Search results for " STORAGE"

showing 10 items of 984 documents

Recommendations on the diagnosis and management of Niemann-Pick disease type C

2009

Niemann-Pick disease type C (NP-C) is a lysosomal storage disease in which impaired intracellular lipid trafficking leads to excess storage of cholesterol and glycosphingolipids in the brain and other tissues. it is characterized clinically by a variety of progressive, disabling neurological symptoms including clumsiness, limb and gait ataxia, dysarthria, dysphagia and cognitive deterioration (dementia). Until recently, there has been no disease-modifying therapy available for NP-C, with treatment limited to supportive measures. In most countries, NP-C is managed through specialist centers, with non-specialist support provided locally. However, effective patient Support is hampered by the a…

Adultmedicine.medical_specialtyNeurology1303 BiochemistryAdolescentEndocrinology Diabetes and Metabolism610 Medicine & healthDiseaseBiochemistry03 medical and health sciencesDysarthriaYoung Adult0302 clinical medicineEndocrinology1311 GeneticsGeneticsLysosomal storage diseasemedicine1312 Molecular BiologyDementiaHumansMass ScreeningIntensive care medicineChildMolecular BiologyMass screening030304 developmental biology0303 health sciencesNiemann–Pick disease type Cbusiness.industryInfant NewbornInfantNiemann-Pick Disease Type CMiddle Agedmedicine.disease3. Good health1310 Endocrinology2712 Endocrinology Diabetes and Metabolism10036 Medical ClinicChild PreschoolPhysical therapyGait Ataxiamedicine.symptombusiness030217 neurology & neurosurgery
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Development and testing of new screening method for keratan sulfate in mucopolysaccharidosis IVA.

2004

Mucopolysaccharidosis IVA (MPS IVA), a progressive lysosomal storage disease, causes skeletal dysplasia through excessive storage of keratan sulfate (KS). We developed an ELISA-sandwich assay that used a MAb specific to KS. Forty-five blood and 59 urine specimens from MPS IVA patients (ages 1–65 y) were analyzed to determine whether KS concentration is a suitable marker for early diagnosis and longitudinal assessment of disease severity. Blood specimens were obtained from patients categorized as phenotypically severe (n = 36) and milder (n = 9). Urine specimens were also analyzed from patients categorized as severe (n = 56) and milder (n = 12), respectively. Blood KS levels (101–1525 ng/mL)…

Adultmedicine.medical_specialtyPathologyAdolescentMucopolysaccharidosisStatistics as TopicEnzyme-Linked Immunosorbent AssayUrineGastroenterologyMucopolysaccharidosis Type IVAExcretionDiagnosis Differentialchemistry.chemical_compoundInternal medicinemedicineLysosomal storage diseaseHumansGenetic TestingChildAgedGlycosaminoglycansCreatininebusiness.industryInfantMucopolysaccharidosis IVReproducibility of ResultsMiddle Agedmedicine.diseasechemistryDysplasiaKeratan SulfateChild PreschoolPediatrics Perinatology and Child HealthMucopolysaccharidosis IVsense organsbusinessBiomarkersPediatric research
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Organogenesis and encapsulation of in vitro-derived propagules of Carrizo citrange [Citrus sinensis (L.) Osb. × Poncirius trifoliata (L.) Raf]

2011

Due to widespread polyembryony, Citrus rootstocks are usually propagated by open-pollinated seed germination, although micropropagation offers many advantages. Encapsulation technology has recently attracted the interest of researchers in the field of plant propagation because it combines the advantages of zygotic or gamic seeds with those of micropropagation. In this study, we examined the encapsulation of Carrizo citrange uninodal microcuttings (3–4 mm long) and evaluated the influence of the calcium alginate coating, a short time storage at cold temperature, and different sowing substrates on the viability and regrowth of the explants. A secondary aim was to develop an efficient protocol…

Adventitious shoot Cytokinins Explant type Micropropagation Germplasm storage Synthetic seedmicropropagationPlant propagationregenerative processesregenerative processes; tissue culture; micropropagation; microcutting; alginate matrixOrganogenesisHorticultureBiologyBasal shootMicropropagationShootBotanyalginate matrixEpicotyltissue culturemicrocuttingRootstockExplant culturePlant Cell, Tissue and Organ Culture (PCTOC)
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A simplified framework to optimize MRI contrast preparation

