Search results for "MAGNETIC RESONANCE"

showing 10 items of 4721 documents

Diagnostic Targeted Resequencing in 349 Patients with Drug-Resistant Pediatric Epilepsies Identifies Causative Mutations in 30 Different Genes

2017

Targeted resequencing gene panels are used in the diagnostic setting to identify gene defects in epilepsy. We performed targeted resequencing using a 30-genes panel and a 95-genes panel in 349 patients with drug-resistant epilepsies beginning in the first years of life. We identified 71 pathogenic variants, 42 of which novel, in 30 genes, corresponding to 20.3% of the probands. In 66% of mutation positive patients seizures onset occurred before age 6 months. The 95-genes panel allowed a genetic diagnosis in 22 (6.3%) patients that would have otherwise been missed using the 30-gene panel. About 50% of mutations were identified in genes coding for sodium and potassium channel components. SCN2…

0301 basic medicineProbandMaleCDKL5Drug Resistancemedicine.disease_causeBioinformaticsEpilepsyAnticonvulsantSTXBP1Age of OnsetChildGenetics (clinical)AlleleMutationepilepsy; next-generation sequencing; gene panel; mutationPhenotypeMagnetic Resonance ImagingSettore MED/39 - Neuropsichiatria Infantile3. Good healthPhenotypeChild PreschoolAnticonvulsantsFemaleSequence AnalysisHumanAdolescentGenotypeGenetic Association StudieBiologyMECP203 medical and health sciencesGeneticgene panelGeneticsmedicineHumansGenetic Predisposition to DiseasePreschoolGeneAllelesGenetic Association StudiesGene Expression ProfilingInfant NewbornComputational BiologyInfantMolecular Sequence AnnotationDNASequence Analysis DNANewbornmedicine.disease030104 developmental biologyepilepsynext-generation sequencingmutation
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Lunasin is a redox sensitive intrinsically disordered peptide with two transiently populated α-helical regions.

2016

Lunasin is a 43 amino acid peptide with anti-cancer, antioxidant, anti-inflammatory and cholesterol-lowering properties. Although the mechanism of action of lunasin has been characterized to some extent, its exact three-dimensional structure as well as the function of the N-terminal sequence remains unknown. We established a novel method for the production of recombinant lunasin that allows efficient isotope labeling for NMR studies. Initial studies showed that lunasin can exist in a reduced or oxidized state with an intramolecular disulfide bond depending on solution conditions. The structure of both forms of the peptide at pH 3.5 and 6.5 was characterized by CD spectroscopy and multidimen…

0301 basic medicineProtein Conformation alpha-HelicalCircular dichroismPhysiologyBeta sheetPeptideIntrinsically disordered proteinsBiochemistryLunasinAntioxidantsHistones03 medical and health sciencesCellular and Molecular Neuroscience0302 clinical medicineEndocrinologyNeoplasmsAnticarcinogenic AgentsHumansAmino Acid SequenceDisulfidesProtein secondary structureNuclear Magnetic Resonance BiomolecularPlant Proteinschemistry.chemical_classificationChemistryAcetylationNuclear magnetic resonance spectroscopyIntrinsically Disordered Proteins030104 developmental biologyBiochemistry030220 oncology & carcinogenesisBiophysicsSoybean ProteinsPeptidesOxidation-ReductionFunction (biology)Peptides
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The Crystal Structure of Gurmarin, a Sweet Taste–Suppressing Protein: Identification of the Amino Acid Residues Essential for Inhibition

2018

International audience; Gurmarin is a highly specific sweet-taste suppressing protein in rodents that is isolated from the Indian plant Gymnemasylvestre. Gurmarin consists of 35 amino acid residues containing three intramolecular disulfide bridges that form a cystine knot. Here, we report the crystal structure of gurmarin at a 1.45 Å resolution and compare it with previously reported NMR solution structures. The atomic structure at this resolution allowed us to identify a very flexible region consisting of hydrophobic residues. Some of these amino acid residues had been identified as a putative binding site for the rat sweet taste receptor in a previous study. By combining alanine-scanning …

