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https://www.readbyqxmd.com/read/28731378/experimental-infection-of-cattle-with-a-novel-prion-derived-from-atypical-h-type-bovine-spongiform-encephalopathy
#1
Hiroyuki Okada, Kentaro Masujin, Kohtaro Miyazawa, Yoshihumi Iwamaru, Morikazu Imamura, Yuichi Matsuura, Shozo Arai, Shigeo Fukuda, Yuichi Murayama, Takashi Yokoyama
H-type bovine spongiform encephalopathy (H-BSE) is an atypical form of BSE in cattle. During passaging of H-BSE in transgenic bovinized (TgBoPrP) mice, a novel phenotype of BSE, termed BSE-SW emerged and was characterized by a short incubation time and host weight loss. To investigate the biological and biochemical properties of the BSE-SW prion, a transmission study was conducted in cattle, which were inoculated intracerebrally with brain homogenate from BSE-SW-infected TgBoPrP mice. The disease incubation period was approximately 15 months...
January 1, 2017: Veterinary Pathology
https://www.readbyqxmd.com/read/28729427/sialylated-glycosylphosphatidylinositols-suppress-the-production-of-toxic-amyloid-%C3%AE-oligomers
#2
William Nolan, Harriet McHale-Owen, Clive Bate
The production of amyloid-β (Aβ) is a key factor driving pathogenesis in Alzheimer's disease (AD). Increasing concentrations of soluble Aβ oligomers within the brain lead to synapse degeneration and the progressive dementia characteristic of AD. Since Aβ exists in both disease-relevant (toxic) and non-toxic forms, the factors that affected the release of toxic Aβ were studied in a cell model. 7PA2 cells expressing the human amyloid precursor protein released Aβ oligomers that caused synapse damage when incubated with cultured neurons...
July 20, 2017: Biochemical Journal
https://www.readbyqxmd.com/read/28726030/intravascular-large-b-cell-lymphoma-presenting-clinically-as-rapidly-progressive-dementia
#3
REVIEW
F M Brett, D Chen, T Loftus, Y Langan, S Looby, S Hutchinson
BACKGROUND: In patients presenting with rapidly progressive dementia, prion disease may enter the differential diagnosis. The commonest malignancies masquerading as prion disease are primary CNS lymphoma and intravascular large B-cell lymphoma, both rare and difficult to diagnose without brain biopsy. CASE PRESENTATION: This 82-year-old lady with a past history of hypertension, presented with rapidly progressive cognitive impairment and ataxia. The possibility of sCJD was raised...
July 19, 2017: Irish Journal of Medical Science
https://www.readbyqxmd.com/read/28724957/heterologous-prion-forming-proteins-interact-to-cross-seed-aggregation-in-saccharomyces-cerevisiae
#4
Kathryn M Keefer, Kevin C Stein, Heather L True
The early stages of protein misfolding remain incompletely understood, as most mammalian proteinopathies are only detected after irreversible protein aggregates have formed. Cross-seeding, where one aggregated protein templates the misfolding of a heterologous protein, is one mechanism proposed to stimulate protein aggregation and facilitate disease pathogenesis. Here, we demonstrate the existence of cross-seeding as a crucial step in the formation of the yeast prion [PSI (+)], formed by the translation termination factor Sup35...
July 19, 2017: Scientific Reports
https://www.readbyqxmd.com/read/28724625/amplification-of-misfolded-prion-proteins-in-blood-and-cerebrospinal-fluid-for-detection-of-creutzfeldt-jakob-disease
#5
Mari L DeMarco
No abstract text is available yet for this article.
July 19, 2017: Clinical Chemistry
https://www.readbyqxmd.com/read/28724527/the-spread-of-prion-like-proteins-by-lysosomes-and-tunneling-nanotubes-implications-for-neurodegenerative-diseases
#6
REVIEW
Guiliana Soraya Victoria, Chiara Zurzolo
Progression of pathology in neurodegenerative diseases is hypothesized to be a non-cell-autonomous process that may be mediated by the productive spreading of prion-like protein aggregates from a "donor cell" that is the source of misfolded aggregates to an "acceptor cell" in which misfolding is propagated by conversion of the normal protein. Although the proteins involved in the various diseases are unrelated, common pathways appear to be used for their intercellular propagation and spreading. Here, we summarize recent evidence of the molecular mechanisms relevant for the intercellular trafficking of protein aggregates involved in prion, Alzheimer's, Huntington's, and Parkinson's diseases...