2018

PURPOSE This article proposes a rigorous optimal control framework for the design of preparation schemes that optimize MRI contrast based on relaxation time differences. METHODS Compared to previous optimal contrast preparation schemes, a drastic reduction of the optimization parameter number is performed. The preparation scheme is defined as a combination of several block pulses whose flip angles, phase terms and inter-pulse delays are optimized to control the magnetization evolution. RESULTS The proposed approach reduces the computation time of B 0 -robust preparation schemes to around a minute (whereas several hours were required with previous schemes), with negligible performance loss. …

AgingMultiple Sclerosis[SDV.IB.IMA]Life Sciences [q-bio]/Bioengineering/ImagingComputer scienceComputationContrast MediaContext (language use)HippocampusCorpus Callosum030218 nuclear medicine & medical imagingReduction (complexity)Magnetics03 medical and health sciences0302 clinical medicineThalamusAlzheimer Disease[INFO.INFO-IM]Computer Science [cs]/Medical ImagingAnimalsHumansComputer SimulationRadiology Nuclear Medicine and imagingPoint (geometry)Gray MatterComputingMilieux_MISCELLANEOUSBlock (data storage)Flexibility (engineering)Phantoms ImagingBrainContrast (statistics)Models TheoreticalOptimal controlMagnetic Resonance ImagingRatsFemaleAlgorithmAlgorithms030217 neurology & neurosurgeryMagnetic Resonance in Medicine
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The Relevance of Mineral Mobilization and -Dissolution on the Reservoir Quality of Sandstones in CO2 Storage Sites

2014

Abstract Rotliegend siliciclastic formations are important reservoirs in central Europe. These sediments consist of pristine red coloured and bleached, high porous and permeable sandstones. To evaluate the relevance of distinct fluids and their fluid-rock alteration reactions on such bleaching processes laboratory static batch experiments under reservoir conditions were conducted. Thereby mineralogical, petrophysical and (hydro-, geo-) chemical rock features were investigated by different analytical methods before and after the experiments. The achieved results suggest that during such fluid-rock interactions a complex interplay between mineral detachment and mineral dissolution processes w…

AltmarkPetrophysicsGeochemistryMineralogySandstoneCo2 storageRotliegendCCSReservoir qualityMineral dissolutionPermeability (earth sciences)Energy(all)BleachingMineral detachmentCO2SiliciclasticAutoclave experimentsPorosityDissolutionGeologyEnergy Procedia
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Detection of mitochondrial electron chain carrier redox status by transhepatic light intensity during rat liver reperfusion.

2003

The aim of the study was to investigate mitochondrial electron transfer during rat liver reperfusion after cold storage and hypothermic machine perfusion. Livers from male Brown Norway rats were preserved (UW) for 10h either by cold storage (CS) or by hypothermic oxygenated perfusion extracorporal (HOPE). Transhepatic photometric analysis allowed determination of the redox status of mitochondrial cytochromes during preservation, rewarming and reperfusion. Mitochondrial electron chain carriers were inhibited at different sites with rotenone and cyanide in some experiments. reversed transcriptional polymerase chain reaction (RT-PCR) was performed after reperfusion concerning transcription of …

AnionsMaleTime FactorsCytochromeLightCold storageCaspase 3ElectronsDNA FragmentationMitochondrionGeneral Biochemistry Genetics and Molecular Biologychemistry.chemical_compoundSuperoxidesAnimalsCaspase-9CryopreservationCyanidesbiologySuperoxideCaspase 3Reverse Transcriptase Polymerase Chain ReactionTumor Necrosis Factor-alphaJNK Mitogen-Activated Protein KinasesTemperatureNADH DehydrogenaseGeneral MedicineRotenoneDNAOrgan PreservationLipid MetabolismCaspase 9MitochondriaRatsCold TemperatureOxygenLight intensitychemistryBiochemistryElectron Transport Chain Complex ProteinsLiverCaspasesReperfusionbiology.proteinCytochromesLipid PeroxidationMitogen-Activated Protein KinasesGeneral Agricultural and Biological SciencesReactive Oxygen SpeciesOxidation-ReductionCryobiology
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Plant Sterols and Antioxidant Parameters in Enriched Beverages: Storage Stability

