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https://www.readbyqxmd.com/read/28333162/the-endoplasmic-reticulum-chaperone-grp78-bip-modulates-prion-propagation-in-vitro-and-in-vivo
#1
Kyung-Won Park, Gyoung Eun Kim, Rodrigo Morales, Fabio Moda, Ines Moreno-Gonzalez, Luis Concha-Marambio, Amy S Lee, Claudio Hetz, Claudio Soto
Prion diseases are fatal neurodegenerative disorders affecting several mammalian species, characterized by the accumulation of the misfolded form of the prion protein, which is followed by the induction of endoplasmic reticulum (ER) stress and the activation of the unfolded protein response (UPR). GRP78, also called BiP, is a master regulator of the UPR, reducing ER stress levels and apoptosis due to an enhancement of the cellular folding capacity. Here, we studied the role of GRP78 in prion diseases using several in vivo and in vitro approaches...
March 23, 2017: Scientific Reports
https://www.readbyqxmd.com/read/28330994/combining-dnp-nmr-with-segmental-and-specific-labeling-to-study-a-yeast-prion-protein-strain-that-is-not-parallel-in-register
#2
Kendra K Frederick, Vladimir K Michaelis, Marc A Caporini, Loren B Andreas, Galia T Debelouchina, Robert G Griffin, Susan Lindquist
The yeast prion protein Sup35NM is a self-propagating amyloid. Despite intense study, there is no consensus on the organization of monomers within Sup35NM fibrils. Some studies point to a β-helical arrangement, whereas others suggest a parallel in-register organization. Intermolecular contacts are often determined by experiments that probe long-range heteronuclear contacts for fibrils templated from a 1:1 mixture of (13)C- and (15)N-labeled monomers. However, for Sup35NM, like many large proteins, chemical shift degeneracy limits the usefulness of this approach...
March 22, 2017: Proceedings of the National Academy of Sciences of the United States of America
https://www.readbyqxmd.com/read/28324664/limited-understanding-of-the-functional-diversity-of-n-linked-glycans-as-a-major-gap-of-prion-biology
#3
Ilia V Baskakov
Among a broad range of hypotheses on the molecular nature of transmissible spongiform encephalopathy or scrapie agents discussed in 1960s was a hypothesis of self-replicating polysaccharides. While the studies of the past 40 years provided unambiguous proof that this is not the case, emerging evidence suggests that carbohydrates in the form of sialylated N-linked glycans, which are a constitutive part of mammalian prions or PrP(Sc), are essential in determining prion fate in an organism. The current extra-view article discusses recent advancements on the role of N-linked glycans and specifically their sialylation status in controlling prion fate...
March 21, 2017: Prion
https://www.readbyqxmd.com/read/28324299/fatal-familial-insomnia-clinical-aspects-and-molecular-alterations
#4
REVIEW
Franc Llorens, Juan-José Zarranz, Andre Fischer, Inga Zerr, Isidro Ferrer
PURPOSE OF REVIEW: Fatal familiar insomnia (FFI) is an autosomal dominant inherited prion disease caused by D178N mutation in the prion protein gene (PRNP D178N) accompanied by the presence of a methionine at the codon 129 polymorphic site on the mutated allele. FFI is characterized by severe sleep disorder, dysautonomia, motor signs and abnormal behaviour together with primary atrophy of selected thalamic nuclei and inferior olives, and expansion to other brain regions with disease progression...
April 2017: Current Neurology and Neuroscience Reports
https://www.readbyqxmd.com/read/28321768/validation-of-%C3%AE-synuclein-as-a-csf-biomarker-for-sporadic-creutzfeldt-jakob-disease
#5
Franc Llorens, Niels Kruse, André Karch, Matthias Schmitz, Saima Zafar, Nadine Gotzmann, Ting Sun, Silja Köchy, Tobias Knipper, Maria Cramm, Ewa Golanska, Beata Sikorska, Pawel P Liberski, Raquel Sánchez-Valle, Andre Fischer, Brit Mollenhauer, Inga Zerr
The analysis of cerebrospinal fluid (CSF) biomarkers gains importance in the differential diagnosis of prion diseases. However, no single diagnostic tool or combination of them can unequivocally confirm prion disease diagnosis. Electrochemiluminescence (ECL)-based immunoassays have demonstrated to achieve high diagnostic accuracy in a variety of sample types due to their high sensitivity and dynamic range. Quantification of CSF α-synuclein (a-syn) by an in-house ECL-based ELISA assay has been recently reported as an excellent approach for the diagnosis of sporadic Creutzfeldt-Jakob disease (sCJD), the most prevalent form of human prion disease...
