Search results for "Bell"

showing 10 items of 1130 documents

Nuclear inclusions of pathogenic ataxin-1 induce oxidative stress and perturb the protein synthesis machinery

2020

Spinocerebellar ataxia type-1 (SCA1) is caused by an abnormally expanded polyglutamine (polyQ) tract in ataxin-1. These expansions are responsible for protein misfolding and self-assembly into intranuclear inclusion bodies (IIBs) that are somehow linked to neuronal death. However, owing to lack of a suitable cellular model, the downstream consequences of IIB formation are yet to be resolved. Here, we describe a nuclear protein aggregation model of pathogenic human ataxin-1 and characterize IIB effects. Using an inducible Sleeping Beauty transposon system, we overexpressed the ATXN1(Q82) gene in human mesenchymal stem cells that are resistant to the early cytotoxic effects caused by the expr…

0301 basic medicineSCA1 Spinocerebellar ataxia type-1Intranuclear Inclusion BodiesClinical BiochemistryMSC mesenchymal stem cellProtein aggregationBiochemistry0302 clinical medicineMutant proteinProtein biosynthesisDE differentially expressed genesNuclear proteinlcsh:QH301-705.5FTIR Fourier-transform infrared spectroscopyAtaxin-1lcsh:R5-920biologyChemistryNuclear ProteinspolyQ polyglutamineRibosomeCell biologySB Sleeping BeautyRibosome ; Polyglutamine ; Ataxin-1 ; Oxidative stress ; Transposon ; Sleeping beauty transposon ; Protein networkSpinocerebellar ataxiaProtein foldingCellular modelFunction and Dysfunction of the Nervous Systemlcsh:Medicine (General)Research PaperiPSC induced pluripotent stem cellAtaxin 1Nerve Tissue ProteinsPPI protein-protein interaction03 medical and health sciencesROS reactive oxygen speciesProtein networkSleeping beauty transposonGSEA Gene Set Enrichment AnalysismedicineHumansNPC neural progenitor cellOrganic Chemistrymedicine.diseaseAFM atomic force microscopyOxidative Stress030104 developmental biologylcsh:Biology (General)IIBs intranuclear inclusion bodiesMS mass spectrometryCardiovascular and Metabolic Diseasesbiology.proteinPolyglutamine030217 neurology & neurosurgery
researchProduct

A Vastly Increased Chemical Variety of RNA Modifications Containing a Thioacetal Structure

2018

International audience; Recently discovered new chemical entities in RNA modifications have involved surprising functional groups that enlarge the chemical space of RNA. Using LC-MS, we found over 100 signals of RNA constituents that contained a ribose moiety in tRNAs from E. coli. Feeding experiments with variegated stable isotope labeled compounds identified 37 compounds that are new structures of RNA modifications. One structure was elucidated by deuterium exchange and high-resolution mass spectrometry. The structure of msms2 i6 A (2-methylthiomethylenethio-N6-isopentenyl-adenosine) was confirmed by methione-D3 feeding experiments and by synthesis of the nucleobase. The msms2 i6 A contai…

0301 basic medicineStereochemistryThioacetal010402 general chemistry01 natural sciencesCatalysisNucleobaseisotope labelling03 medical and health scienceschemistry.chemical_compoundAcetalsRNA modificationsTandem Mass Spectrometry[SDV.BBM.GTP]Life Sciences [q-bio]/Biochemistry Molecular Biology/Genomics [q-bio.GN]RiboseEscherichia coliMoietySulfhydryl Compoundschemistry.chemical_classificationChemistrythioacetalsRNA[SDV.BBM.BM]Life Sciences [q-bio]/Biochemistry Molecular Biology/Molecular biologyGeneral Chemistryradical-SAM enzymesChemical space0104 chemical sciencesLC-MSRNA Bacterial030104 developmental biologyEnzymeNucleic Acid ConformationHydrogen–deuterium exchangeChromatography Liquid
researchProduct

Mitigating anticipated effects of systematic errors supports sister-group relationship between Xenacoelomorpha and Ambulacraria.

2019

International audience; Xenoturbella and the acoelomorph worms (Xenacoelomorpha) are simple marine animals with controversial affinities. They have been placed as the sister group of all other bilaterian animals (Nephrozoa hypothesis), implying their simplicity is an ancient characteristic ]; alternatively, they have been linked to the complex Ambulacraria (echinoderms and hemichordates) in a clade called the Xenambulacraria , suggesting their simplicity evolved by reduction from a complex ancestor. The difficulty resolving this problem implies the phylogenetic signal supporting the correct solution is weak and affected by inadequate modeling, creating a misleading non-phylogenetic signal. …

