keyword
MENU ▼
Read by QxMD icon Read
search

Spongiform

keyword
https://www.readbyqxmd.com/read/28545018/fixed-differences-in-the-3-utr-of-buffalo-prnp-gene-provide-binding-sites-for-mirnas-post-transcriptional-regulation
#1
Hui Zhao, Siqi Wang, Lixia Guo, Yanli Du, Linlin Liu, Tengfei Ma, Newton O Otecko, Canpeng Li, Yaping Zhang
Bovine spongiform encephalopathy, a member of transmissible spongiform encephalopathies, has not been reported in buffaloes, Bubalus bubalis. Prion protein (PrP), encoded by the prion protein gene (PRNP), is fundamental in the pathogenesis of transmissible spongiform encephalopathies. We previously showed that buffaloes express more PrP proteins but lower PRNP mRNA than cattle in several pivotal tissues like the obex. Therefore, we sought to establish whether genetic variability in PRNP 3'UTR, mediated by miRNA down-regulation, causes PrP expression differences between cattle and buffaloes...
May 2, 2017: Oncotarget
https://www.readbyqxmd.com/read/28539446/infectious-prions-in-the-pregnancy-microenvironment-of-cwd-infected-reeves-muntjac-deer
#2
Amy V Nalls, Erin McNulty, Clare E Hoover, Laura A Pulscher, Edward A Hoover, Candace K Mathiason
Ample evidence exists for the presence of infectious agents at the maternal-fetal interface, often with grave outcomes to the developing fetus (i.e. zika virus, brucella, cytomegalovirus, toxoplasma). While less studied, pregnancy-related transmissible spongiform encephalopathies (TSEs) have been implicated in several species, including humans. Our previous work has shown that prions can be transferred from mother-to-offspring resulting in the development of clinical TSE disease in offspring born to CWD-infected muntjac dams (64)...
May 24, 2017: Journal of Virology
https://www.readbyqxmd.com/read/28513534/unraveling-prion-protein-interactions-with-aptamers-and-other-prp-binding-nucleic-acids
#3
REVIEW
Bruno Macedo, Yraima Cordeiro
Transmissible spongiform encephalopathies (TSEs) are a group of neurodegenerative disorders that affect humans and other mammals. The etiologic agents common to these diseases are misfolded conformations of the prion protein (PrP). The molecular mechanisms that trigger the structural conversion of the normal cellular PrP (PrP(C)) into the pathogenic conformer (PrP(Sc)) are still poorly understood. It is proposed that a molecular cofactor would act as a catalyst, lowering the activation energy of the conversion process, therefore favoring the transition of PrP(C) to PrP(Sc)...
May 17, 2017: International Journal of Molecular Sciences
https://www.readbyqxmd.com/read/28508264/biological-safety-of-a-highly-purified-10-liquid-intravenous-immunoglobulin-preparation-from-human-plasma
#4
Caroline Goussen, Steve Simoneau, Soline Bérend, Christine Jehan-Kimmel, Anne Bellon, Céline Ducloux, Bruno You, Philippe Paolantonacci, Monique Ollivier, Ludovic Burlot, Sami Chtourou, Benoît Flan
BACKGROUND: A highly purified 10% liquid intravenous immunoglobulin, IQYMUNE(®), has been developed using an innovative manufacturing process including an affinity chromatography step for the removal of anti-A and anti-B hemagglutinins. OBJECTIVES: The pathogen (viruses and prions) clearance efficacy of the manufacturing process and its robustness for critical steps were investigated. METHODS: The manufacturing process of IQYMUNE(®) includes two dedicated complementary virus reduction steps: solvent/detergent (S/D) treatment and 20 nm nanofiltration as well as two contributing steps, namely caprylic acid fractionation and anion-exchange chromatography...
May 15, 2017: BioDrugs: Clinical Immunotherapeutics, Biopharmaceuticals and Gene Therapy
https://www.readbyqxmd.com/read/28491313/positron-emission-tomography-imaging-in-a-case-of-e200k-mutation-related-spongiform-encephalopathy-with-non-diagnostic-magnetic-resonance-imaging-and-cerebrospinal-fluid-testing
#5
Pravin George, Christopher R Newey, Karin P Mente, Erik P Pioro
OBJECTIVE: Creutzfeldt-Jakob disease is a rapidly progressive spongiform encephalopathy. The E200K mutation is found in a majority of genetically transmitted Creutzfeldt-Jakob disease cases. METHODS: We describe the case and associated neuroimaging of an E200K-129M gene-mutation-related fatal spongiform encephalopathy with resultant clinical insomnia and thalamic changes. RESULTS: A 46-year-old Caucasian male presented with, who was well until 2 months prior to admission, a rapidly progressive dementia followed by a change in personality with auditory and visual hallucinations...