March 21, 2017: Molecular Neurobiology
https://www.readbyqxmd.com/read/28320943/prion-protein-dynamics-before-aggregation
#6
Kinshuk Raj Srivastava, Lisa J Lapidus
Prion diseases, like Alzheimer's disease and Parkinson disease, are rapidly progressive neurodegenerative disorders caused by misfolding followed by aggregation and accumulation of protein deposits in neuronal cells. Here we measure intramolecular polypeptide backbone reconfiguration as a way to understand the molecular basis of prion aggregation. Our hypothesis is that when reconfiguration is either much faster or much slower than bimolecular diffusion, biomolecular association is not stable, but as the reconfiguration rate becomes similar to the rate of biomolecular diffusion, the association is more stable and subsequent aggregation is faster...
March 20, 2017: Proceedings of the National Academy of Sciences of the United States of America
https://www.readbyqxmd.com/read/28320296/biological-basis-for-amyloidogenesis-in-alzheimer-s-disease
#7
REVIEW
T V Andreeva, W J Lukiw, E I Rogaev
Certain cellular proteins normally soluble in the living organism under certain conditions form aggregates with a specific cross-β sheet structure called amyloid. These intra- or extracellular insoluble aggregates (fibers or plaques) are hallmarks of many neurodegenerative pathologies including Alzheimer's disease (AD), Huntington's disease, Parkinson's disease, prion disease, and other progressive neurological diseases that develop in the aging human central nervous system. Amyloid diseases (amyloidoses) are widespread in the elderly human population, a rapidly expanding demographic in many global populations...
February 2017: Biochemistry. Biokhimii︠a︡
https://www.readbyqxmd.com/read/28320265/role-of-atypical-protein-kinases-in-maintenance-of-long-term-memory-and-synaptic-plasticity
#8
REVIEW
A A Borodinova, A B Zuzina, P M Balaban
Investigation of biochemical mechanisms underlying the long-term storage of information in nervous system is one of main problems of modern neurobiology. As a molecular basis of long-term memory, long-term changes in kinase activities, increase in the level and changes in the subunit composition of receptors in synaptic membranes, local activity of prion-like proteins, and epigenetic modifications of chromatin have been proposed. Perhaps a combination of all or of some of these factors underlies the storage of long-term memory in the brain...
March 2017: Biochemistry. Biokhimii︠a︡
https://www.readbyqxmd.com/read/28314768/pseudomonas-aeruginosa-infection-liberates-transmissible-cytotoxic-prion-amyloids
#9
Ron Balczon, K Adam Morrow, Chun Zhou, Bradley Edmonds, Mikhail Alexeyev, Jean-Francois Pittet, Brant M Wagener, Stephen A Moser, Silas Leavesley, Xiangming Zha, Dara W Frank, Troy Stevens
Patients who recover from pneumonia subsequently have elevated rates of death after hospital discharge as a result of secondary organ damage, the causes of which are unknown. We used the bacterium Pseudomonas aeruginosa, a common cause of hospital-acquired pneumonia, as a model for investigating this phenomenon. We show that infection of pulmonary endothelial cells by P. aeruginosa induces production and release of a cytotoxic amyloid molecule with prion characteristics, including resistance to various nucleases and proteases...
March 17, 2017: FASEB Journal: Official Publication of the Federation of American Societies for Experimental Biology
https://www.readbyqxmd.com/read/28300846/regulation-of-rhoa-activity-by-the-cellular-prion-protein
#10
Hee-Jun Kim, Hong-Seok Choi, Jeong-Ho Park, Mo-Jong Kim, Hyoung-Gon Lee, Robert Bob Petersen, Yong-Sun Kim, Jae-Bong Park, Eun-Kyoung Choi
The cellular prion protein (PrP(C)) is a highly conserved glycosylphosphatidylinositol (GPI)-anchored membrane protein that is involved in the signal transduction during the initial phase of neurite outgrowth. The Ras homolog gene family member A (RhoA) is a small GTPase that is known to have an essential role in regulating the development, differentiation, survival, and death of neurons in the central nervous system. Although recent studies have shown the dysregulation of RhoA in a variety of neurodegenerative diseases, the role of RhoA in prion pathogenesis remains unclear...
