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https://www.readbyqxmd.com/read/28098532/oral-transmission-of-l-type-bovine-spongiform-encephalopathy-agent-among-cattle
#1
Hiroyuki Okada, Yoshifumi Iwamaru, Morikazu Imamura, Kohtaro Miyazawa, Yuichi Matsuura, Kentaro Masujin, Yuichi Murayama, Takashi Yokoyama
To determine oral transmissibility of the L-type bovine spongiform encephalopathy (BSE) prion, we orally inoculated 16 calves with brain homogenates of the agent. Only 1 animal, given a high dose, showed signs and died at 88 months. These results suggest low risk for oral transmission of the L-BSE agent among cattle.
February 2017: Emerging Infectious Diseases
https://www.readbyqxmd.com/read/28096245/prion-like-characteristics-of-polyglutamine-containing-proteins
#2
Margaret M P Pearce, Ron R Kopito
Transmissible spongiform encephalopathies are infectious neurodegenerative diseases caused by the conversion of prion protein (PrP) into a self-replicating conformation that spreads via templated conversion of natively folded PrP molecules within or between cells. Recent studies provide compelling evidence that prion-like behavior is a general property of most protein aggregates associated with neurodegenerative diseases. Many of these disorders are associated with spontaneous protein aggregation, but genetic mutations can increase the aggregation propensity of specific proteins, including expansion of polyglutamine (polyQ) tracts, which is causative of nine inherited neurodegenerative diseases...
January 17, 2017: Cold Spring Harbor Perspectives in Medicine
https://www.readbyqxmd.com/read/28093855/typical-and-atypical-appearance-of-early-onset-alzheimer-s-disease-a-clinical-neuroimaging-and-neuropathological-study-symposium-fundamentals-learned-from-diversity-among-typical-and-atypical-appearances
#3
Shinobu Kawakatsu 川勝 忍, Ryota Kobayashi, Hiroshi Hayashi
The International Working Group (IWG) has classified Alzheimer's disease (AD) as two different types, the typical form and the atypical form, but clinicopathological studies of atypical AD are limited. Because atypical AD cases usually present with early-onset dementia, we investigated 12 patients with early-onset AD, including two patients with typical AD and 10 patients with atypical AD. Of these patients, six had the posterior variant, three had the frontal variant and one had the logopenic variant mixed with semantic dementia...
January 17, 2017: Neuropathology: Official Journal of the Japanese Society of Neuropathology
https://www.readbyqxmd.com/read/28077719/suppressing-n-acetyl-l-aspartate-synthesis-prevents-loss-of-neurons-in-a-murine-model-of-canavan-leukodystrophy
#4
Jiho Sohn, Peter Bannerman, Fuzheng Guo, Travis Burns, Laird Miers, Christopher Croteau, Naveen K Singhal, Jennifer A McDonough, David Pleasure
: Canavan disease is a leukodystrophy caused by aspartoacylase (ASPA) deficiency. The lack of functional ASPA, an enzyme enriched in oligodendroglia that cleaves N-acetyl-l-aspartate (NAA) to acetate and l-aspartic acid, elevates brain NAA and causes "spongiform" vacuolation of superficial brain white matter and neighboring gray matter. In children with Canavan disease, neuroimaging shows early-onset dysmyelination and progressive brain atrophy. Neuron loss has been documented at autopsy in some cases...
January 11, 2017: Journal of Neuroscience: the Official Journal of the Society for Neuroscience
https://www.readbyqxmd.com/read/28071987/a-computer-aided-diagnosis-system-using-artificial-intelligence-for-the-diagnosis-and-characterization-of-thyroid-nodules-on-ultrasound-initial-clinical-assessment
#5
Young Jun Choi, Jung Hwan Baek, Hye Sun Park, Woo Hyun Shim, Tae Yong Kim, YoungKee Shong, Jeong Hyun Lee
Background We describe an initial clinical assessment of a new, commercially available, computer-aided diagnosis (CAD) system using artificial intelligence (AI) for thyroid ultrasound, and evaluate its performance in the diagnosis of malignant thyroid nodules and categorization of nodule characteristics. Methods This prospective study protocol was reviewed and approved by the institutional review board. Patients with thyroid nodules with decisive diagnosis, whether benign or malignant on the basis of cytopathologic or US results, were consecutively enrolled from November 2015 to February 2016...