0301 basic medicineProtein ConformationPhysiologyCrystal structureCrystallography X-Ray03 medical and health sciencesBehavioral NeuroscienceGPCRsweet tastetaste receptorPhysiology (medical)goût sucréAnimalsHumansG protein-coupled receptorAmino AcidsBinding siteReceptorNuclear Magnetic Resonance BiomolecularPlant ProteinsGurmarininhibiteur030102 biochemistry & molecular biologybiologyChemistryMutagenesisCystine knotGymnema sylvestreSweet tastebiology.organism_classificationRecombinant ProteinsSensory SystemsRats3. Good healthinhibitorHEK293 Cells030104 developmental biologyBiochemistryGymnema sylvestreknottin[SDV.AEN]Life Sciences [q-bio]/Food and NutritionHydrophobic and Hydrophilic InteractionsChemical Senses
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Zero-field nuclear magnetic resonance of chemically exchanging systems.

2019

Zero- to ultralow-field (ZULF) nuclear magnetic resonance (NMR) is an emerging tool for precision chemical analysis. In this work, we study dynamic processes and investigate the influence of chemical exchange on ZULF NMR J-spectra. We develop a computational approach that allows quantitative calculation of J-spectra in the presence of chemical exchange and apply it to study aqueous solutions of [15N]ammonium (15N\documentclass[12pt]{minimal} \usepackage{amsmath} \usepackage{wasysym} \usepackage{amsfonts} \usepackage{amssymb} \usepackage{amsbsy} \usepackage{mathrsfs} \usepackage{upgreek} \setlength{\oddsidemargin}{-69pt} \begin{document}$${\mathrm{H}}_4^ +$$\end{document}H4+) as a model syst…

0301 basic medicineReaction kinetics and dynamicsSciencePhysics::Medical PhysicsGeneral Physics and AstronomyModel system02 engineering and technologyGeneral Biochemistry Genetics and Molecular BiologyArticle03 medical and health sciencesNuclear magnetic resonanceZero fieldHyperpolarization (physics)lcsh:ScienceDissolutionQuantitative Biology::Biomolecules3403 Macromolecular and Materials ChemistryMultidisciplinaryAqueous solution34 Chemical SciencesChemical exchangeQ500Diagnostic markersGeneral ChemistryNuclear magnetic resonance spectroscopy021001 nanoscience & nanotechnologyequipment and supplies030104 developmental biologylcsh:Qddc:5000210 nano-technologyhuman activitiesSolution-state NMR51 Physical Sciences
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Secondary Progression in Multiple Sclerosis: Neuronal Exhaustion or Distinct Pathology?

2016

Prevention of progression in neurological diseases, particularly in multiple sclerosis (MS) but also in neurodegenerative diseases, remains a significant challenge. MS patients switch from a relapsing-remitting to a progressive disease course, but it is not understood why and how this conversion occurs and why some patients never experience disease progression. Do aging and accumulation of neuronal damage induce progression, or do cognitive symptoms and accelerated grey matter (GM) atrophy point to distinct processes affecting networks? This review weighs accepted dogma against real data on the secondary progressive phase of the disease, highlighting current challenges in this important fie…

0301 basic medicineSenescencePathologymedicine.medical_specialtyMultiple SclerosisDiseaseGrey matter03 medical and health sciences0302 clinical medicineAtrophymedicineHumansSecondary progressiveNeuronsbusiness.industryGeneral NeuroscienceMultiple sclerosisNeurodegenerationBrainmedicine.diseaseMagnetic Resonance Imaging030104 developmental biologymedicine.anatomical_structureDisease ProgressionbusinessNeuroscience030217 neurology & neurosurgeryProgressive diseaseTrends in Neurosciences
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The O-antigen of Plesiomonas shigelloides serotype O36 containing pseudaminic acid

