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https://www.readbyqxmd.com/read/29155708/enrichment-of-detergent-insoluble-protein-aggregates-from-human-postmortem-brain
#1
Ian Diner, Tram Nguyen, Nicholas T Seyfried
In this study, we describe an abbreviated single-step fractionation protocol for the enrichment of detergent-insoluble protein aggregates from human postmortem brain. The ionic detergent N-lauryl-sarcosine (sarkosyl) effectively solubilizes natively folded proteins in brain tissue allowing the enrichment of detergent-insoluble protein aggregates from a wide range of neurodegenerative proteinopathies, such as Alzheimer's disease (AD), Parkinson's disease and amyotrophic lateral sclerosis, and prion diseases...
October 24, 2017: Journal of Visualized Experiments: JoVE
https://www.readbyqxmd.com/read/29151170/the-function-of-the-cellular-prion-protein-in-health-and-disease
#2
REVIEW
Joel C Watts, Matthew E C Bourkas, Hamza Arshad
The essential role of the cellular prion protein (PrP(C)) in prion disorders such as Creutzfeldt-Jakob disease is well documented. Moreover, evidence is accumulating that PrP(C) may act as a receptor for protein aggregates and transduce neurotoxic signals in more common neurodegenerative disorders, such as Alzheimer's disease. Although the pathological roles of PrP(C) have been thoroughly characterized, a general consensus on its physiological function within the brain has not yet been established. Knockout studies in various organisms, ranging from zebrafish to mice, have implicated PrP(C) in a diverse range of nervous system-related activities that include a key role in the maintenance of peripheral nerve myelination as well as a general ability to protect against neurotoxic stimuli...
November 18, 2017: Acta Neuropathologica
https://www.readbyqxmd.com/read/29142239/differential-overexpression-of-serpina3-in-human-prion-diseases
#3
S Vanni, F Moda, M Zattoni, E Bistaffa, E De Cecco, M Rossi, G Giaccone, F Tagliavini, S Haïk, J P Deslys, G Zanusso, J W Ironside, I Ferrer, G G Kovacs, G Legname
Prion diseases are fatal neurodegenerative disorders with sporadic, genetic or acquired etiologies. The molecular alterations leading to the onset and the spreading of these diseases are still unknown. In a previous work we identified a five-gene signature able to distinguish intracranially BSE-infected macaques from healthy ones, with SERPINA3 showing the most prominent dysregulation. We analyzed 128 suitable frontal cortex samples, from prion-affected patients (variant Creutzfeldt-Jakob disease (vCJD) n = 20, iatrogenic CJD (iCJD) n = 11, sporadic CJD (sCJD) n = 23, familial CJD (gCJD) n = 17, fatal familial insomnia (FFI) n = 9, Gerstmann-Sträussler-Scheinker syndrome (GSS)) n = 4), patients with Alzheimer disease (AD, n = 14) and age-matched controls (n = 30)...
November 15, 2017: Scientific Reports
https://www.readbyqxmd.com/read/29142140/imaging-and-csf-analyses-effectively-distinguish-cjd-from-its-mimics
#4
Peter Rudge, Harpreet Hyare, Alison Green, John Collinge, Simon Mead
OBJECTIVE: To review clinical and investigation findings in patients referred to a specialist prion clinic who were suspected to have sporadic Creutzfeldt-Jakob disease (sCJD) and yet were found to have an alternative final diagnosis. METHODS: Review the clinical findings and investigations in 214 patients enrolled into the UK National Prion Monitoring Cohort Study between October 2008 and November 2015 who had postmortem confirmed sCJD and compare these features with 50 patients referred over the same period who had an alternative final diagnosis (CJD mimics)...
November 15, 2017: Journal of Neurology, Neurosurgery, and Psychiatry
https://www.readbyqxmd.com/read/29142106/soluble-a%C3%AE-aggregates-can-inhibit-prion-propagation
#5
Claire J Sarell, Emma Quarterman, Daniel C-M Yip, Cassandra Terry, Andrew J Nicoll, Jonathan D F Wadsworth, Mark A Farrow, Dominic M Walsh, John Collinge
Mammalian prions cause lethal neurodegenerative diseases such as Creutzfeldt-Jakob disease (CJD) and consist of multi-chain assemblies of misfolded cellular prion protein (PrP(C)). Ligands that bind to PrP(C) can inhibit prion propagation and neurotoxicity. Extensive prior work established that certain soluble assemblies of the Alzheimer's disease (AD)-associated amyloid β-protein (Aβ) can tightly bind to PrP(C), and that this interaction may be relevant to their toxicity in AD. Here, we investigated whether such soluble Aβ assemblies might, conversely, have an inhibitory effect on prion propagation...