January 10, 2017: Thyroid: Official Journal of the American Thyroid Association
https://www.readbyqxmd.com/read/28068881/eu-approved-rapid-tests-might-underestimate-bovine-spongiform-encephalopathy-infection-in-goats
#6
Daniela Meloni, Elena Bozzetta, Jan P M Langeveld, Martin H Groschup, Wilfred Goldmann, Olivier Andrèoletti, Isabelle Lantier, Lucien Van Keulen, Alex Bossers, Danilo Pitardi, Romolo Nonno, Theodoros Sklaviadis, Francesco Ingravalle, Simone Peletto, Silvia Colussi, Pier Luigi Acutis
We report the diagnostic sensitivity of 3 EU-approved rapid tests (ELISAs; 1 from IDEXX and 2 from Bio-Rad) for the detection of transmissible spongiform encephalopathy diseases in goats. Ninety-eight goat brainstem samples were tested. All the rapid tests had 100% specificity and ≥80% sensitivity, with the IDEXX test significantly more sensitive than the 2 Bio-Rad tests. All tests detected 100% of samples from goats with clinical scrapie, but missed 8% (IDEXX) to 33% (Bio-Rad SG) of samples from preclinical goats...
January 1, 2017: Journal of Veterinary Diagnostic Investigation
https://www.readbyqxmd.com/read/28059790/combined-creutzfeldt-jakob-jacob-alzheimer-s-disease-cases-are-important-in-search-for-microbes-in-alzheimer-s-disease
#7
Frank O Bastian
The question whether Alzheimer's disease is infectious as brought up in the recent editorial published in the Journal of Alzheimer's Disease is complicated by the controversy whether the causal agent is a microbe or a misfolded host protein (amyloid). The replicating amyloid (prion) theory, based upon data from studies of Creutzfeldt-Jakob disease (CJD) and other transmissible spongiform encephalopathies (TSEs), has been challenged since the prion can be separated from TSE infectivity, and spiroplasma, a wall-less bacterium, has been shown to be involved in the pathogenesis of CJD...
December 3, 2017: Journal of Alzheimer's Disease: JAD
https://www.readbyqxmd.com/read/28041803/development-of-radioiodinated-acridine-derivatives-for-in-vivo-imaging-of-prion-deposits-in-the-brain
#8
Masao Kawasaki, Takeshi Fuchigami, Nobuya Kobashi, Takehiro Nakagaki, Kazunori Sano, Ryuichiro Atarashi, Sakura Yoshida, Mamoru Haratake, Noriyuki Nishida, Morio Nakayama
Prion diseases are caused by deposition of abnormal prion protein aggregates (PrP(Sc)) in the central nervous system. This study aimed to develop in vivo imaging probes that can detect cerebral PrP(Sc) deposits. We synthesized several quinacrine-based acridine (AC) derivatives with 2,9-substitution and radioiodinated them. The AC derivatives were evaluated as prion-imaging probes using recombinant mouse prion protein (rMoPrP) aggregates and brain sections of mouse-adapted bovine spongiform encephalopathy (mBSE)-infected mice...
December 19, 2016: Bioorganic & Medicinal Chemistry
https://www.readbyqxmd.com/read/28028861/creutzfeldt-jakob-disease
#9
REVIEW
Yasushi Iwasaki 岩崎 靖
This review will explore the clinical and pathological findings of the various forms of Creutzfeldt-Jakob disease (CJD). Clinical findings of CJD are characterized by rapidly progressive cognitive dysfunction, diffusion-weighted magnetic resonance imaging (DWI) hyperintensity, myoclonus, periodic sharp-wave complexes on electroencephalogram and akinetic mutism state. Neuropathologic findings of CJD are characterized by spongiform changes in gray matter, gliosis-particularly hypertrophic astrocytosis-neuropil rarefaction, neuron loss and prion protein (PrP) deposition...