2017: SAGE open medical case reports
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
#6
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/28451263/polymorphism-at-129-dictates-metastable-conformations-of-the-human-prion-protein-n-terminal-%C3%AE-sheet
#7
S Alexis Paz, Eric Vanden-Eijnden, Cameron F Abrams
We study the thermodynamic stability of the native state of the human prion protein using a new free-energy method, replica-exchange on-the-fly parameterization. This method is designed to overcome hidden-variable sampling limitations to yield nearly error-free free-energy profiles along a conformational coordinate. We confirm that all four (M129V, D178N) polymorphs have a ground-state conformation with three intact β-sheet hydrogen bonds. Additionally, they are observed to have distinct metastabilities determined by the side-chain at position 129...
February 1, 2017: Chemical Science
https://www.readbyqxmd.com/read/28449707/altered-ca-2-homeostasis-induces-calpain-cathepsin-axis-activation-in-sporadic-creutzfeldt-jakob-disease
#8
Franc Llorens, Katrin Thüne, Beata Sikorska, Matthias Schmitz, Waqas Tahir, Natalia Fernández-Borges, Maria Cramm, Nadine Gotzmann, Margarita Carmona, Nathalie Streichenberger, Uwe Michel, Saima Zafar, Anna-Lena Schuetz, Ashish Rajput, Olivier Andréoletti, Stefan Bonn, Andre Fischer, Pawel P Liberski, Juan Maria Torres, Isidre Ferrer, Inga Zerr
Sporadic Creutzfeldt-Jakob disease (sCJD) is the most prevalent form of human prion disease and it is characterized by the presence of neuronal loss, spongiform degeneration, chronic inflammation and the accumulation of misfolded and pathogenic prion protein (PrP(Sc)). The molecular mechanisms underlying these alterations are largely unknown, but the presence of intracellular neuronal calcium (Ca(2+)) overload, a general feature in models of prion diseases, is suggested to play a key role in prion pathogenesis...
April 27, 2017: Acta Neuropathologica Communications
https://www.readbyqxmd.com/read/28441344/collagenous-extracellular-matrix-biomaterials-for-tissue-engineering-lessons-from-the-common-sea-urchin-tissue
#9
REVIEW
Kheng Lim Goh, David F Holmes
Scaffolds for tissue engineering application may be made from a collagenous extracellular matrix (ECM) of connective tissues because the ECM can mimic the functions of the target tissue. The primary sources of collagenous ECM material are calf skin and bone. However, these sources are associated with the risk of having bovine spongiform encephalopathy or transmissible spongiform encephalopathy. Alternative sources for collagenous ECM materials may be derived from livestock, e.g., pigs, and from marine animals, e...
April 25, 2017: International Journal of Molecular Sciences
https://www.readbyqxmd.com/read/28429419/semi-synthesis-of-murine-prion-protein-by-native-chemical-ligation-and-chemical-activation-for-preparation-of-polypeptide-%C3%AE-thioester
#10
Lei Shi, Huai Chen, Si-Yu Zhang, Ting-Ting Chu, Yu-Fen Zhao, Yong-Xiang Chen, Yan-Mei Li
Prions are suspected as pathogen of the fatal transmissible spongiform encephalopathies. Strategies to access homogenous prion protein (PrP) are required to fully comprehend the molecular mechanism of prion diseases. However, the polypeptide fragments from PrP show a high tendency to form aggregates, which is a gigantic obstacle of protein synthesis and purification. In this study, murine prion sequence 90 to 230 that is the core three-dimensional structure domain was constructed from three segments murine PrP (mPrP)(90-177), mPrP(178-212), and mPrP(213-230) by combining protein expression, chemical synthesis and chemical ligation...
April 21, 2017: Journal of Peptide Science: An Official Publication of the European Peptide Society
https://www.readbyqxmd.com/read/28421568/prions-prionoid-complexes-and-amyloids-the-bad-the-good-and-something-in-between
#11
Iva Hafner Bratkovič
