectious agent. In effect, PrP(Sc) can adopt several structures that represent different prion strains. The interspecies transmission of TSEs is difficult because of differences between the host and donor primary PrP sequence...
2017: Progress in Molecular Biology and Translational Science
https://www.readbyqxmd.com/read/28820380/similarities-of-variant-creutzfeldt-jakob-disease-strain-in-mother-and-son-in-spain-to-uk-reference-case
#7
Abigail B Diack, Aileen Boyle, Diane Ritchie, Chris Plinston, Dorothy Kisielewski, Jesús de Pedro-Cuesta, Alberto Rábano, Robert G Will, Jean C Manson
We investigated transmission characteristics of variant Creutzfeldt-Jakob disease in a mother and son from Spain. Despite differences in patient age and disease manifestations, we found the same strain properties in these patients as in UK vCJD cases. A single strain of agent appears to be responsible for all vCJD cases to date.
September 2017: Emerging Infectious Diseases
https://www.readbyqxmd.com/read/28820136/protective-effect-of-val129-prp-against-bovine-spongiform-encephalopathy-but-not-variant-creutzfeldt-jakob-disease
#8
Natalia Fernández-Borges, Juan Carlos Espinosa, Alba Marín-Moreno, Patricia Aguilar-Calvo, Emmanuel A Asante, Tetsuyuki Kitamoto, Shirou Mohri, Olivier Andréoletti, Juan María Torres
Bovine spongiform encephalopathy (BSE) is the only known zoonotic prion that causes variant Creutzfeldt-Jakob disease (vCJD) in humans. The major risk determinant for this disease is the polymorphic codon 129 of the human prion protein (Hu-PrP), where either methionine (Met129) or valine (Val129) can be encoded. To date, all clinical and neuropathologically confirmed vCJD cases have been Met129 homozygous, with the exception of 1 recently reported Met/Val heterozygous case. Here, we found that transgenic mice homozygous for Val129 Hu-PrP show severely restricted propagation of the BSE prion strain, but this constraint can be partially overcome by adaptation of the BSE agent to the Met129 Hu-PrP...
September 2017: Emerging Infectious Diseases
https://www.readbyqxmd.com/read/28791720/infectivity-in-bone-marrow-from-sporadic-cjd-patients
#9
Alvina Huor, Jean Yves Douet, Caroline Lacroux, Séverine Lugan, Cécile Tillier, Naima Aron, Hervé Cassard, Mark Arnold, Juan Maria Torres, James W Ironside, Olivier Andréoletti
Prion infectivity was recently identified in the blood of both sporadic and variant Creutzfeldt-Jakob disease (CJD) patients. In variant CJD (vCJD), the widespread distribution of prions in peripheral tissues of both asymptomatic and symptomatic patients is likely to explain the occurrence of the observed prionaemia. However, in sporadic CJD (sCJD), prion infectivity is described to be located principally in the central nervous system. In this study, we investigated the presence of prion infectivity in bone marrow collected after death in patients affected with different sCJD agents...
November 2017: Journal of Pathology
https://www.readbyqxmd.com/read/28518033/distribution-and-quantitative-estimates-of-variant-creutzfeldt-jakob-disease-prions-in-tissues-of-clinical-and-asymptomatic-patients
#10
Jean Y Douet, Caroline Lacroux, Naima Aron, Mark W Head, Séverine Lugan, Cécile Tillier, Alvina Huor, Hervé Cassard, Mark Arnold, Vincent Beringue, James W Ironside, Olivier Andréoletti
In the United-Kingdom, ≈1 of 2,000 persons could be infected with variant Creutzfeldt-Jakob disease (vCJD). Therefore, risk of transmission of vCJD by medical procedures remains a major concern for public health authorities. In this study, we used in vitro amplification of prions by protein misfolding cyclic amplification (PMCA) to estimate distribution and level of the vCJD agent in 21 tissues from 4 patients who died of clinical vCJD and from 1 asymptomatic person with vCJD. PMCA identified major levels of vCJD prions in a range of tissues, including liver, salivary gland, kidney, lung, and bone marrow...
June 2017: Emerging Infectious Diseases
https://www.readbyqxmd.com/read/28455699/an-in-vivo-11-c-r-pk11195-pet-and-in-vitro-pathology-study-of-microglia-activation-in-creutzfeldt-jakob-disease
#11
Leonardo Iaccarino, Rosa Maria Moresco, Luca Presotto, Orso Bugiani, Sandro Iannaccone, Giorgio Giaccone, Fabrizio Tagliavini, Daniela Perani
Microgliosis is part of the immunobiology of Creutzfeldt-Jakob disease (CJD). This is the first report using (11)C-(R)-PK11195 PET imaging in vivo to measure 18 kDa translocator protein (TSPO) expression, indexing microglia activation, in symptomatic CJD patients, followed by a postmortem neuropathology comparison. One genetic CJD (gCJD) patient, two sporadic CJD (sCJD) patients, one variant CJD (vCJD) patient (mean ± SD age, 47.50 ± 15.95 years), and nine healthy controls (mean ± SD age, 44.00 ± 11...