2016

The structure of the repeating unit of O-antigen of Plesiomonas shigelloides serotype O36 has been investigated by 1H and 13C NMR spectroscopy, matrix-assisted laser-desorption/ionization time-of-flight mass spectrometry and chemical methods. The new structure of trisaccharide has been established: →4)-β-Pse5Ac7(R3Hb)-(2 → 4)-β-D-Galp-(1 → 3)-β-D-GlcpNAc-(1→ These trisaccharide O-antigen units substitute the core undecasaccharide at C-4 of the β-D-GlcpNAc residue. The core oligosaccharide and lipid A are identical with these of the serotype O17 (PCM 2231) (Maciejewska, A., Lukasiewicz, J., Kaszowska, M., Jachymek, W., Man-Kupisinska, A.; Lugowski, C. Mar. Drugs.2013, 11 (2), 440–454; Lukasi…

0301 basic medicineSerotypeMagnetic Resonance SpectroscopyStereochemistryMass spectrometrySerogroupBiochemistryAnalytical ChemistryLipid A03 medical and health sciencesResidue (chemistry)AntigenMALDI-TOF MSTrisaccharidechemistry.chemical_classification030102 biochemistry & molecular biologybiologyChemistryOrganic ChemistryO AntigensGeneral MedicineO-antigenbiology.organism_classificationPlesiomonas shigelloidesNMRMatrix-assisted laser desorption/ionization030104 developmental biologyBiochemistryCarbohydrate SequencePlesiomonas shigelloidesPlesiomonasCarbohydrate Research
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No diagnostic utility of antibody patterns against Klebsiella pneumoniae capsular serotypes in patients with axial spondyloarthritis vs. patients wit…

2017

OBJECTIVES: To investigate whether antibody response patterns against Klebsiella pneumoniae capsular serotypes can discriminate patients with axial spondyloarthritis (axSpA) from patients with non-specific low back pain (LBP).METHOD: Immunoglobulin (Ig)G and IgA antibodies against K. pneumoniae capsular serotypes K2, K26, K36, and K50 were measured, and antibody seropositivity compared between groups and analysed for patient correlation in five different groups: (a) 96 patients fulfilling the Assessment of SpondyloArthritis International Society (ASAS) classification criteria for axSpA; (b) 38 patients with either a positive magnetic resonance imaging (MRI) scan as defined by ASAS or a posi…

0301 basic medicineSerotypeMaleCross-sectional studyKlebsiella pneumoniaeDenmarkGastroenterology0302 clinical medicineImmunology and AllergyHLA-B27 Antigenbiologymedicine.diagnostic_testGeneral MedicineLow back painAntibodies BacterialMagnetic Resonance ImagingKlebsiella pneumoniaeC-Reactive ProteinFemaleAntibodymedicine.symptomAdultmedicine.medical_specialtyAdolescentImmunologyHuman leukocyte antigenSerogroup03 medical and health sciencesYoung AdultRheumatologyInternal medicineJournal ArticlemedicineHumansSpondylitis AnkylosingLow back painSacroiliitisBacterial Capsules030203 arthritis & rheumatologyAnkylosing spondylitisbusiness.industryta1182Magnetic resonance imagingaxial spondyloarthritismedicine.diseasebiology.organism_classificationantibody response patternsImmunoglobulin A030104 developmental biologyCase-Control StudiesImmunoglobulin GImmunologybiology.proteinSpondylarthropathiesbusinessLow Back Pain
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Targeting Bacterial Sortase A with Covalent Inhibitors: 27 New Starting Points for Structure-Based Hit-to-Lead Optimization.