Prions are infectious agents causing transmissible spongiform encephalopathies in humans and animals. These protein-based particles template conformational changes in a host-encoded prion protein to an insoluble self-like conformation. Prions are also present in yeast, where they support protein-based epigenetic inheritance. There is emerging evidence that prion-like (prionoid) particles can support a variety of pathological and beneficial functions. The recent data on the prionoid spread of other pathological amyloids are discussed in light of differences between prions and prion-like aggregates...
April 19, 2017: Swiss Medical Weekly
https://www.readbyqxmd.com/read/28421536/lysosomal-quality-control-in-prion-diseases
#12
REVIEW
Priyanka Majumder, Oishee Chakrabarti
Prion diseases are transmissible, familial or sporadic. The prion protein (PrP), a normal cell surface glycoprotein, is ubiquitously expressed throughout the body. While loss of function of PrP does not elicit apparent phenotypes, generation of misfolded forms of the protein or its aberrant metabolic isoforms has been implicated in a number of neurodegenerative disorders such as scrapie, kuru, Creutzfeldt-Jakob disease, fatal familial insomnia, Gerstmann-Sträussler-Scheinker and bovine spongiform encephalopathy...
April 18, 2017: Molecular Neurobiology
https://www.readbyqxmd.com/read/28413741/serial-magnetic-resonance-imaging-in-creutzfeldt-jakob-disease-a-case-report-and-literature-review
#13
Ahmed H Qavi, Tasnim F Imran, Zachariah Hasan, Fariha Ilyas, Usman Ghani, Salman Assad, Shabih Hasan
Creutzfeldt-Jakob disease (CJD) is a rare, degenerative, invariably fatal brain disorder. CJD usually appears in later life and runs a rapid course. Typically, the onset of symptoms occurs about age 60 and about 90% of individuals die within one year. We report a case of 67-year-old male presented with progressive aphasia, confusion, dysphagia and inability to carry out activities of daily life (ADLs) over a period of three to four weeks. The patient had past medical history of chronic atrial fibrillation and hypertension...
March 14, 2017: Curēus
https://www.readbyqxmd.com/read/28402718/prions-on-the-run-how-extracellular-vesicles-serve-as-delivery-vehicles-for-self-templating-protein-aggregates
#14
REVIEW
Shu Liu, André Hossinger, Sarah Göbbels, Ina M Vorberg
Extracellular vesicles (EVs) are actively secreted, membrane-bound communication vehicles that exchange biomolecules between cells. EVs also serve as dissemination vehicles for pathogens, including prions, proteinaceous infectious agents that cause transmissible spongiform encephalopathies (TSEs) in mammals. Increasing evidence accumulates that diverse protein aggregates associated with common neurodegenerative diseases are packaged into EVs as well. Vesicle-mediated intercellular transmission of protein aggregates can induce aggregation of homotypic proteins in acceptor cells and might thereby contribute to disease progression...
March 4, 2017: Prion
https://www.readbyqxmd.com/read/28402042/mm1-type-sporadic-creutzfeldt-jakob-disease-with-1-month-total-disease-duration-and-early-pathologic-indicators
#15
Yasushi Iwasaki, Hiroko Kato, Tetsuo Ando, Maya Mimuro, Tetsuyuki Kitamoto, Mari Yoshida
A 62-year-old man presented with abnormal behavior and cognitive impairment. Diffusion-weighted images (DWI) obtained on MRI showed extensive hyperintense regions in the cerebral cortex and striatum. Myoclonus was recognized, and the patient died 1 month after the onset; his condition did not reach the akinetic mutism state. The brain weighed 1300 g and showed no apparent atrophy. Extensive spongiform changes were observed in the cerebral neocortex, striatum, thalamus and cerebellar cortex, but gliosis was mild or absent...
April 12, 2017: Neuropathology: Official Journal of the Japanese Society of Neuropathology
https://www.readbyqxmd.com/read/28393682/new-feed-ingredients-the-insect-opportunity
#16
L W D van Raamsdonk, H J van der Fels-Klerx, J de Jong
In the framework of sustainability and a circular economy, new ingredients for feed are desired and, to this end, initiatives for implementing such novel ingredients have been started. The initiatives include a range of different sources, of which insects are of particular interest. Within the European Union, generally, a new feed ingredient should comply with legal constraints in terms of 'yes, provided that' its safety commits to a range of legal limits for heavy metals, mycotoxins, pesticides, contaminants, pathogens etc...