April 28, 2017: Molecular Neurobiology
https://www.readbyqxmd.com/read/28444687/creutzfeldt-jakob-disease-lookback-study-21-years-of-surveillance-for-transfusion-transmission-risk
#12
Lauren A Crowder, Lawrence B Schonberger, Roger Y Dodd, Whitney R Steele
BACKGROUND: Transfusion transmission of human prion diseases has been observed for variant Creutzfeldt-Jakob disease (vCJD), but not for the classic forms of prion disease (CJD: sporadic, genetic, and iatrogenic). Although the presence of prions or misfolded prion proteins in blood has been documented in some patients with the most common form of CJD, sporadic CJD, no transfusion-transmitted cases of CJD have been recognized. Since 1995, the American Red Cross has conducted a lookback study of the recipients of blood products from donors who develop CJD to assess the risk of blood-borne CJD transmission in the United States...
August 2017: Transfusion
https://www.readbyqxmd.com/read/28332471/cjd-surveillance-in-the-republic-of-ireland-from-2005-to-2015-a-suggested-algorithm-for-referrals
#13
Teresa Loftus, Daphne Chen, Seamus Looby, Albi Chalissery, Rachel Howley, Ciara Heaney, Josephine Heffernan, Michael Farrell, Francesca Brett
Definitive diagnosis of Creutzfeldt Jakob disease (CJD) remains tissue-based. Possible and probable CJD are useful clinical terms but may be used indiscriminately. The aim of this study was to assess the effectiveness of the Irish surveillance system and to ascertain how diagnostic accuracy in identifying clinically "definite" cases might be improved. We reviewed the clinical information, relevant investigations, and samples n = 100; (autopsy n = 87; biopsy n = 13) in 96 patients between January 1, 2005 and December 31, 2015...
March 23, 2017: Clinical Neuropathology
https://www.readbyqxmd.com/read/28261810/geographic-exposure-risk-of-variant-creutzfeldt-jakob-disease-in-us-blood-donors-a-risk-ranking-model-to-evaluate-alternative-donor-deferral-policies
#14
Hong Yang, Yin Huang, Luisa Gregori, David M Asher, Travis Bui, Richard A Forshee, Steven A Anderson
BACKGROUND: Variant Creutzfeldt-Jakob disease (vCJD) has been transmitted by blood transfusion (TTvCJD). The US Food and Drug Administration (FDA) recommends deferring blood donors who resided in or traveled to 30 European countries where they may have been exposed to bovine spongiform encephalopathy (BSE) through beef consumption. Those recommendations warrant re-evaluation, because new cases of BSE and vCJD have markedly abated. STUDY DESIGN AND METHODS: The FDA developed a risk-ranking model to calculate the geographic vCJD risk using country-specific case rates and person-years of exposure of US blood donors...
April 2017: Transfusion
https://www.readbyqxmd.com/read/28258683/-patient-with-creutzfeld-jakob-disease-a-case-report
#15
Żanna Pastuszak, Kazimierz Tomczykiewicz, Adam Stępień, Renata Piusińska-Macoch, Joanna Klimczuk, Agnieszka Rolewska, Dariusz Galbarczyk
Creutzfeldt-Jakob disease (CJD) is a rare syndrome of central nervous system caused by infectious protein called prion. There are four types of CJD: sporadic (sCJD), familial (fCJD), jatrogenic (jCJD) and variant (vCJD). The most frequent symptoms are rapidly progressing dementia, mioclonias, akinetic mutism and signs of cerebellum dysfunction. In sCJD, MRI often shows high signal intensity in the putamen and caudate nucleus on T2-weighted images while in vCJD pulvinar sign is often observed. 70% patients with CJD often has characteristic generalized periodic sharp wave pattern in electroencephalography...