July 19, 2017: Journal of Cell Biology
https://www.readbyqxmd.com/read/28722340/a-novel-small-molecule-inhibitor-of-prion-replication-and-mutant-prion-protein-toxicity
#7
Tania Massignan, Valeria Sangiovanni, Silvia Biggi, Claudia Stincardini, Saioa R Elezgarai, Giulia Maietta, Ivan A Andreev, Nina K Ratmanova, Dmitry S Belov, Evgeny R Lukyanenko, Grigory M Belov, Maria Letizia Barreca, Andrea Altieri, Alexander V Kurkin, Emiliano Biasini
Prion diseases are a class of neurodegenerative disorders characterized by the accumulation in the brain of a self-replicating, misfolded isoform (PrPSc) of the cellular prion protein (PrPC), a cell-surface glycoprotein of uncertain function. Emerging evidence suggests that PrPC may passively serve as a substrate for the replication of PrPSc, and actively transduce its toxic effects to neuronal cells. The vast majority of previous drug screening campaigns have only targeted PrPSc replication, with largely unsuccessful results...
July 19, 2017: ChemMedChem
https://www.readbyqxmd.com/read/28721847/classical-scrapie-transmission-in-arr-arr-genotype-sheep
#8
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/28721656/circrna-a-novel-type-of-biomarker-for-cancer
#9
REVIEW
He-da Zhang, Lin-Hong Jiang, Da-Wei Sun, Jun-Chen Hou, Zhen-Ling Ji
Circular RNAs (circRNAs) are a class of long, non-coding RNAs molecules that shape a covalently closed continuous loop which have no 5'-3' polarity and contain no polyA tail. CircRNAs also possess relatively jarless framework and are highly tissue-specific expressed in the eukaryotic transcriptome. Emerging evidences have discovered that thousands of endogenous circRNAs are present in mammalian cells and they mediate gene expression at the transcriptional or post-transcriptional level by binding to microRNAs or other molecules and then inhibit their function...
July 18, 2017: Breast Cancer: the Journal of the Japanese Breast Cancer Society
https://www.readbyqxmd.com/read/28716950/analysis-of-small-critical-regions-of-swi1-conferring-prion-formation-maintenance-and-transmission
#10
Stephanie Valtierra, Zhiqiang Du, Liming Li
Saccharomyces cerevisiae contains several prion elements, which are epigenetically transmitted as self-perpetuating protein conformations. One such prion is [SWI(+) ], whose protein determinant is Swi1, a subunit of the SWI/SNF chromatin-remodeling complex. We previously reported that [SWI(+) ] formation results in a partial loss-of-function phenotype of poor growth in non-glucose media and abolishment of multicellularity. We also showed that the first 38 amino acids of Swi1 propagated [SWI(+)]. We show here that a region as small as the first 32 amino acids of Swi1 (Swi11-32) can decorate [SWI(+)] aggregation and stably maintain and transmit [SWI(+)] independently of full-length Swi1...
July 17, 2017: Molecular and Cellular Biology
https://www.readbyqxmd.com/read/28715601/methamphetamine-increases-prion-protein-and-induces-dopamine-dependent-expression-of-protease-resistant-prpsc
#11
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
#12
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/28712869/evaluation-of-human-platelet-lysate-and-dimethyl-sulfoxide-as-cryoprotectants-for-the-cryopreservation-of-human-adipose-derived-stem-cells
#13
Chuan Wang, Ran Xiao, Yi-Lin Cao, Hong-Yu Yin
Cryopreservation provides an effective technique to maintain the functional properties of human adipose-derived stem cells (ASCs). Dimethylsulfoxide (DMSO) and fetal bovine serum (FBS) are frequently used as cryoprotectants for this purpose. However, the use of DMSO can result in adverse effects and toxic reactions and FBS can introduce risks of viral, prion, zoonose contaminations and evoke immune responses after injection. It is therefore crucial to reduce DMSO concentrations and use serum-free solution in the cryopreservation process...
July 13, 2017: Biochemical and Biophysical Research Communications
https://www.readbyqxmd.com/read/28711596/the-relevance-of-contact-independent-cell-to-cell-transfer-of-tdp-43-and-sod1-in-amyotrophic-lateral-sclerosis
#14
REVIEW
Maya A Hanspal, Christopher M Dobson, Justin J Yerbury, Janet R Kumita
Amyotrophic lateral sclerosis (ALS) is a neurodegenerative disease involving the formation of cytoplasmic aggregates by proteins including TDP-43 and SOD1, in affected cells in the central nervous system (CNS). Pathology spreads from an initial site of onset to contiguous anatomical regions. There is evidence that for disease-associated proteins, including TDP-43 and SOD1, non-native protein conformers can promote misfolding of the natively folded counterparts, and cell-to-cell transfer of pathological aggregates may underlie the spread of the disease throughout the CNS...