November 2017: Open Biology
https://www.readbyqxmd.com/read/29133694/manganese-in-manganism-parkinson-s-disease-huntington-s-disease-amyotrophic-lateral-sclerosis-and-batten-disease-a-narrative-review
#6
REVIEW
Owen Proudfoot
The collective evidence to date suggests that environmental exposure to excessive amounts of manganese (Mn) can cause a neurodegenerative condition known as manganism. It is now also relatively clear that Mn is involved in the pathogenesis of Alzheimer's disease and at least some prion diseases. The potential involvement of Mn in a panel of other neurodegenerative conditions including Parkinson's disease, Huntington's disease, amyotrophic lateral sclerosis, and Batten disease has been suggested and investigated, but the results to date are somewhat inconclusive...
November 2017: Neurology India
https://www.readbyqxmd.com/read/29133563/a-promising-anti-prion-trimethoxychalcone-binds-to-the-globular-domain-of-prp-c-and-changes-its-cellular-location
#7
N C Ferreira, L M Ascari, A G Hughson, G R Cavalheiro, C F Góes, P N Fernandes, J R Hollister, R A da Conceição, D S Silva, A M T Souza, M L C Barbosa, F A Lara, R A P Martins, B Caughey, Y Cordeiro
The search for anti-prion compounds has been encouraged by the fact that transmissible spongiform encephalopathies (TSEs) share molecular mechanisms with more prevalent neurodegenerative pathologies, such as Parkinson's and Alzheimer's diseases. Cellular prion protein conversion into protease-resistant forms (PrP(Res) or PrP(Sc)) is a critical step in the development of TSEs, thus being one of the main targets in the screening for anti-prion compounds. In this work, three trimethoxychalcones (J1, J8, J20) and one oxadiazole (Y17), previously identified in vitro as potential anti-prion compounds, were evaluated through different approaches in order to gain inferences about their mechanism of action...
November 13, 2017: Antimicrobial Agents and Chemotherapy
https://www.readbyqxmd.com/read/29130469/-heparan-sulphates-amyloidosis-and-neurodegeneration
#8
REVIEW
C Vera, J A Alvarez-Orozco, A Maiza, S Chantepie, R N Chehin, M O Ouidja, D Papy-Garcia
INTRODUCTION: A number of neurodegenerative disorders have been linked directly to the accumulation of amyloid fibres. These fibres are made up of proteins or peptides with altered structures and which join together in vivo in association with heparan sulphate-type polysaccharides. AIMS: To examine the most recent concepts in the biology of heparan sulphates and their role in the aggregation of the peptide Abeta, of tau protein, of alpha-synuclein and of prions...
November 16, 2017: Revista de Neurologia
https://www.readbyqxmd.com/read/29126445/ykl-40-in-the-brain-and-cerebrospinal-fluid-of-neurodegenerative-dementias
#9
Franc Llorens, Katrin Thüne, Waqas Tahir, Eirini Kanata, Daniela Diaz-Lucena, Konstantinos Xanthopoulos, Eleni Kovatsi, Catharina Pleschka, Paula Garcia-Esparcia, Matthias Schmitz, Duru Ozbay, Susana Correia, Ângela Correia, Ira Milosevic, Olivier Andréoletti, Natalia Fernández-Borges, Ina M Vorberg, Markus Glatzel, Theodoros Sklaviadis, Juan Maria Torres, Susanne Krasemann, Raquel Sánchez-Valle, Isidro Ferrer, Inga Zerr
BACKGROUND: YKL-40 (also known as Chitinase 3-like 1) is a glycoprotein produced by inflammatory, cancer and stem cells. Its physiological role is not completely understood but YKL-40 is elevated in the brain and cerebrospinal fluid (CSF) in several neurological and neurodegenerative diseases associated with inflammatory processes. Yet the precise characterization of YKL-40 in dementia cases is missing. METHODS: In the present study, we comparatively analysed YKL-40 levels in the brain and CSF samples from neurodegenerative dementias of different aetiologies characterized by the presence of cortical pathology and disease-specific neuroinflammatory signatures...
November 10, 2017: Molecular Neurodegeneration
https://www.readbyqxmd.com/read/29120943/native-prion-protein-homodimers-are-destabilized-by-oligomeric-amyloid-%C3%AE-1-42-species-as-shown-by-single-molecule-imaging
#10
Sachin S Tiwari, Yuki M Shirai, Yuri L Nemoto, Kumiko Kojima, Kenichi G N Suzuki
Prion proteins (PrPc) are receptors for amyloid β 1-42 (Aβ1-42) oligomers, but we do not know the impact of Aβ1-42 binding to PrPc on the interaction of membrane-bound PrPc with molecules that regulate downstream biological pathways. Stability of the PrPc dimeric complex and subsequent intermolecular interactions with membranous or cytoplasmic molecules are important for physiological functions of PrPc including neuroprotection. The principal aim of this study was to determine whether homodimer lifetime of PrPc is affected by the presence of Aβ1-42 oligomers...