2012

Plant sterols (PS) stability, antioxidant parameters, and color were studied during 6 months of storage at 4, 24, and 37 °C in three PS-enriched functional beverages. Beverages were skimmed milk with fruit juice and PS (MFJPS), fruit juice and PS (FJPS), and skimmed milk with PS (MPS). No loss in total PS content occurred during storage observing the same values at any given storage time point. Total carotenoids decreased 36% with storage at two months and then remained stable. Total polyphenols showed fluctuations throughout the storage, remaining stable at 6 months and reaching initial values. The antioxidant capacity (TEAC method) increased 18% at 6 months, and there was an increase in c…

Antioxidantfood.ingredientmedicine.medical_treatmentAntioxidantsBeveragessymbols.namesakefoodSkimmed milkmedicineFood scienceCarotenoidchemistry.chemical_classificationPhytosterolsGeneral ChemistryMaillard reactionAntioxidant capacityFood StoragechemistrySpainPolyphenolFruitFood FortifiedsymbolsFruit juiceDairy ProductsGeneral Agricultural and Biological SciencesPlant sterolsJournal of Agricultural and Food Chemistry
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Strength of pre-Roman amphorae: Comparison of the different types

2015

Abstract Given that amphorae were used for storage or for long and/or short distance transport, they must have been able to retain their content and, at the same time, be able to resist important load during transport. This paper concerns aspects of the techno-functionality of pre-Roman Iberian amphorae. The basic performance requirement concerned here is the mechanical strength (transverse rupture strength: TRS). Strength is related to the adequacy for storage or short and/or long distance transport that, up to now, is only assessed by typological identification or archaeological evidence. Experimental implementation on actual archaeological material has confirmed that strength increased w…

ArcheologyInelastic deformationArchaeologyTexture (geology)Archaeological evidenceShort distanceFlexural strengthMechanical loading Inelastic deformation Strength Iberian amphora Trade StorageMechanical strengthPerformance requirementGeotechnical engineeringSiliciclasticSettore GEO/09 -Georis. Miner.e Appl.Mineral.-Petrogr. per l'Ambi.ed i B.Cult.GeologyJournal of Archaeological Science: Reports
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The Waterfront Theorem: an integrated and creative planning approach

2016

Waterfronts are sensitive interfaces between land and sea, creative interfaces between urban and environmental components, social interfaces between nodes and networks. They not only coincide with the areas of infrastructure that are gathering on the coast, not only with the shoreline or the environmental components of the coastal territories. They are hired by the project as complex interaction of crossing flows, stratified identity of urban communities: fundamental element of the environmental system, synthesis of landscape, space and community, a place of solidification of the identity of the social, cultural and economic community (Carta, 2009). The management of urban interface of wate…

Architectural engineeringMathematical optimizationProcess (engineering)Water storage tankGreen roofContext (language use)Urban regenerationwaterfront urban regeneration urban planning urban design waterfront regenerationPlanning approachGreen infrastructureSettore ICAR/21 - UrbanisticaMathematics
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Refitting Solutions Promoted by $$\ell _{12}$$ Sparse Analysis Regularizations with Block Penalties

2019

International audience; In inverse problems, the use of an l(12) analysis regularizer induces a bias in the estimated solution. We propose a general refitting framework for removing this artifact while keeping information of interest contained in the biased solution. This is done through the use of refitting block penalties that only act on the co-support of the estimation. Based on an analysis of related works in the literature, we propose a new penalty that is well suited for refitting purposes. We also present an efficient algorithmic method to obtain the refitted solution along with the original (biased) solution for any convex refitting block penalty. Experiments illustrate the good be…

Artifact (error)Total variationComputer scienceRegular polygon02 engineering and technologyInverse problem01 natural sciences[INFO.INFO-AI]Computer Science [cs]/Artificial Intelligence [cs.AI]010104 statistics & probabilityRefitting0202 electrical engineering electronic engineering information engineeringBias correction020201 artificial intelligence & image processingBias correction0101 mathematics[MATH]Mathematics [math]AlgorithmBlock (data storage)Scale Space and Variational Methods in Computer Vision - 7th International Conference, SSVM 2019, Hofgeismar, Germany, June 30 – July 4, 2019, Proceedings
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