March 16, 2017: Cell Death & Disease
https://www.readbyqxmd.com/read/28298604/prion-strain-characterization-of-a-novel-subtype-of-creutzfeldt-jakob-disease
#11
Roberta Galeno, Michele Angelo Di Bari, Romolo Nonno, Franco Cardone, Marco Sbriccoli, Silvia Graziano, Loredana Ingrosso, Michele Fiorini, Angelina Valanzano, Giulia Pasini, Anna Poleggi, Ramona Vinci, Anna Ladogana, Maria Puopolo, Salvatore Monaco, Umberto Agrimi, Gianluigi Zanusso, Maurizio Pocchiari
In 2007, we reported a patient with an atypical form of Creutzfeldt-Jakob disease (CJD), heterozygous for methionine-valine (MV) at codon 129, who showed a novel pathological prion protein (PrP(TSE)) conformation with an atypical glycoform (AG) profile and an intraneuronal PrP deposition. In the present study, we further characterized the conformational properties of this pathological prion protein (PrP(TSE) MV(AG)) showing that PrP(TSE) MV(AG) is composed by multiple conformers with biochemical properties distinct from PrP(TSE) type 1 and type 2 of MV sporadic CJD (sCJD)...
March 15, 2017: Journal of Virology
https://www.readbyqxmd.com/read/28294542/pras40-alleviates-neurotoxic-prion-peptide-induced-apoptosis-via-mtor-akt-signaling
#12
Wei Yang, Li-Feng Yang, Zhi-Qi Song, Syed Zahid Ali Shah, Yong-Yong Cui, Chao-Si Li, Hua-Fen Zhao, Hong-Li Gao, Xiang-Mei Zhou, De-Ming Zhao
AIMS: The proline-rich Akt substrate of 40-kDa (PRAS40) protein is a direct inhibitor of mTORC1 and an interactive linker between the Akt and mTOR pathways. The mammalian target of rapamycin (mTOR) is considered to be a central regulator of cell growth and metabolism. Several investigations have demonstrated that abnormal mTOR activity may contribute to the pathogenesis of several neurodegenerative disorders and lead to cognitive deficits. METHODS: Here, we used the PrP peptide 106-126 (PrP(106-126) ) in a cell model of prion diseases (also known as transmissible spongiform encephalopathies, TSEs) to investigate the mechanisms of mTOR-mediated cell death in prion diseases...
March 14, 2017: CNS Neuroscience & Therapeutics
https://www.readbyqxmd.com/read/28292187/distinct-mechanisms-determine-%C3%AE-synuclein-fibril-morphology-during-growth-and-maturation
#13
Arshdeep Sidhu, Ine Segers-Nolten, Vincent Raussens, Mireille M A E Claessens, Vinod Subramaniam
Amyloid polymorphs have become one of the focal points of molecular studies of neurodegenerative diseases like Parkinson's disease. Due to their distinct biochemical properties and prion-like characteristics, insights into the molecular origin and stability of amyloid polymorphs over time are crucial for understanding the potential role of amyloid polymorphism in these diseases. Here, we systematically study the fibrillization of recombinantly produced human α-synuclein (αSyn) over an extended period of time to unravel the origin and temporal evolution of polymorphism...
March 15, 2017: ACS Chemical Neuroscience
https://www.readbyqxmd.com/read/28291503/regenerative-and-antibacterial-properties-of-acellular-fish-skin-grafts-and-human-amnion-chorion-membrane-implications-for-tissue-preservation-in-combat-casualty-care
#14
Skuli Magnusson, Baldur Tumi Baldursson, Hilmar Kjartansson, Ottar Rolfsson, Gudmundur Fertram Sigurjonsson
BACKGROUND: Improvised explosive devices and new directed energy weapons are changing warfare injuries from penetrating wounds to large surface area thermal and blast injuries. Acellular fish skin is used for tissue repair and during manufacturing subjected to gentle processing compared to biologic materials derived from mammals. This is due to the absence of viral and prion disease transmission risk, preserving natural structure and composition of the fish skin graft. OBJECTIVES: The aim of this study was to assess properties of acellular fish skin relevant for severe battlefield injuries and to compare those properties with those of dehydrated human amnion/chorion membrane...
March 2017: Military Medicine
https://www.readbyqxmd.com/read/28289062/mapping-neurodegenerative-disease-onset-and-progression
#15
William W Seeley
Brain networks have been of long-standing interest to neurodegeneration researchers, including but not limited to investigators focusing on conventional prion diseases, which are known to propagate along neural pathways. Tools for human network mapping, however, remained inadequate, limiting our understanding of human brain network architecture and preventing clinical research applications. Until recently, neuropathological studies were the only viable approach to mapping disease onset and progression in humans but required large autopsy cohorts and laborious methods for whole-brain sectioning and staining...