July 12, 2017: Biochimica et Biophysica Acta
https://www.readbyqxmd.com/read/28710688/death-by-food
#15
Roger W Byard
Although death from food is not an uncommon finding in forensic facilities worldwide, the range of underlying lethal mechanisms and associated conditions that should be sought at the time of autopsy is quite disparate. Deaths may occur from i) infectious agents including bacteria, viruses, protozoa, cestodes, nematodes and prions; ii) natural toxins including amanita toxins, tetrodotoxin, ciguatera and scombroid; iii) anaphylaxis; iv) poisoning; v) mechanical issues around airway and gut obstruction and/or perforation; and vi) miscellaneous causes...
July 15, 2017: Forensic Science, Medicine, and Pathology
https://www.readbyqxmd.com/read/28710326/cortical-influences-drive-amyotrophic-lateral-sclerosis
#16
REVIEW
Andrew Eisen, Heiko Braak, Kelly Del Tredici, Roger Lemon, Albert C Ludolph, Matthew C Kiernan
The early motor manifestations of sporadic amyotrophic lateral sclerosis (ALS), while rarely documented, reflect failure of adaptive complex motor skills. The development of these skills correlates with progressive evolution of a direct corticomotoneuronal system that is unique to primates and markedly enhanced in humans. The failure of this system in ALS may translate into the split hand presentation, gait disturbance, split leg syndrome and bulbar symptomatology related to vocalisation and breathing, and possibly diffuse fasciculation, characteristic of ALS...
July 14, 2017: Journal of Neurology, Neurosurgery, and Psychiatry
https://www.readbyqxmd.com/read/28710283/amorphous-protein-aggregates-stimulate-plasminogen-activation-leading-to-release-of-cytotoxic-fragments-that-are-clients-for-extracellular-chaperones
#17
Patrick Constantinescu, Rebecca A Brown, Amy R Wyatt, Marie Ranson, Mark R Wilson
The misfolding of proteins and their accumulation in extracellular tissue compartments as insoluble amyloid or amorphous protein aggregates is a hallmark feature of many debilitating protein deposition diseases such as Alzheimers disease, prion diseases and type II diabetes. The plasminogen activation system (PAS) is best known as an extracellular fibrinolytic system, but was previously reported to also be capable of degrading amyloid fibrils. Here we show that amorphous protein aggregates interact with tPA and plasminogen, via an exposed lysine dependent mechanism, to efficiently generate plasmin...
July 14, 2017: Journal of Biological Chemistry
https://www.readbyqxmd.com/read/28709995/the-contribution-of-biophysical-and-structural-studies-of-protein-self-assembly-to-the-design-of-therapeutic-strategies-for-amyloid-diseases
#18
Nunilo Cremades, Christopher M Dobson
Many neurodegenerative disorders, including Alzheimer's, Parkinson's and the prion diseases, are characterized by a conformational conversion of normally soluble proteins or peptides into pathological species, by a process of misfolding and self-assembly that leads ultimately to the formation of amyloid fibrils. Recent studies support the idea that multiple intermediate species with a wide variety of degrees of neuronal toxicity are generated during such processes. The development of a high level of knowledge of the nature and structure of the pathogenic amyloid species would significantly enhance efforts to underline the molecular origins of these disorders and also to develop both accurate diagnoses and effective therapeutic interventions for these types of conditions...
July 12, 2017: Neurobiology of Disease
https://www.readbyqxmd.com/read/28709498/targeting-fyn-kinase-in-alzheimer-s-disease
#19
REVIEW
Haakon B Nygaard
The past decade has brought tremendous progress in unraveling the pathophysiology of Alzheimer's disease (AD). While increasingly sophisticated immunotherapy targeting soluble and aggregated brain amyloid-beta (Aβ) continues to dominate clinical research in AD, a deeper understanding of Aβ physiology has led to the recognition of distinct neuronal signaling pathways linking Aβ to synaptotoxicity and neurodegeneration and to new targets for therapeutic intervention. Identifying specific signaling pathways involving Aβ has allowed for the development of more precise therapeutic interventions targeting the most relevant molecular mechanisms leading to AD...
June 13, 2017: Biological Psychiatry
https://www.readbyqxmd.com/read/28708131/a-clinicopathological-approach-to-the-diagnosis-of-dementia
#20
REVIEW
Fanny M Elahi, Bruce L Miller
The most definitive classification systems for dementia are based on the underlying pathology which, in turn, is categorized largely according to the observed accumulation of abnormal protein aggregates in neurons and glia. These aggregates perturb molecular processes, cellular functions and, ultimately, cell survival, with ensuing disruption of large-scale neural networks subserving cognitive, behavioural and sensorimotor functions. The functional domains affected and the evolution of deficits in these domains over time serve as footprints that the clinician can trace back with various levels of certainty to the underlying neuropathology...
July 14, 2017: Nature Reviews. Neurology
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