November 8, 2017: Neuroreport
https://www.readbyqxmd.com/read/29114657/chemistry-of-mammalian-metallothioneins-and-their-interaction-with-amyloidogenic-peptides-and-proteins
#11
Elena Atrián-Blasco, Alice Santoro, Dean L Pountney, Gabriele Meloni, Christelle Hureau, Peter Faller
Cu and Zn ions are essential in most living beings. Their metabolism is critical for health and mis-metabolism can be lethal. In the last two decades, a large body of evidence has reported the role of copper, zinc and iron, and oxidative stress in several neurodegenerative diseases like Alzheimer's, Parkinson's, prion diseases, etc. To what extent this mis-metabolism is causative or a consequence of these diseases is still a matter of research. In this context metallothioneins (MTs) appear to play a central gate-keeper role in controlling aberrant metal-protein interactions...
November 8, 2017: Chemical Society Reviews
https://www.readbyqxmd.com/read/29104136/nucleic-acid-aptamers-for-neurodegenerative-diseases
#12
REVIEW
Alix Bouvier-Müller, Frédéric Ducongé
The increased incidence of neurodegenerative diseases represents a huge challenge for societies. These diseases are characterized by neuronal death and include several different pathologies, such as Alzheimer's disease, Parkinson's disease, multiple sclerosis, Huntington's disease and transmissible spongiform encephalopathies. Most of these pathologies are often associated with the aggregation of misfolded proteins, such as amyloid-ß, tau, α-synuclein, huntingtin and prion proteins. However, the precise mechanisms that lead to neuronal dysfunction and death in these diseases remain poorly understood...
November 2, 2017: Biochimie
https://www.readbyqxmd.com/read/29095718/the-vascular-facet-of-late-onset-alzheimer-s-disease-an-essential-factor-in-a-complex-multifactorial-disorder
#13
Yasser Iturria-Medina, Vladimir Hachinski, Alan C Evans
PURPOSE OF REVIEW: This article provides a brief overview of relevant cerebrovascular mechanisms implicated in late-onset Alzheimer's disease (LOAD) development, and highlights the main reasons for incorporating novel cerebrovascular biomarkers to the models defining a multifactorial LOAD pathogenesis. We also discuss how novel brain mapping techniques and multifactorial data-driven models are having a critical role on understanding LOAD and may be particularly useful for identifying effective therapeutic agents for this disorder...
December 2017: Current Opinion in Neurology
https://www.readbyqxmd.com/read/29076790/functional-amyloids-interrelationship-with-other-amyloids-and-therapeutic-assessment-to-treat-neurodegenerative-diseases
#14
Sutapa Som Chaudhury, Chitrangada Das Mukhopadhyay
Misfolded β-sheet structures of proteins leading to neurodegenerative diseases like Alzheimer's (AD) and Parkinson's diseases (PD) are in the spotlight since long. However, not much was known about the functional amyloids till the last decade. Researchers have become increasingly more concerned with the degree of involvement of these functional amyloids in human physiology. Interestingly, it has been found that the human body is exposed to a tremendous systemic amyloid burden, especially, during aging. Though many findings regarding these functional amyloids come up every day, some questions still remain unanswered like do these functional amyloids directly involve in the fibrillization of Amyloid beta (Aβ) 42 peptide or enhance the Aβ42 aggregation rate; whether functional bacterial amyloids (FuBA) co-localize with the senile plaques of AD or not...
October 27, 2017: International Journal of Neuroscience
https://www.readbyqxmd.com/read/29066368/investigating-the-efficacy-of-a-combination-a%C3%AE-targeted-treatment-in-a-mouse-model-of-alzheimer-s-disease
#15
Mingzhe Liu, Stefan Jevtic, Kelly Markham-Coultes, Nicholas P K Ellens, Meaghan A O'Reilly, Kullervo Hynynen, Isabelle Aubert, JoAnne McLaurin
Amyloid-beta peptide (Aβ) plays a critical role in the pathogenesis of Alzheimer's disease (AD). Here, we explored the use of a combination treatment to reduce amyloid load through microglial phagocytosis in a mouse model of AD. We hypothesized that using an initial treatment of magnetic resonance image guided focused ultrasound (MRIgFUS) to transiently increase the blood-brain barrier (BBB) permeability and enhance the delivery of an Aβ-antibody (BAM-10), followed by scyllo-inositol treatment would result in accelerated clearance...