March 13, 2017: Cold Spring Harbor Perspectives in Biology
https://www.readbyqxmd.com/read/28282581/inhibition-of-amyloid-peptide-fibril-formation-by-gold-sulfur-complexes
#16
Wenji Wang, Cong Zhao, Dengsen Zhu, Gehui Gong, Weihong Du
Amyloid-related diseases are characterized by protein conformational change and amyloid fibril deposition. Metal complexes are potential inhibitors of amyloidosis. Nitrogen-coordinated gold complexes have been used to disaggregate prion neuropeptide (PrP106-126) and human islet amyloid polypeptide (hIAPP). However, the roles of metal complexes in peptide fibril formation and related bioactivity require further exploration. In this work, we investigated the interactions of amyloid peptides PrP106-126 and hIAPP with two tetracoordinated gold-sulfur complexes, namely, dichloro diethyl dithiocarbamate gold complex and dichloro pyrrolidine dithiocarbamate gold complex...
February 21, 2017: Journal of Inorganic Biochemistry
https://www.readbyqxmd.com/read/28282052/a-synthetic-redox-biofilm-made-from-metalloprotein-prion-domain-chimera-nanowires
#17
Lucie Altamura, Christophe Horvath, Saravanan Rengaraj, Anaëlle Rongier, Kamal Elouarzaki, Chantal Gondran, Anthony L B Maçon, Charlotte Vendrely, Vincent Bouchiat, Marc Fontecave, Denis Mariolle, Patrice Rannou, Alan Le Goff, Nicolas Duraffourg, Michael Holzinger, Vincent Forge
Engineering bioelectronic components and set-ups that mimic natural systems is extremely challenging. Here we report the design of a protein-only redox film inspired by the architecture of bacterial electroactive biofilms. The nanowire scaffold is formed using a chimeric protein that results from the attachment of a prion domain to a rubredoxin (Rd) that acts as an electron carrier. The prion domain self-assembles into stable fibres and provides a suitable arrangement of redox metal centres in Rd to permit electron transport...
February 2017: Nature Chemistry
https://www.readbyqxmd.com/read/28281930/combined-trna-modification-defects-impair-protein-homeostasis-and-synthesis-of-the-yeast-prion-protein-rnq1
#18
Raffael Schaffrath, Roland Klassen
Modified nucleosides in tRNA anticodon loops such as 5-methoxy-carbonyl-methyl-2-thiouridine (mcm(5)s(2)U) and pseuduridine (Ψ) are thought to be required for an efficient decoding process. In Saccharomyces cerevisiae, the simultaneous presence of mcm(5)s(2)U and Ψ38 in tRNA(Gln)UUG was shown to mediate efficient synthesis of the Q/N rich [PIN(+)] prion forming protein Rnq1. (1) In the absence of these two tRNA modifications, higher than normal levels of hypomodified tRNA(Gln)UUG, but not its isoacceptor tRNA(Gln)CUG can restore Rnq1 synthesis...
January 2, 2017: Prion
https://www.readbyqxmd.com/read/28281929/in-vitro-amplification-of-h-type-atypical-bovine-spongiform-encephalopathy-by-protein-misfolding-cyclic-amplification
#19
Matthew J O'Connor, Keith Bishop, Robert G Workman, Ben C Maddison, Kevin C Gough
The in vitro amplification of prions by serial protein misfolding cyclic amplification has been shown to detect PrP(Sc) to levels at least as sensitive as rodent bioassay but in a fraction of the time. Bovine spongiform encephalopathy is a zoonotic prion disease in cattle and has been shown to occur in 3 distinct forms, classical BSE (C-BSE) and 2 atypical BSE forms (L-BSE and H-BSE). Atypical forms are usually detected in asymptomatic, older cattle and are suggested to be spontaneous forms of the disease. Here, we show the development of a serial protein misfolding cyclic amplification method for the detection of H-BSE...
January 2, 2017: Prion
https://www.readbyqxmd.com/read/28281928/amyloid-cores-in-prion-domains-key-regulators-for-prion-conformational-conversion
#20
María Rosario Fernández, Cristina Batlle, Marcos Gil-García, Salvador Ventura
Despite the significant efforts devoted to decipher the particular protein features that encode for a prion or prion-like behavior, they are still poorly understood. The well-characterized yeast prions constitute an ideal model system to address this question, because, in these proteins, the prion activity can be univocally assigned to a specific region of their sequence, known as the prion forming domain (PFD). These PFDs are intrinsically disordered, relatively long and, in many cases, of low complexity, being enriched in glutamine/asparagine residues...
January 2, 2017: Prion
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