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95 results for “Prion”
dataset related to article: "PMCA-Based Detection of Prions in the Olfactory Mucosa of Patients With Sporadic Creutzfeldt-Jakob Disease"
<ol> <li>ESTIMATING PRION CONCENTRATION IN THE OM OF SCJD MM1, MV2 AND VV2 PATIENTS BY MEANS OF QUANTITATIVE PMCA (QPMCA)</li> <li>EVALUATION OF THE BIOCHEMICAL PROPERTIES OF PMCA GENERATED PRODUCTS</li> <li>PMCA ANALYSIS OF SCJD BRAIN HOMOGENATES</li> <li>PMCA ANALYSIS OF SCJD OLFACTORY MUCOSA</li> <li>RT-QuIC ANALYSIS OF OM AND BH OF SCJD PATIENTS AND CONTROLS</li> <li>WESTERN BLOT ANALYSIS OF BRAIN HOMOGENATES</li> <li>STATISTICAL ANALYSIS</li> </ol>
Diagnosis of prion diseases by RT-QuIC results in improved surveillance
<p><span><u>Objective:</u> We present the National Prion Disease Pathology Surveillance Center's (NPDPSC) experience using cerebrospinal fluid (CSF) real time quaking induced conversion (RT-QuIC) as a diagnostic test, examine factors associated with false negative RT-QuIC results, and investigate RT-QuIC's impact on prion disease surveillance. </span></p> <p> </p> <p><span><u>Methods:</u> Between May 2015-April 2018, the NPDPSC received 10,498 CSF specimens that were included in the study. Sensitivity and specificity analyses were performed using 567 autopsy verified cases. Prion disease type, demographic characteristics, specimen color, and time variables were examined for association with RT-QuIC results. The effect of including positive RT-QuIC cases in prion disease surveillance was examined.</span></p> <p> </p> <p><span><u>Results:</u> The diagnostic sensitivity and specificity of RT-QuIC across all prion diseases was 90.3% and 98.5%, respectively. Diagnostic sensitivity was lower for fatal familial insomnia, Gerstmann-Sträussler-Scheinker disease, sporadic fatal insomnia, variably protease sensitive prionopathy, and the VV1 and MM2 subtypes of sCJD. Individuals with prion disease and negative RT-QuIC results were younger, had elevated tau levels, and non-elevated 14-3-3 levels compared to RT-QuIC positive cases. Sensitivity was high throughout the disease course. Some cases that initially tested RT-QuIC negative had a subsequent specimen test positive. Including positive RT-QuIC cases in surveillance statistics increased laboratory-based case ascertainment of prion disease by 90% over autopsy alone. </span></p> <p> </p> <p><span><u>Conclusions:</u> RT-QuIC has high sensitivity and specificity for diagnosing prion diseases. Sensitivity limitations are associated with prion disease type, age, and related CSF diagnostic results. RT-QuIC greatly improves laboratory-based prion disease ascertainment for surveillance purposes. </span></p> <p> </p> <p><span><u>Classification of Evidence:</u> This study provides Class III evidence that 2<sup>nd</sup> generation real time quaking-induced conversion (RT-QuIC) identifies prion disease with sensitivity of 90.3% and specificity of 98.5%, among patients being screened for these diseases due to concerning symptoms. </span></p>
Rotational electron diffraction patterns of TIA-1 prion-like domain, A381T mutant
<p>Rotational electron diffraction patterns of TIA-1 prion-like domain, A381T mutant. Collected with a JEOL CRYO ARM 300 electron microscope operated at an accelerating voltage of 300kV on a DirectElectron DE64 detector at RIKEN, SPring-8 Center. The PDB ID associated with this data is 7VI5.The PDB ID associated with this data is 7VI4.</p>
Rotational electron diffraction patterns of TIA-1 prion-like domain, wild type
<p>Rotational electron diffraction patterns of TIA-1 prion-like domain, wild type. Collected with a JEOL CRYO ARM 300 electron microscope operated at an accelerating voltage of 300kV on a DirectElectron DE64 detector at RIKEN, SPring-8 Center. The PDB ID associated with this data is 7VI5.</p>
ELF3 prion-like domain Martini clustering simulations
<p>This dataset contains Martini coarse-grain simulations of ELF3-PrD, with each simulation containing 100 PrD monomers. These trajectories were created as part of a publication exploring the temperature-responsive condensation of the ELF3-PrD in the scientific pulication titled, "Molecular dynamics simulations illuminate the role of sequence context in the ELF3-PrD-based temperature sensing mechanism in plants." Included are trajectories for ELF3-PrD variants including wildtype (7 glutamine-long polyQ tract), 0Q (variable poly-glutamine tract removed), 19Q (polyQ tract extenden to 19 glutamine residues) and the F527A mutant. Each variant includes trajectories at 290K, 300K, 320K and 405K. There are three replicates for each condition, except for wildtype 300K and 19Q 340K, of which two replicates are provided.</p>
Contextual variations in calls of two non-oscine birds: the blue petrel and the Antarctic prion
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Diagnosis of prion diseases by RT-QuIC results in improved surveillance
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Autoantibodies against the prion protein in individuals with PRNP mutations