2019

Because of its essential role as a bacterial virulence factor, enzyme sortase A (SrtA) has become an attractive target for the development of new antivirulence drugs against Gram-positive infections. Here we describe 27 compounds identified as covalent inhibitors of

0301 basic medicineStaphylococcus aureusMagnetic Resonance SpectroscopyAntivirulenceVirulence Factors030106 microbiologySmall Molecule Libraries03 medical and health sciencesMiceBacterial ProteinsCatalytic DomainDrug DiscoveryAnimalschemistry.chemical_classificationBinding SitesChemistryHit to leadFibroblastsAminoacyltransferasesAnti-Bacterial AgentsMolecular Docking SimulationCysteine Endopeptidases030104 developmental biologyInfectious DiseasesEnzymeBiochemistryCovalent bondSortase ABacterial virulenceNIH 3T3 CellsStructure basedACS infectious diseases
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Monitoring few molecular binding events in scalable confined aqueous compartments by raster image correlation spectroscopy (CADRICS)

2016

The assembly of scalable liquid compartments for binding assays in array formats constitutes a topic of fundamental importance in life sciences. This challenge can be addressed by mimicking the structure of cellular compartments with biological native conditions. Here, inkjet printing is employed to develop up to hundreds of picoliter aqueous droplet arrays stabilized by oil-confinement with mild surfactants (Tween-20). The aqueous environments constitute specialized compartments in which biomolecules may exploit their function and a wide range of molecular interactions can be quantitatively investigated. Raster Image Correlation Spectroscopy (RICS) is employed to monitor in each compartmen…

0301 basic medicineStreptavidinBiomedical EngineeringMolecular bindingBiotinBioengineeringNanotechnology02 engineering and technologydroplets microarrays inkjet printing Raster Image Correlation Spectroscopy water-in-oil emulsion StreptvidinBiochemistry03 medical and health scienceschemistry.chemical_compoundCompartment (pharmacokinetics)Cellular compartmentchemistry.chemical_classificationAqueous solutionSpectrum AnalysisBiomoleculeWaterGeneral Chemistrycomputer.file_formatMicroarray Analysis021001 nanoscience & nanotechnology030104 developmental biologychemistryPrintingInkStreptavidinRaster graphics0210 nano-technologycomputerTwo-dimensional nuclear magnetic resonance spectroscopyLab on a Chip
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Characterizing microstructural tissue properties in multiple sclerosis with diffusion MRI at 7 T and 3 T: The impact of the experimental design

2019

The recent introduction of advanced magnetic resonance (MR) imaging techniques to characterize focal and global degeneration in multiple sclerosis (MS), like the Composite Hindered and Restricted Model of Diffusion, or CHARMED, diffusional kurtosis imaging (DKI) and Neurite Orientation Dispersion and Density Imaging (NODDI) made available new tools to image axonal pathology non-invasively in vivo. These methods already showed greater sensitivity and specificity compared to conventional diffusion tensor-based metrics (e.g., fractional anisotropy), overcoming some of its limitations. While previous studies uncovered global and focal axonal degeneration in MS patients compared to healthy contr…

0301 basic medicineTime FactorsUltra-high field MRIAxonal pathologyCohort Studies0302 clinical medicineNuclear magnetic resonancemethods [Diffusion Magnetic Resonance Imaging]MicrostructureNODDImedicine.diagnostic_testGeneral NeuroscienceWATER DIFFUSIONmedicine.anatomical_structureResearch DesignKurtosisMulti-shell diffusion MRIAxonal degenerationWHITE-MATTERTENSORAdultMaterials sciencetherapy [Multiple Sclerosis]Sensitivity and SpecificityWhite matterMultiple sclerosis03 medical and health sciencesFractional anisotropyImage Interpretation Computer-Assistedmedicinediagnostic imaging [Nerve Degeneration]Journal ArticleHumansddc:610OPTIMIZATIONMultiple sclerosisinstrumentation [Diffusion Magnetic Resonance Imaging]diagnostic imaging [Multiple Sclerosis]Magnetic resonance imagingQUANTIFICATIONmedicine.diseaseMODELPATHOLOGYDiffusion Magnetic Resonance Imaging030104 developmental biologyRESOLUTIONDENSITYNerve Degeneration030217 neurology & neurosurgeryDiffusion MRI
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