April 10, 2017: Food Additives & Contaminants. Part A, Chemistry, Analysis, Control, Exposure & Risk Assessment
https://www.readbyqxmd.com/read/28373833/the-biological-function-of-the-prion-protein-a-cell-surface-scaffold-of-signaling-modules
#17
Rafael Linden
The prion glycoprotein (PrP(C)) is mostly located at the cell surface, tethered to the plasma membrane through a glycosyl-phosphatydil inositol (GPI) anchor. Misfolding of PrP(C) is associated with the transmissible spongiform encephalopathies (TSEs), whereas its normal conformer serves as a receptor for oligomers of the β-amyloid peptide, which play a major role in the pathogenesis of Alzheimer's Disease (AD). PrP(C) is highly expressed in both the nervous and immune systems, as well as in other organs, but its functions are controversial...
2017: Frontiers in Molecular Neuroscience
https://www.readbyqxmd.com/read/28372173/species-specific-detection-of-processed-animal-proteins-in-feed-by-raman-spectroscopy
#18
Luisa Mandrile, Giuseppina Amato, Daniela Marchis, Gianmario Martra, Andrea Mario Rossi
The existing European Regulation (EC n° 51/2013) prohibits the use of animals meals in feedstuffs in order to prevent Bovine Spongiform Encephalopathy infection and diffusion, however the legislation is rapidly moving towards a partial lifting of the "feed ban" and the competent control organisms are urged to develop suitable analytical methods able to avoid food safety incidents related to animal origin products. The limitations of the official methods (i.e. light microscopy and Polymerase Chain Reaction) suggest exploring new analytic ways to get reliable results in a short time...
August 15, 2017: Food Chemistry
https://www.readbyqxmd.com/read/28358272/evaluation-of-rapid-post-mortem-test-kits-for-bovine-spongiform-encephalopathy-bse-screening-in-japan-their-analytical-sensitivity-to-atypical-bse-prions
#19
Ken'ichi Hagiwara, Yoshifumi Iwamaru, Naoko Tabeta, Takashi Yokoyama, Minoru Tobiume
A classical type of bovine spongiform encephalopathy (C-BSE), recognized in 1987, had a large impact on public health due to its zoonotic link to variant Creutzfeldt-Jakob disease by the human consumption of dietary products contaminated with the C-BSE prion. Thus, a number of countries implemented BSE surveillance using rapid post-mortem test kits that were approved for detection of the C-BSE prion in the cattle brain. However, as atypical BSE (L- and H-BSE) cases emerged in subsequent years, the efficacy of the kits for the detection of atypical BSE prions became a matter of concern...
March 4, 2017: Prion
https://www.readbyqxmd.com/read/28351484/hypocapnic-hypothesis-of-leigh-disease
#20
Ewa Pronicka
Leigh syndrome (LS) is a neurogenetic disorder of children caused by mutations in at least 75 genes which impair mitochondrial bioenergetics. The changes have typical localization in basal ganglia and brainstem, and typical histological picture of spongiform appearance, vascular proliferation and gliosis. ATP deprivation, free radicals and lactate accumulation are suspected to be the causes. Hypocapnic hypothesis proposed in the paper questions the energy deprivation as the mechanism of LS. We assume that the primary harmful factor is hypocapnia (decrease in pCO2) and respiratory alkalosis (increase in pH) due to hyperventilation, permanent or in response to stress...
April 2017: Medical Hypotheses
keyword
keyword
50169
1
2
Fetch more papers »
Fetching more papers... Fetching...
Read by QxMD. Sign in or create an account to discover new knowledge that matter to you.
Remove bar
Read by QxMD icon Read
×

Search Tips

Use Boolean operators: AND/OR

diabetic AND foot
diabetes OR diabetic

Exclude a word using the 'minus' sign

Virchow -triad

Use Parentheses

water AND (cup OR glass)

Add an asterisk (*) at end of a word to include word stems

Neuro* will search for Neurology, Neuroscientist, Neurological, and so on

Use quotes to search for an exact phrase

"primary prevention of cancer"
(heart or cardiac or cardio*) AND arrest -"American Heart Association"