<p><span><span><span><span><span><span><span><span><span><span><span><i>Objective. </i>To determine whether naturally occurring autoantibodies against the prion protein are present in individuals with genetic prion disease mutations and controls, and if so, whether they are protective against prion disease. </span></span></span></span></span></span></span></span></span></span></span></p> <p><span><span><span><span><span><span><span><span><span><span><span><i>Methods. </i>In this case-control study, we collected 124 blood samples from individuals with a variety of pathogenic<i>PRNP</i>mutations and 78 control individuals with a positive family history of genetic prion disease but lacking disease-associated<i>PRNP</i>mutations. Antibody reactivity was measured using an indirect ELISA for the detection of human IgG<sub>1-4 </sub>antibodies against wild-type human prion protein. Multivariate linear regression models were constructed to analyze differences in autoantibody reactivity between a) <i>PRNP</i>mutation carriers versus controls and b) asymptomatic versus symptomatic <i>PRNP</i>mutation carriers. Robustness of results was examined in matched cohorts.</span></span></span></span></span></span></span></span></span></span></span></p> <p><span><span><span><span><span><span><span><span><span><span><span><i>Results. </i>We found that antibody reactivity was present in a subset of both <i>PRNP</i>mutation carriers and controls. Autoantibody levels were not influenced by <i>PRNP </i>mutation status nor clinical manifestation of prion disease. <i>Post hoc</i>analyses showed anti-PrP<sup>C</sup>autoantibody titers to be independent of personal history of autoimmune disease and other immunological disorders, as well as <i>PRNP</i>codon 129 polymorphism. </span></span></span></span></span></span></span></span></span></span></span></p> <p><span><span><span><span><span><span><span><span><span><span><span><i>Conclusions.</i>Pathogenic <i>PRNP</i>variants do not notably stimulate antibody-mediated anti-PrP<sup>C</sup>immunity. Anti-PrP<sup>C</sup>IgG autoantibodies are not associated with the onset of prion disease. The presence of anti-PrP<sup>C</sup>autoantibodies in the general population without any disease-specific association suggests that relatively high titers of naturally occurring antibodies are well tolerated. Clinicaltrials.gov identifier NCT02837705. </span></span></span></span></span></span></span></span></span></span></span></p>
Year-round at-sea movements of fairy prions from south-eastern Australia
<p>Effective conservation assessments require detailed information of species' ecological niches during the whole annual cycle. For seabirds, this implies investigating the at-sea distribution and foraging behaviour during both the breeding and non-breeding periods. However, until recently, collecting information about the smallest species has been precluded by the excessive size of the required devices. This lack of knowledge is exacerbated in the case of polytypic genera with species sharing very similar appearance and behaviour, such as the super-abundant prions (<em>Pachyptila</em> spp.). The present study investigates the year-round at-sea distribution and foraging ecology of the fairy prion (<em>Pachyptila turtur</em>) in south-eastern Australia. Using miniaturized GPS during the breeding season and geolocators (GLS) during the non-breeding period, the results highlight the importance of the continental shelf-edge waters for fairy prions throughout the year. In addition, contrary to previous assumptions, the GLS data revealed an unsuspected post-breeding migration to the waters south of Australia, during which individuals likely undergo a rapid moult of flight feathers. Understanding the at-sea distribution and ecology of prions during the whole annual cycle will be fundamental to their conservation as it can reveal species- or population-specific threats that have been overlooked because of their status of abundant species.</p>
ELF3 prion-like domain REST2 simulation trajectories
<p>This dataset contains ELF3 prion-like domain (PrD) trajectories obtained with replica exchange with solute tempering (REST2). These trajectories were created as part of a publication exploring the temperature-responsive condensation of the ELF3-PrD in the scientific pulication titled, "Molecular dynamics simulations illuminate the role of sequence context in the ELF3-PrD-based temperature sensing mechanism in plants." Included are trajectories for ELF3-PrD variants including wildtype, 0Q (variable poly-glutamine tract removed), and an F527A mutant, each at a range of temperatures between 290K and 405K.</p>
Expanding molecular grammar of polar residues and arginine in FUS prion-like domain
<p>Codes to reporoduce simualtion results presented in the paper "Expanding molecular grammar of polar residues and arginine in FUS prion-like domain". </p> <p>Simulation_Codes.zip - Contains python codes for calcualtion of contacts, intrachain distances and angle-distance probability distributions along with files to check the output of the codes. </p> <p>ThT_Assay_Fitting_Scripts.zip - Contains scripts to fit ThT curve.</p>
Data for Measuring prion propagation in single bacteria elucidates mechanism of loss.