0301 basic medicineXenoturbellaAmbulacrariamedia_common.quotation_subjectAcoelomorpha ; Ambulacraria ; Metazoa ; Nephrozoa ; Phylogenomics ; Phylogeny ; Systematic Error ; XenoturbellaNephrozoaContext (language use)phylogeny[SDV.BID.SPT]Life Sciences [q-bio]/Biodiversity/Systematics Phylogenetics and taxonomyGeneral Biochemistry Genetics and Molecular Biologysystematic error03 medical and health sciences0302 clinical medicineXenoturbellaAnimalsSimplicityAmbulacrariaChordatamedia_commonLong branch attractionbiologyMetazoa[SDV.BID.EVO]Life Sciences [q-bio]/Biodiversity/Populations and Evolution [q-bio.PE]Acoelomorphaphylogenomicsbiology.organism_classificationBiological EvolutionInvertebratesXenacoelomorpha[SDV.BA.ZI]Life Sciences [q-bio]/Animal biology/Invertebrate Zoology030104 developmental biologySister groupEvolutionary biologyOutgroupGeneral Agricultural and Biological Sciences030217 neurology & neurosurgeryEchinodermata
researchProduct

Fluorogenic Tetrazine-Siliconrhodamine Probe for the Labeling of Noncanonical Amino Acid Tagged Proteins

2018

Tetrazine-bearing fluorescent labels enable site-specific tagging of proteins that are genetically manipulated with dienophile modified noncanonical amino acids. The inverse electron demand Diels-Alder reaction between the tetrazine and the dienophile fulfills the criteria of bioorthogonality allowing fluorescent labeling schemes of live cells. Here, we describe the detailed synthetic and labeling protocols of a near infrared emitting siliconrhodamine-tetrazine probe suitable for super-resolution imaging of residue-specifically engineered proteins in mammalian cells.

0301 basic medicinechemistry.chemical_classificationSuper-resolution microscopy010402 general chemistry01 natural sciencesFluorescence0104 chemical sciencesAmino acid03 medical and health sciencesTetrazinechemistry.chemical_compoundFluorescent labelling030104 developmental biologychemistryBiophysics
researchProduct

MECP2 impairs neuronal structure by regulating KIBRA

2016

Using a Drosophila model of MECP2 gain-of-function, we identified memory associated KIBRA as a target of MECP2 in regulating dendritic growth. We found that expression of human MECP2 increased kibra expression in Drosophila, and targeted RNAi knockdown of kibra in identified neurons fully rescued dendritic defects as induced by MECP2 gain-of-function. Validation in mouse confirmed that Kibra is similarly regulated by Mecp2 in a mammalian system. We found that Mecp2 gain-of-function in cultured mouse cortical neurons caused dendritic impairments and increased Kibra levels. Accordingly, Mecp2 loss-of-function in vivo led to decreased Kibra levels in hippocampus, cortex, and cerebellum. Togeth…

0301 basic medicinecongenital hereditary and neonatal diseases and abnormalitiesCerebellumMethyl-CpG-Binding Protein 2Dendritic morphologyHippocampusDisease modelsHippocampusArticlelcsh:RC321-571MECP2Mice03 medical and health sciencesMemoryRNA interferencemental disordersmedicineAnimalsHumanslcsh:Neurosciences. Biological psychiatry. NeuropsychiatryCerebral CortexNeuronsGene knockdownMECP2 duplication syndromebiologybiology.organism_classificationMECP2nervous system diseasesCortex (botany)Disease Models AnimalDrosophila melanogaster030104 developmental biologymedicine.anatomical_structureNeurologyCerebral cortexDrosophilaDrosophila melanogasterNeuroscienceNeurobiology of Disease
researchProduct

Barbus meridionalis Risso, 1827 populations status in the Vişeu River basin (Maramureş Mountains Nature Park)

2017

Abstract The ecological state of lotic ecosystems occupied naturally by Barbus meridionalis, in the Vişeu Basin within the Maramureş Mountains Natural Park, vary among good to reduced. The inventoried human activities which negatively influence the ecologic state of the Barbus meridionalis species habitats and populations are the organic and mining pollution, and poaching. The habitats with low and inadequate conditions created a reduced status of the Barbus meridionalis populations; the status of Barbus meridionalis populations is not so much affected in the cases of habitats of average to good condition. Barbus meridionalis is considered a relatively common fish species in the researched …

0301 basic medicinegeography.geographical_feature_categoryhuman impactevaluationEcologyEcologyEcology (disciplines)Drainage basinFisheryBarbus meridionalis03 medical and health sciencesmediterranean barbell030104 developmental biologyGeographyNature parkhabitatsromaniaQH540-549.5Transylvanian Review of Systematical and Ecological Research
researchProduct

Dynamics of a Protein Interaction Network Associated to the Aggregation of polyQ-Expanded Ataxin-1

2020

Background: Several experimental models of polyglutamine (polyQ) diseases have been previously developed that are useful for studying disease progression in the primarily affected central nervous system. However, there is a missing link between cellular and animal models that would indicate the molecular defects occurring in neurons and are responsible for the disease phenotype in vivo. Methods: Here, we used a computational approach to identify dysregulated pathways shared by an in vitro and an in vivo model of ATXN1(Q82) protein aggregation, the mutant protein that causes the neurodegenerative polyQ disease spinocerebellar ataxia type-1 (SCA1). Results: A set of common dysregulated pathwa…

