keyword
MENU ▼
Read by QxMD icon Read
search

Prion

keyword
https://www.readbyqxmd.com/read/29331212/prion-protein-as-a-toxic-acceptor-of-amyloid-%C3%AE-oligomers
#1
REVIEW
Silvia A Purro, Andrew J Nicoll, John Collinge
The initial report that cellular prion protein (PrPC) mediates toxicity of amyloid-β species linked to Alzheimer's disease was initially treated with scepticism, but growing evidence supports this claim. That there is a high-affinity interaction is now clear, and its molecular basis is being unraveled, while recent studies have identified possible downstream toxic mechanisms. Determination of the clinical significance of such interactions between PrPC and disease-associated amyloid-β species will require experimental medicine studies in humans...
February 15, 2018: Biological Psychiatry
https://www.readbyqxmd.com/read/29330304/efficient-prion-disease-transmission-through-common-environmental-materials
#2
Sandra Pritzkow, Rodrigo Morales, Adam Lyon, Luis Concha-Marambio, Akihiko Urayama, Claudio Soto
Prion diseases are a group of fatal neurodegenerative diseases associated with a protein-based infectious agent, termed prion. Compelling evidence suggests that natural transmission of prion diseases is mediated by environmental contamination with infectious prions. We hypothesized that several natural and man-made materials, commonly found in the environments of wild and captive animals, can bind prions and may act as vectors for disease transmission. To test our hypothesis, we exposed surfaces composed of various common environmental materials (i...
January 12, 2018: Journal of Biological Chemistry
https://www.readbyqxmd.com/read/29330303/mammalian-amyloidogenic-proteins-promote-prion-nucleation-in-yeast
#3
Pavithra Chandramowlishwaran, Meng Sun, Kristin L Casey, Andrey V Romanyuk, Anastasiya V Grizel, Julia V Sopova, Aleksandr A Rubel, Carmen Nussbaum-Krammer, Ina M Vorberg, Yury O Chernoff
Fibrous cross-β aggregates (amyloids) and their transmissible forms (prions) cause diseases in mammals (including humans) and control heritable traits in yeast. Initial nucleation of a yeast prion by transiently overproduced prion-forming protein or its (typically, QN-rich) prion domain is efficient only in the presence of another aggregated (in most cases, QN-rich) protein. Here, we demonstrate that a fusion of the prion domain of yeast protein Sup35 to some non-QN-rich mammalian proteins, associated with amyloid diseases, promotes nucleation of Sup35 prions in the absence of preexisting aggregates...
January 12, 2018: Journal of Biological Chemistry
https://www.readbyqxmd.com/read/29330300/real-time-imaging-of-yeast-cells-reveals-several-distinct-mechanisms-of-curing-of-the-ure3-prion
#4
Xiaohong Zhao, Jenna Lanz, Danielle Steinberg, Tyler Pease, Joseph M Ahearn, Evgeny E Bezsonov, Elena Staguhn, Evan Eisenberg, Daniel C Masison, Lois E Greene
The [URE3] yeast prion is the self-propagating amyloid form of the Ure2 protein. [URE3] is cured by overexpression of several yeast proteins, including Ydj1, Btn2, Cur1, Hsp42, and human DnaJB6. To better understand [URE3] curing, we used real-time imaging with a yeast strain expressing a GFP-labeled full-length Ure2 construct to monitor the curing of [URE3] over time. [URE3] yeast cells exhibited numerous fluorescent foci, and expression of the GFP-labeled Ure2 affected neither mitotic stability of [URE3] nor the rate of [URE3] curing by the curing proteins...
January 12, 2018: Journal of Biological Chemistry
https://www.readbyqxmd.com/read/29329958/contact-tracing-for-the-control-of-infectious-disease-epidemics-chronic-wasting-disease-in-deer-farms
#5
Chris Rorres, Maria Romano, Jennifer A Miller, Jana M Mossey, Tony H Grubesic, David E Zellner, Gary Smith
Contact tracing is a crucial component of the control of many infectious diseases, but is an arduous and time consuming process. Procedures that increase the efficiency of contact tracing increase the chance that effective controls can be implemented sooner and thus reduce the magnitude of the epidemic. We illustrate a procedure using Graph Theory in the context of infectious disease epidemics of farmed animals in which the epidemics are driven mainly by the shipment of animals between farms. Specifically, we created a directed graph of the recorded shipments of deer between deer farms in Pennsylvania over a timeframe and asked how the properties of the graph could be exploited to make contact tracing more efficient should Chronic Wasting Disease (a prion disease of deer) be discovered in one of the farms...