<pre>Single-cell microfluidics data for: Jager, K., Orozco-Hildago, M.T., Springstein, B.L., Joly-Smith, E., Papazotos, F., McDonough, E., Fleming, E., McCallum, G., Yuan, A.H., Hilfinger, A., Hochschild, A. and Potvin-Trottier, L. (2023) Measuring prion propagation in single bacteria elucidates mechanism of loss.</pre>
Therapeutic Antibodies Against Prion Diseases From PRNP Mutation Carriers
ClinicalTrials.gov study NCT02837705. IPD Sharing: YES. Countries: 7. Publications: 1.
Biomarker Profiling in Individuals at Risk for Prion Disease
ClinicalTrials.gov study NCT05124392. IPD Sharing: NO. Countries: 1. Publications: 1.
Autoantibodies against the prion protein in individuals with PRNP mutations
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Year-round at-sea movements of fairy prions from south-eastern Australia
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Data from: Pioneer cells established by the [SWI+] prion can promote dispersal and out-crossing in yeast
To thrive in an ever-changing environment, microbes must widely distribute their progeny to colonize new territory. Simultaneously, they must evolve and adapt to the stresses of unpredictable surroundings. In both of these regards, diversity is key—if an entire population moved together or responded to the environment in the same way, it could easily go extinct. Here, we show that the epigenetic prion switch [SWI+] establishes a specialized subpopulation with a "pioneer" phenotypic program in Saccharomyces cerevisiae. Cells in the pioneer state readily disperse in water, enabling them to migrate and colonize new territory. Pioneers are also more likely to find and mate with genetically diverse partners, as inhibited mating-type switching causes mother cells to shun their own daughters. In the nonprion [swi−] state, cells instead have a "settler" phenotype, forming protective flocs and tending to remain in their current position. Settler cells are better able to withstand harsh conditions like drought and alkaline pH. We propose that these laboratory observations reveal a strategy employed in the wild to rapidly diversify and grant distinct, useful roles to cellular subpopulations that benefit the population as a whole.
Data from: A continuous model of physiological prion aggregation suggests a role for Orb2 in gating long-term synaptic information
The regulation of mRNA translation at the level of the synapse is believed to be fundamental in memory and learning at cellular level. The family of Cytoplasmic Polyadenylation Element Binding (CPEB) proteins emerged as an important RNA binding protein family during development and in adult neurons. Drosophila Orb2 (homolog of mouse CPEB3 protein and of the neural isoform of Aplysia CPEB) has been found to be involved in the translation of plasticity-dependent mRNAs and has been associated to Long Term Memory. Orb2 protein presents two main isoforms, Orb2A and Orb2B, which form an activity induced amyloid-like functional aggregate, that is thought to be the translation-inducing state of the RNA binding protein. Here we present a first two-states continuous differential model for Orb2A-Orb2B aggregation. This model provides new working hypotheses for studying the role of prion-like CPEB proteins in long term synaptic plasticity. Moreover, this model can be used as a first step to integrate translation- and protein aggregation-dependent phenomena in synaptic facilitation rules.
The Role of the Coagulation Pathway at the Synapse in Prion Diseases
ClinicalTrials.gov study NCT02480725. IPD Sharing: Not stated. Countries: 0. Publications: 29.
Data from: Prion protein inhibits fast axonal transport through a mechanism involving casein kinase 2
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Allen Brain Atlas
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DANDI Archive for NWB datasets
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International Brain Laboratory public data
The International Brain Laboratory public data releases expose standardized mouse decision-making experiments, including Neuropixels recordings, widefield calcium imaging, behavior, and session metadata accessed through the ONE API.
OpenNeuro
OpenNeuro is a free, open platform for sharing neuroimaging datasets, with public search, dataset pages, and download paths for web, S3, DataLad, and the OpenNeuro CLI.