0301 basic medicinelcsh:QH426-470Ataxin 1Mice TransgenicNerve Tissue ProteinsProtein aggregationBlood–brain barrierblood-brain-barrierArticledrugspolyQ03 medical and health sciences0302 clinical medicineataxin-1Interaction networkIn vivoMutant proteinCerebellumGeneticsmedicineAnimalsGene Regulatory NetworksProtein Interaction MapsGenetics (clinical)NeuronsbiologypathwayGene Expression Profilingmedicine.diseaselcsh:Genetics030104 developmental biologymedicine.anatomical_structureGene Expression Regulationnetworkbiology.proteinSpinocerebellar ataxiaPeptidesNeuroscience030217 neurology & neurosurgeryFunction (biology)Genes
researchProduct

Vaccination and Vaccine Effectiveness: A Commentary of Special Issue Editors

2020

The Special Issue “Vaccination and Vaccine Effectiveness”, published in the journal Vaccines, has the main aim to increase international literature data on vaccine effectiveness and safety and on vaccination strategies in order to reduce vaccine hesitancy and improve vaccination coverage rates. The main topics included in the call for papers were vaccines administered to infants, adolescents, adults, elderly people, at-risk populations (due to comorbidities and personal risk factors) and healthcare workers and strategies adopted to promote vaccination adherence among these categories. This Special Issue started from the assumption that, despite vaccination being universally recognized as on…

0301 basic medicinemedicine.medical_specialtyImmunologyPopulationlcsh:MedicineRubella and varicella vaccinationVaccines AdministeredMeasles03 medical and health sciences0302 clinical medicineQuality of life (healthcare)Internationally adopted childrenDrug DiscoveryHealth caremedicinemeaslesElderly peoplePharmacology (medical)Research article030212 general & internal medicinehepatitis B vaccinationeducationPharmacologyHuman papillomavirus vaccinationeducation.field_of_studyvaccine effectivenesshealthcare workersbusiness.industrylcsh:Rvaccinationmedicine.diseaseinfluenza vaccinationVaccinationEditorial030104 developmental biologyInfectious DiseasesFamily medicineHealthcare workervaccine hesitancymumpsHerpes zoster vaccinationbusinessVaccines
researchProduct

Identification and Characterization of Extracellular Vesicles and Its DNA Cargo Secreted During Murine Embryo Development

2020

Extracellular vesicles (EVs) are known to transport DNA, but their implications in embryonic implantation are unknown. The aim of this study was to investigate EVs production and secretion by preimplantation embryos and assess their DNA cargo. Murine oocytes and embryos were obtained from six- to eight-week-old females, cultured until E4.5 and analyzed using transmission electron microscopy to examine EVs production. EVs were isolated from E4.5-day conditioned media and quantified by nanoparticle tracking analysis, characterized by immunogold, and their DNA cargo sequenced. Multivesicular bodies were observed in murine oocytes and preimplantation embryos together with the secretion of EVs t…

0301 basic medicinemurine blastocystslcsh:QH426-470Embryonic DevelopmentexosomesArticleEmbryo Culture Techniques03 medical and health scienceschemistry.chemical_compoundMice0302 clinical medicineGeneticsmedicineAnimalsSecretionBlastocystParticle Sizeapoptotic bodiesGenetics (clinical)030219 obstetrics & reproductive medicineChemistryADP-Ribosylation FactorsTetraspanin 30BlastocoelHigh-Throughput Nucleotide SequencingEmbryoImmunogold labellingSequence Analysis DNADNAEmbryonic stem cellMicrovesiclesCell biologylcsh:Genetics030104 developmental biologymedicine.anatomical_structureBlastocystADP-Ribosylation Factor 6Culture Media Conditionedembryonic structuresOocytespreimplantation embryosFemaleextracellular vesiclesmicrovesiclesDNAGenes
researchProduct

Cell Proliferation High-Content Screening on Adherent Cell Cultures

2019

Pulse-chase experiments using 5-bromo-2'-deoxyuridine (BrdU), or the more recent EdU (5-etynil-2'-deoxyuridine), enable the identification of cells going through S phase. This chapter describes a high-content proliferation assay pipeline for adherent cell cultures. High-throughput imaging is followed by high-content data analysis using a non-supervised ImageJ macroinstruction that segments the individual nuclei, determines the nucleoside analogue absence/presence, and measures the signal of up to two additional nuclear markers. Based upon the specific combination with proliferation-specific protein immunostaining, the percentage of cells undergoing different phases of the cell cycle (G0, G1…

0303 health sciences030219 obstetrics & reproductive medicineCell growthChemistryCell cycleMolecular biologyNeural stem cellDeoxyuridine03 medical and health scienceschemistry.chemical_compound0302 clinical medicineLabellingHigh-content screeningImmunostainingEx vivo030304 developmental biology
researchProduct