October 21, 2017: Brain Research
https://www.readbyqxmd.com/read/29064456/reduced-abundance-and-subverted-functions-of-proteins-in-prion-like-diseases-gained-functions-fascinate-but-lost-functions-affect-aetiology
#16
REVIEW
W Ted Allison, Michèle G DuVal, Kim Nguyen-Phuoc, Patricia L A Leighton
Prions have served as pathfinders that reveal many aspects of proteostasis in neurons. The recent realization that several prominent neurodegenerative diseases spread via a prion-like mechanism illuminates new possibilities for diagnostics and therapeutics. Thus, key proteins in Alzheimer Disease and Amyotrophic lateral sclerosis (ALS), including amyloid-β precursor protein, Tau and superoxide dismutase 1 (SOD1), spread to adjacent cells in their misfolded aggregated forms and exhibit template-directed misfolding to induce further misfolding, disruptions to proteostasis and toxicity...
October 24, 2017: International Journal of Molecular Sciences
https://www.readbyqxmd.com/read/29041968/dynamic-changes-of-oligomeric-amyloid-%C3%AE-levels-in-plasma-induced-by-spiked-synthetic-a%C3%AE-42
#17
Seong Soo A An, Byoung-Sub Lee, Ji Sun Yu, Kuntaek Lim, Gwang Je Kim, Ryan Lee, Shinwon Kim, Sungmin Kang, Young Ho Park, Min Jeong Wang, Young Soon Yang, Young Chul Youn, SangYun Kim
BACKGROUND: A reliable blood-based assay is required to properly diagnose and monitor Alzheimer's disease (AD). Many attempts have been made to develop such a diagnostic tool by measuring amyloid-β oligomers (AβOs) in the blood, but none have been successful in terms of method reliability. We present a multimer detection system (MDS), initially developed for the detection of prion oligomers in the blood, to detect AβOs. METHODS: To characterize Aβ in the blood, plasma was spiked with synthetic amyloid-β (Aβ) and incubated over time...
October 17, 2017: Alzheimer's Research & Therapy
https://www.readbyqxmd.com/read/29038736/down-but-not-out-the-consequences-of-pretangle-tau-in-the-locus-coeruleus
#18
REVIEW
Termpanit Chalermpalanupap, David Weinshenker, Jacki M Rorabaugh
Degeneration of locus coeruleus (LC) is an underappreciated hallmark of Alzheimer's disease (AD). The LC is the main source of norepinephrine (NE) in the forebrain, and its degeneration is highly correlated with cognitive impairment and amyloid-beta (Aβ) and tangle pathology. Hyperphosphorylated tau in the LC is among the first detectable AD-like neuropathology in the brain, and while the LC/NE system impacts multiple aspects of AD (e.g., cognition, neuropathology, and neuroinflammation), the functional consequences of hyperphosphorylated tau accrual on LC neurons are not known...
2017: Neural Plasticity
https://www.readbyqxmd.com/read/29027580/screening-for-amyloid-proteins-in-the-yeast-proteome
#19
Tatyana A Ryzhova, Julia V Sopova, Sergey P Zadorsky, Vera A Siniukova, Aleksandra V Sergeeva, Svetlana A Galkina, Anton A Nizhnikov, Aleksandr A Shenfeld, Kirill V Volkov, Alexey P Galkin
The search for novel pathological and functional amyloids represents one of the most important tasks of contemporary biomedicine. Formation of pathological amyloid fibrils in the aging brain causes incurable neurodegenerative disorders such as Alzheimer's, Parkinson's Huntington's diseases. At the same time, a set of amyloids regulates vital processes in archaea, prokaryotes and eukaryotes. Our knowledge of the prevalence and biological significance of amyloids is limited due to the lack of universal methods for their identification...
October 11, 2017: Current Genetics
https://www.readbyqxmd.com/read/29027011/let-s-make-microglia-great-again-in-neurodegenerative-disorders
#20
REVIEW
Marie-Victoire Guillot-Sestier, Terrence Town
All of the common neurodegenerative disorders-Alzheimer's disease, Parkinson's disease, amyotrophic lateral sclerosis and prion diseases-are characterized by accumulation of misfolded proteins that trigger activation of microglia; brain-resident mononuclear phagocytes. This chronic form of neuroinflammation is earmarked by increased release of myriad cytokines and chemokines in patient brains and biofluids. Microglial phagocytosis is compromised early in the disease process, obfuscating clearance of abnormal proteins...
October 12, 2017: Journal of Neural Transmission
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