December 14, 2017: Epidemics
https://www.readbyqxmd.com/read/29329906/development-of-a-quick-bioassay-for-the-evaluation-of-transmission-properties-of-acquired-prion-diseases
#6
Yoshiko Munesue, Taishi Shimazaki, Zechen Qi, Norikazu Isoda, Hirofumi Sawa, Keisuke Aoshima, Takashi Kimura, Shirou Mohri, Tetsuyuki Kitamoto, Atsushi Kobayashi
Evaluation of transmission properties is important for the differential diagnosis of a subgroup of acquired Creutzfeldt-Jakob disease (CJD) with methionine homozygosity at polymorphic codon 129 of the PRNP gene, an intermediate type abnormal prion protein (PrP), and kuru plaques, denoted as acquired CJD-MMiK. The present study aimed to develop a quick evaluation system of the transmission properties of acquired CJD-MMiK. In the PrP-humanized mice intraperitoneally inoculated with brain homogenates from an acquired CJD-MMiK patient, accumulation of abnormal PrP was observed in follicular dendritic cells of the spleen at 75 days post-inoculation...
January 9, 2018: Neuroscience Letters
https://www.readbyqxmd.com/read/29321951/utility-of-the-phenacyl-protecting-group-in-traceless-protein-semisynthesis-through-ligation-desulfurization-chemistry
#7
Maria Matveenko, Stefanie Hackl, Christian F W Becker
Semisynthesis of proteins via expressed protein ligation is a widely applicable method, even more so because of the possibility of ligation at non-cysteine sites using β-mercapto amino acids that can be converted to the corresponding native amino acids by desulfurization. A drawback of this ligation- desulfurization approach is the removal of any unprotected native cysteine residues within the ligated protein segments. Here, we show that the phenacyl (PAc) moiety can be successfully used to protect cysteines within recombinantly generated protein segments...
January 2018: ChemistryOpen
https://www.readbyqxmd.com/read/29312526/amyotrophic-lateral-sclerosis-as-a-protein-level-non-genomic-disease-therapy-with-s2rm-exosome-released-molecules
#8
REVIEW
Greg Maguire
Amyotrophic lateral sclerosis (ALS) is a rapidly progressing neurodegenerative disease that leads to death. No effective treatments are currently available. Based on data from epidemiological, etiological, laboratory, and clinical studies, I offer a new way of thinking about ALS and its treatment. This paper describes a host of extrinsic factors, including the exposome, that disrupt the extracellular matrix and protein function such that a spreading, prion-like disease leads to neurodegeneration in the motor tracts...
November 26, 2017: World Journal of Stem Cells
https://www.readbyqxmd.com/read/29311311/structural-heterogeneity-and-intersubject-variability-of-a%C3%AE-in-familial-and-sporadic-alzheimer-s-disease
#9
Carlo Condello, Thomas Lemmin, Jan Stöhr, Mimi Nick, Yibing Wu, Alison M Maxwell, Joel C Watts, Christoffer D Caro, Abby Oehler, C Dirk Keene, Thomas D Bird, Sjoerd G van Duinen, Lars Lannfelt, Martin Ingelsson, Caroline Graff, Kurt Giles, William F DeGrado, Stanley B Prusiner
Point mutations in the amyloid-β (Aβ) coding region produce a combination of mutant and WT Aβ isoforms that yield unique clinicopathologies in familial Alzheimer's disease (fAD) and cerebral amyloid angiopathy (fCAA) patients. Here, we report a method to investigate the structural variability of amyloid deposits found in fAD, fCAA, and sporadic AD (sAD). Using this approach, we demonstrate that mutant Aβ determines WT Aβ conformation through prion template-directed misfolding. Using principal component analysis of multiple structure-sensitive fluorescent amyloid-binding dyes, we assessed the conformational variability of Aβ deposits in fAD, fCAA, and sAD patients...
January 8, 2018: Proceedings of the National Academy of Sciences of the United States of America
https://www.readbyqxmd.com/read/29310723/iatrogenic-creutzfeldt-jakob-disease-with-amyloid-%C3%AE-pathology-an-international-study
#10
Ignazio Cali, Mark L Cohen, Stéphane Haїk, Piero Parchi, Giorgio Giaccone, Steven J Collins, Diane Kofskey, Han Wang, Catriona A McLean, Jean-Philippe Brandel, Nicolas Privat, Véronique Sazdovitch, Charles Duyckaerts, Tetsuyuki Kitamoto, Ermias D Belay, Ryan A Maddox, Fabrizio Tagliavini, Maurizio Pocchiari, Ellen Leschek, Brian S Appleby, Jiri G Safar, Lawrence B Schonberger, Pierluigi Gambetti
The presence of pathology related to the deposition of amyloid-β (Aβ) has been recently reported in iatrogenic Creutzfeldt-Jakob disease (iCJD) acquired from inoculation of growth hormone (GH) extracted from human cadaveric pituitary gland or use of cadaveric dura mater (DM) grafts.To investigate this phenomenon further, a cohort of 27 iCJD cases - 21 with adequate number of histopathological sections - originating from Australia, France, Italy, and the Unites States, were examined by immunohistochemistry, amyloid staining, and Western blot analysis of the scrapie prion protein (PrPSc), and compared with age-group matched cases of sporadic CJD (sCJD), Alzheimer disease (AD) or free of neurodegenerative diseases (non-ND)...