December 28, 2016: Neuropathology: Official Journal of the Japanese Society of Neuropathology
https://www.readbyqxmd.com/read/28003548/detection-of-prions-in-blood-from-patients-with-variant-creutzfeldt-jakob-disease
#10
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
#11
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
https://www.readbyqxmd.com/read/27995680/bay-81-8973-a-full-length-recombinant-factor-viii-manufacturing-processes-and-product-characteristics
#12
REVIEW
S Garger, J Severs, L Regan, A Hesslein, J Ignowski, P Wu, E Long, S Gupta, S Liu, W Wang
BAY 81-8973 (Kovaltry(®) , Bayer, Berkeley, CA, USA) is an unmodified, full-length recombinant human factor VIII (FVIII) approved for prophylaxis and on-demand treatment of bleeding episodes in patients with haemophilia A. The BAY 81-8973 manufacturing process is based on the process used for sucrose-formulated recombinant FVIII (rFVIII-FS), with changes and enhancements made to improve production efficiency, further augment pathogen safety, and eliminate animal- and human-derived raw materials from the production processes...
December 19, 2016: Haemophilia: the Official Journal of the World Federation of Hemophilia
https://www.readbyqxmd.com/read/27995109/chitosan-myristate-nanogel-as-an-artificial-chaperone-protects-neuroserpin-from-misfolding
#13
Habib Nazem, Afshin Mohsenifar, Sahar Majdi
BACKGROUND: Molecular chaperon-like activity for protein refolding was studied using nanogel chitosan-myristic acid (CMA) and the protein neuroserpin (NS), a member of the serine proteinase inhibitor superfamily (serpin). MATERIALS AND METHODS: Recombinant his-tag fusion NS was expressed in Escherichia coli. For confirmation of refolding of the purified NS, structural analysis was performed by circular dichroism and spectrofluorometric along with its inhibitory activity, which was assayed by single-chain tissue plasminogen activator...
2016: Advanced Biomedical Research
https://www.readbyqxmd.com/read/27983987/differentially-expressed-genes-in-iron-induced-prion-protein-conversion
#14
Minsun Kim, Eun-Hee Kim, Bo-Ran Choi, Hee-Jong Woo
The conversion of the cellular prion protein (PrP(C)) to the protease-resistant isoform is the key event in chronic neurodegenerative diseases, including transmissible spongiform encephalopathies (TSEs). Increased iron in prion-related disease has been observed due to the prion protein-ferritin complex. Additionally, the accumulation and conversion of recombinant PrP (rPrP) is specifically derived from Fe(III) but not Fe(II). Fe(III)-mediated PK-resistant PrP (PrP(res)) conversion occurs within a complex cellular environment rather than via direct contact between rPrP and Fe(III)...
October 28, 2016: Biochemical and Biophysical Research Communications
https://www.readbyqxmd.com/read/27959774/increased-circulating-micrornas-mir-342-3p-and-mir-21-5p-in-natural-sheep-prion-disease
#15
David Sanz Rubio, Óscar López-Pérez, Alvaro de Andrés Pablo, Rosa Bolea, Rosario Osta, Juan J Badiola, Pilar Zaragoza, Inmaculada Martín-Burriel, Janne M Toivonen
Scrapie is a transmissible spongiform encephalopathy (TSE), or prion disease, of sheep and goats. As no simple diagnostic tests are yet available to detect TSEs in vivo, easily accessible biomarkers could facilitate the eradication of scrapie agents from the food chain. To this end, we analysed by quantitative RT-PCR a selected set of candidate microRNAs (miRNAs) from circulating blood plasma of naturally infected, classical scrapie sheep that demonstrated clear scrapie symptoms and pathology. Significant scrapie-associated increase was repeatedly found for miR-342-3p and miR-21-5p...
December 13, 2016: Journal of General Virology
https://www.readbyqxmd.com/read/27931923/assessing-the-sensitivity-of-european-surveillance-for-detecting-bse-in-cattle-according-to-international-standards
#16
Amie Adkin, Robin Simons, Mark Arnold
European surveillance for Bovine Spongiform Encephalopathy (BSE), initiated in 2001, has shown a steady exponential decline in the number of infected cattle, demonstrating that control measures have been effective. In 2016 23 European countries out of 28 demonstrated negligible risk status for the disease. The international standard setting body, the World Organization for Animal Health (OIE), prescribes that for countries where there is a non-negligible BSE risk, surveillance should allow the detection of one case per 100,000 in the adult cattle population with 95% confidence (Type A surveillance)...