February 20, 2017: Polski Merkuriusz Lekarski: Organ Polskiego Towarzystwa Lekarskiego
https://www.readbyqxmd.com/read/28231300/detection-and-partial-discrimination-of-atypical-and-classical-bovine-spongiform-encephalopathies-in-cattle-and-primates-using-real-time-quaking-induced-conversion-assay
#16
Etienne Levavasseur, Anne-Gaëlle Biacabe, Emmanuel Comoy, Audrey Culeux, Katarina Grznarova, Nicolas Privat, Steve Simoneau, Benoit Flan, Véronique Sazdovitch, Danielle Seilhean, Thierry Baron, Stéphane Haïk
The transmission of classical bovine spongiform encephalopathy (C-BSE) through contaminated meat product consumption is responsible for variant Creutzfeldt-Jakob disease (vCJD) in humans. More recent and atypical forms of BSE (L-BSE and H-BSE) have been identified in cattle since the C-BSE epidemic. Their low incidence and advanced age of onset are compatible with a sporadic origin, as are most cases of Creutzfeldt-Jakob disease (CJD) in humans. Transmissions studies in primates and transgenic mice expressing a human prion protein (PrP) indicated that atypical forms of BSE may be associated with a higher zoonotic potential than classical BSE, and require particular attention for public health...
2017: PloS One
https://www.readbyqxmd.com/read/28193766/molecular-mechanisms-of-chronic-wasting-disease-prion-propagation
#17
Julie A Moreno, Glenn C Telling
Prion disease epidemics, which have been unpredictable recurrences, are of significant concern for animal and human health. Examples include kuru, once the leading cause of death among the Fore people in Papua New Guinea and caused by mortuary feasting; bovine spongiform encephalopathy (BSE) and its subsequent transmission to humans in the form of variant Creutzfeldt-Jakob disease (vCJD), and repeated examples of large-scale prion disease epidemics in animals caused by contaminated vaccines. The etiology of chronic wasting disease (CWD), a relatively new and burgeoning prion epidemic in deer, elk, and moose (members of the cervid family), is more enigmatic...
February 13, 2017: Cold Spring Harbor Perspectives in Medicine
https://www.readbyqxmd.com/read/28151391/variant-creutzfeldt-jakob-disease-deferral-in-canada-impact-of-stop-dates
#18
Sheila F O'brien, Wenli Fan, Qi-Long Yi, Samra Uzicanin, Lori Osmond, Mindy Goldman
BACKGROUND: To reduce the risk of variant Creutzfeldt-Jakob disease (vCJD) transmission via blood transfusion in Canada, potential donors who spent a cumulative time in the United Kingdom, Western Europe or Saudi Arabia are deferred. "Stop dates" for accumulated time were later implemented for 3 months spent in the United Kingdom or France (1980-1996) and for 5 years elsewhere in Western Europe (1980-2007); Saudi Arabia deferral was implemented with the "stop date" (1980-1996). We evaluated the long-term impact of these deferrals and "stop dates", as well as the consistency of donors' answers to post-implementation screening questions...
December 15, 2016: Blood Transfusion, Trasfusione del Sangue
https://www.readbyqxmd.com/read/28003548/detection-of-prions-in-blood-from-patients-with-variant-creutzfeldt-jakob-disease
#19
Luis Concha-Marambio, Sandra Pritzkow, Fabio Moda, Fabrizio Tagliavini, James W Ironside, Paul E Schulz, Claudio Soto
Human prion diseases are infectious and invariably fatal neurodegenerative diseases. They include sporadic Creutzfeldt-Jakob disease (sCJD), the most common form, and variant CJD (vCJD), which is caused by interspecies transmission of prions from cattle infected by bovine spongiform encephalopathy. Development of a biochemical assay for the sensitive, specific, early, and noninvasive detection of prions (PrP(Sc)) in the blood of patients affected by prion disease is a top medical priority to increase the safety of the blood supply...
December 21, 2016: Science Translational Medicine
https://www.readbyqxmd.com/read/28003547/detection-of-prions-in-the-plasma-of-presymptomatic-and-symptomatic-patients-with-variant-creutzfeldt-jakob-disease
#20
Daisy Bougard, Jean-Philippe Brandel, Maxime Bélondrade, Vincent Béringue, Christiane Segarra, Hervé Fleury, Jean-Louis Laplanche, Charly Mayran, Simon Nicot, Alison Green, Arlette Welaratne, David Narbey, Chantal Fournier-Wirth, Richard Knight, Robert Will, Pierre Tiberghien, Stéphane Haïk, Joliette Coste
Variant Creutzfeldt-Jakob disease (vCJD) is a human prion disease resulting from the consumption of meat products contaminated by the agent causing bovine spongiform encephalopathy. Evidence supporting the presence of a population of silent carriers that can potentially transmit the disease through blood transfusion is increasing. The development of a blood-screening assay for both symptomatic vCJD patients and asymptomatic carriers is urgently required. We show that a diagnostic assay combining plasminogen-bead capture and protein misfolding cyclic amplification (PMCA) technologies consistently detected minute amounts of abnormal prion protein from French and British vCJD cases in the required femtomolar range...
December 21, 2016: Science Translational Medicine
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