January 8, 2018: Acta Neuropathologica Communications
https://www.readbyqxmd.com/read/29310497/differential-effects-of-divalent-cations-on-elk-prion-protein-fibril-formation-and-stability
#11
Daniel Samorodnitsky, Eric M Nicholson
Misfolding of the normally folded prion protein of mammals (PrPC) into infectious fibrils causes a variety of diseases, from scrapie in sheep to chronic wasting disease (CWD) in cervids. The misfolded form of PrPC, termed PrPSc, or in this case PrPCWD, interacts with PrPC to create more PrPCWD. This process is not clearly defined but is affected by the presence and interactions of biotic and abiotic cofactors. These include nucleic acids, lipids, glycosylation, pH, and ionic character. PrPC has been shown to act as a copper-binding protein in vivo, though it also binds to other divalents as well...
January 9, 2018: Prion
https://www.readbyqxmd.com/read/29310343/cerebrospinal-fluid-real-time-quaking-induced-conversion-test-for-sporadic-creutzfeldt-jakob-disease-in-an-18-year-old-woman-a-case-report
#12
Yuan Yao, Xiaoping Dong, Hongzhi Guan, Qiang Lu
RATIONALE: Sporadic Creutzfeldt-Jakob disease (sCJD) mainly occurs in the elderly, with the peak age of onset ranging from 55 to 75 years. The symptoms of sCJD are not unique, and laboratory tests such as magnetic resonance imaging (MRI), electroencephalogram (EEG) and cerebrospinal fluid (CSF)14-3-3 protein have low sensitivity or specificity. Therefore, excluding treatable diseases and establishing a diagnosis could be difficult in young patients with suspected sCJD. Recently, real-time quaking-induced conversion (RT-QuIC) has been used in the diagnosis of sCJD, with more than 95% sensitivity and 100% specificity...
December 2017: Medicine (Baltimore)
https://www.readbyqxmd.com/read/29310300/a-new-electrochemical-immunosensor-for-sensitive-detection-of-prion-based-on-prussian-blue-analogue
#13
Junjing Li, Xiaoxia Yan, Xiaoyu Li, Xiaohua Zhang, Jinhua Chen
Based on Co-Co Prussian blue analogue (Co-Co PBA), a novel immunosensor has been developed for sensitive detection of prion protein (PrPC). Gold nanoparticles (AuNPs)-modified Co-Co PBA nanocubes (PBA-AuNPs) worked as a support of the antibody (Ab2) of PrPC to obtain Ab2-PBA-AuNPs composite and also as the signal source for PrPC assay. When PrPC existed, Ab2-PBA-AuNPs could be introduced to the surface of another antibody of PrPC (Ab1) modified AuNPs/GC electrode (the gold nanoparticles-modified glassy carbon electrode) through specific antigen-antibody interaction between PrPC and its antibodies to form the Ab1-PrPC-Ab2 sandwich structure...
March 1, 2018: Talanta
https://www.readbyqxmd.com/read/29308725/the-role-of-the-prion-protein-in-the-internalization-of-%C3%AE-synuclein-amyloids
#14
Elena De Cecco, Giuseppe Legname
Synucleinopathies are a group of neurodegenerative diseases characterized by the accumulation of α-synuclein amyloids in several regions of the brain. α-Synuclein fibrils are able to spread via cell-to-cell transfer, and once inside the cells, they can template the misfolding and aggregation of the endogenous α-synuclein. Multiple mechanisms have been shown to participate in the process of propagation: endocytosis, tunneling nanotubes and micropinocytosis. Recently, we published a research showing that the cellular form of the prion protein (PrPC) acts as a receptor for α-synuclein amyloid fibrils, facilitating their internalization through and endocytic pathway...