December 1, 2016: Preventive Veterinary Medicine
https://www.readbyqxmd.com/read/27927234/cytotoxic-edema-and-diffusion-restriction-as-an-early-pathoradiologic-marker-in-canavan-disease-case-report-and-review-of-the-literature
#17
Steven T Merrill, Gary R Nelson, Nicola Longo, Joshua L Bonkowsky
BACKGROUND: Canavan disease is a devastating autosomal recessive leukodystrophy leading to spongiform degeneration of the white matter. There is no cure or treatment for Canavan disease, and disease progression is poorly understood. RESULTS: We report a new presentation of a patient found to have Canavan disease; brain magnetic resonance imaging (MRI) revealed white matter cytotoxic edema, indicative of an acute active destructive process. We performed a comprehensive review of published cases of Canavan disease reporting brain MRI findings, and found that cytotoxic brain edema is frequently reported in early Canavan disease...
December 7, 2016: Orphanet Journal of Rare Diseases
https://www.readbyqxmd.com/read/27925304/dura-mater-graft-associated-creutzfeldt-jakob-disease-with-30-year-incubation-period
#18
Masahiro Shijo, Hiroyuki Honda, Sachiko Koyama, Koji Ishitsuka, Koichiro Maeda, Junya Kuroda, Mitsugu Tanii, Takanari Kitazono, Toru Iwaki
Over 60% of all patients with dura mater graft-associated Creutzfeldt-Jakob disease (dCJD) have been diagnosed in Japan. The incubation period has ranged from 1 to 30 years and the age at onset from 15 to 80 years. Here, we report a 77-year-old male Japanese autopsied dCJD case with the longest incubation period so far in Japan. He received a cadaveric dural graft at the right cranial convexity following a craniotomy for meningioma at the age of 46. At 30 years post-dural graft placement, disorientation was observed as an initial symptom of dCJD...
December 7, 2016: Neuropathology: Official Journal of the Japanese Society of Neuropathology
https://www.readbyqxmd.com/read/27913587/suppressing-n-acetyl-l-aspartate-naa-synthesis-prevents-loss-of-neurons-in-a-murine-model-of-canavan-leukodystrophy
#19
Jiho Sohn, Peter Bannerman, Fuzheng Guo, Travis Burns, Laird Miers, Christopher Croteau, Naveen K Singhal, Jennifer A McDonough, David Pleasure
Canavan disease is a leukodystrophy caused by aspartoacylase (ASPA) deficiency. The lack of functional ASPA, an enzyme enriched in oligodendroglia that cleaves N-acetyl-L-aspartate (NAA) to acetate and L-aspartic acid, elevates brain NAA and causes "spongiform" vacuolation of superficial brain white matter and neighboring gray matter. In children with Canavan disease, neuroimaging shows early-onset dysmyelination and progressive brain atrophy. Neuron loss has been documented at autopsy in some cases. Prior studies have shown that mice homozygous for the Aspa nonsense mutation Nur7 also develop brain vacuolation...
December 2, 2016: Journal of Neuroscience: the Official Journal of the Society for Neuroscience
https://www.readbyqxmd.com/read/27892798/transmissibility-of-gerstmann-str%C3%A3-ussler-scheinker-syndrome-in-rodent-models-new-insights-into-the-molecular-underpinnings-of-prion-infectivity
#20
Romolo Nonno, Michele Angelo Di Bari, Umberto Agrimi, Laura Pirisinu
Prion diseases, or transmissible spongiform encephalopathies, have revealed the bewildering phenomenon of transmissibility in neurodegenerative diseases. Hence, the experimental transmissibility of prion-like neurodegenerative diseases via template directed misfolding has become the focus of intense research. Gerstmann-Sträussler-Scheinker disease (GSS) is an inherited prion disease associated with mutations in the prion protein gene. However, with the exception of a few GSS cases with P102L mutation characterized by co-accumulation of protease-resistant PrP core (PrP(res)) of ∼21 kDa, attempts to transmit to rodents GSS associated to atypical misfolded prion protein with ∼8 kDa PrP(res) have been unsuccessful...
November 2016: Prion
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