January 8, 2018: Prion
https://www.readbyqxmd.com/read/29308690/overexpression-of-a-conserved-hsp40-chaperone-reduces-toxicity-of-several-neurodegenerative-disease-proteins
#15
Sei-Kyoung Park, Fatih Arslan, Vydehi Kanneganti, Sami J Barmada, Pravinkumar Purushothaman, Subhash Chandra Verma, Susan W Liebman
TDP-43 and FUS are DNA/RNA binding proteins associated with neuronal inclusions in amyotrophic lateral sclerosis (ALS) patients. Other neurodegenerative diseases are also characterized by neuronal protein aggregates, e.g. Huntington's disease, associated with polyglutamine (polyQ) expansions in the protein huntingtin. Here we discuss our recent paper establishing similarities between aggregates of TDP-43 that have short glutamine and asparagine (Q/N)-rich modules and are soluble in detergents, with those of polyQ and PIN4C that have large Q/N-rich domains and are detergent-insoluble...
January 8, 2018: Prion
https://www.readbyqxmd.com/read/29304167/the-language-disorder-of-prion-disease-is-characteristic-of-a-dynamic-aphasia-and-is-rarely-an-isolated-clinical-feature
#16
Diana Caine, Akin Nihat, Philippa Crabb, Peter Rudge, Lisa Cipolotti, John Collinge, Simon Mead
BACKGROUND: Akinetic mutism is a key diagnostic feature of prion diseases, however, their rapidly progressive nature makes detailed investigation of the language disorder in a large cohort extremely challenging. This study aims to position prion diseases in the nosology of language disorders and improve early clinical recognition. METHODS: A systematic, prospective investigation of language disorders in a large cohort of patients diagnosed with prion diseases. 568 patients were included as a sub-study of the National Prion Monitoring Cohort...
2018: PloS One
https://www.readbyqxmd.com/read/29303785/mechanisms-of-disordered-neurodegenerative-function-concepts-and-facts-about-the-different-roles-of-the-protein-kinase-rna-like-endoplasmic-reticulum-kinase-perk
#17
Yasmeen M Taalab, Nour Ibrahim, Ahmed Maher, Mubashir Hassan, Wael Mohamed, Ahmed A Moustafa, Mohamed Salama, Dina Johar, Larry Bernstein
Neurodegenerative diseases, such as Alzheimer's disease, Huntington's disease, Parkinson's disease, prion disease, and amyotrophic lateral sclerosis, are a dissimilar group of disorders that share a hallmark feature of accumulation of abnormal intraneuronal or extraneuronal misfolded/unfolded protein and are classified as protein misfolding disorders. Cellular and endoplasmic reticulum (ER) stress activates multiple signaling cascades of the unfolded protein response (UPR). Consequently, translational and transcriptional alterations in target gene expression occur in response directed toward restoring the ER capacity of proteostasis and reestablishing the cellular homeostasis...
January 5, 2018: Reviews in the Neurosciences
https://www.readbyqxmd.com/read/29301985/phase-separation-of-a-yeast-prion-protein-promotes-cellular-fitness
#18
Titus M Franzmann, Marcus Jahnel, Andrei Pozniakovsky, Julia Mahamid, Alex S Holehouse, Elisabeth Nüske, Doris Richter, Wolfgang Baumeister, Stephan W Grill, Rohit V Pappu, Anthony A Hyman, Simon Alberti
Despite the important role of prion domains in neurodegenerative disease, their physiological function has remained enigmatic. Previous work with yeast prions has defined prion domains as sequences that form self-propagating aggregates. Here, we uncovered an unexpected function of the canonical yeast prion protein Sup35. In stressed conditions, Sup35 formed protective gels via pH-regulated liquid-like phase separation followed by gelation. Phase separation was mediated by the N-terminal prion domain and regulated by the adjacent pH sensor domain...
January 5, 2018: Science
https://www.readbyqxmd.com/read/29301257/prion-strains-and-transmission-barrier-phenomena
#19
REVIEW
Angélique Igel-Egalon, Vincent Béringue, Human Rezaei, Pierre Sibille
Several experimental evidences show that prions are non-conventional pathogens, which physical support consists only in proteins. This finding raised questions regarding the observed prion strain-to-strain variations and the species barrier that happened to be crossed with dramatic consequences on human health and veterinary policies during the last 3 decades. This review presents a focus on a few advances in the field of prion structure and prion strains characterization: from the historical approaches that allowed the concept of prion strains to emerge, to the last results demonstrating that a prion strain may in fact be a combination of a few quasi species with subtle biophysical specificities...
January 1, 2018: Pathogens
https://www.readbyqxmd.com/read/29300791/chaperone-functional-specificity-promotes-yeast-prion-diversity
#20
Andrea N Killian, Justin K Hines
No abstract text is available yet for this article.
January 2018: PLoS Pathogens
keyword
keyword
2473
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"