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Genes & Diseases 2024年第3期11卷 66-68页

作者： Aswathi n Jincy M.Jose sreelata B.nair Prabha s.n Pappachan s Department of Medical Genetics Lifeline Super Specialty HospitalPathanamthittaKerala 691523India Lifeline super specialty Hospital PathanamthittaKerala 691523India

Alagille syndrome(ALGs;OMIM#118450)is a highlyvariable,multi-systemic,autosomal dominant disorder,caused by mutations in the Jagged Canonical notch Ligand 1(JAG1)gene(20p12.2)or in the neurogenic locus notch homolog protein 2(nOTCH2)gene(1p13).^(1)It primarily affects the liver,heart,eyes,face,kidney,skin,vertebrae,and skeleton.^(1,2)The JAG1 gene has 26 exons and about 90%of the ALGs cases result from pathogenic variants in the JAG1 and around 7%of cases have deletions in chromosome 20 that include this gene.

关键词： JAG1 cases notch

Towards defining soil quality of Mediterranean calcareous agricultural soils:Reference values and potential core indicator set

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International soil and Water Conservation Research 2024年第1期12卷 145-155页

作者： Germán A.Durán Daniel sacristán Edelweis Farrús Jaume Vadell Institut de Recerca i FormacióAgrària i Pesquera de les Illes Balears C/Eusebi Estada14507009PalmaSpain Plant Biology Department University of Valencia46100BurjassotValenciaSpain soil and Environmental Quality Department Centro de Investigaciones sobre Desertificación-CIDE(CSIC-Universitat de València-Generalitat Valenciana)Carretera Moncada-Náquera km 4.546113MoncadaValenciaSpain Universitat de les Illes Balears Cra.De Valldemossakm 7507122PalmaSpain

To avoid soil degradation,adapt to climate change and comply with the sustainable Development goals 2030(Un General Assembly),establishing the quality/fertility of the agricultural soils of the Mediterranean region and evaluate how these evolve with time is *** enables adequate management practices to be *** Mediterranean calcareous region has received little attention in this sense,in spite its soil ***,24 different representative calcareous agricultural soils of the Mediterranean region,including the main management strategies of agricultural soils(rainfed and irrigated),were sampled from the island of Mallorca;and their physical,chemical and biological properties *** values obtained for most of the soil characteristics allowed to establish an initial approach to the reference values for the type of calcareous agricultural soils considered,and to clearly distinguish the effect of rainfed or irrigation management practices on soils properties and *** enzyme activities were not stimulated by the irrigation conditions assayed or they were only in dry conditions,suggesting optimum enzyme activities could be obtained when alternating dry and humid soil *** organic carbon,calcium carbonate and active lime revealed of significant importance in the collection of ***,the results obtained clearly indicate the heterogeneity of the region and its implications on the different soil ***,this study could serve as a starting point to adequately establish the quality(fertility)for Mediterranean calcareous agricultural soils and their reference values by conducting further research in this region and including more type of soils.

关键词： soil fertility Mediterranean agricultural soils Calcareous soils soil enzymes soil management

A Probabilistic Trust Model and Control Algorithm to Protect 6G networks against Malicious Data Injection Attacks in Edge Computing Environments

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Computer Modeling in Engineering & sciences 2024年第10期141卷 631-654页

作者： Borja Bordel sánchez Ramón Alcarria Tomás Robles Department of Computer systems Universidad Politécnica de MadridMadrid28031Spain Department of Geospatial Engineering Universidad Politécnica de MadridMadrid28031Spain

Future 6G communications are envisioned to enable a large catalogue of pioneering *** will range from networked Cyber-Physical systems to edge computing devices,establishing real-time feedback control loops critical for managing Industry 5.0 deployments,digital agriculture systems,and essential *** provision of extensive machine-type communications through 6G will render many of these innovative systems autonomous and *** full automation will enhance industrial efficiency significantly,it concurrently introduces new cyber risks and *** particular,unattended systems are highly susceptible to trust issues:malicious nodes and false information can be easily introduced into control ***,Denialof-service attacks can be executed by inundating the network with valueless *** anomaly detection schemes require the entire transformation of the control software to integrate new steps and can only mitigate anomalies that conform to predefined mathematical *** based on an exhaustive data collection to detect anomalies are precise but extremely *** models,with their limited understanding of mobile networks,can achieve precision rates no higher than 75%.Therefore,more general and transversal protection mechanisms are needed to detect malicious behaviors *** paper introduces a probabilistic trust model and control algorithm designed to address this *** model determines the probability of any node to be *** channels are pruned for those nodes whose probability is below a given *** trust control algorithmcomprises three primary phases,which feed themodel with three different probabilities,which are weighted and ***,anomalous nodes are identified using Gaussian mixture models and clustering ***,traffic patterns are studied using digital Bessel functions and the functional scalar ***,the in

关键词： 6G networks noise injection attacks Gaussian mixture model Bessel function traffic filter Volterra filter

superplasticity of fine-grained Mg-10Li alloy prepared by severe plastic deformation and understanding its deformation mechanisms

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Journal of Magnesium and Alloys 2024年第1期12卷 316-331页

作者： H.T.Jeong s.W.lee W.J.Kim Department of Materials science and Engineering Hongik UniversityMapo-guSangsu-dong 72-1Seoul 121-791Republic of Korea

The superplastic behavior and associated deformation mechanisms of a fine-grained Mg-10.1 Li-0.8Al-0.6Zn alloy(LAZ1011)with a grain size of 3.2μm,primarily composed of the BCCβphase and a small amount of the HCPαphase,were examined in a temperature range of 473 K to 623 *** microstructural refinement of this alloy was achieved by employing high-ratio differential speed *** best superplasticity was achieved at 523 K and at strain rates of 10^(-4)-5×10^(-4)s^(-1),where tensile elongations of 550±600%were *** the heating and holding stage of the tensile samples prior to tensile loading,a significant increase in grain size was observed at temperatures above 573 ***,it was important to consider this effect when analyzing and understanding the superplastic deformation behavior and *** the investigated strain rate range,the superplastic flow at low strain rates was governed by lattice diffusion-controlled grain boundary sliding,while at high strain rates,lattice diffusion-controlled dislocation climb creep was the rate-controlling deformation *** was concluded that solute drag creep is unlikely to *** the late stages of deformation at 523 K,it was observed that grain boundary sliding led to the agglomeration of theαphase,resulting in significant strain *** mechanism maps were constructed forβ-Mg-Li alloys in the form of 2D and 3D formats as a function of strain rate,stress,temperature,and grain size,using the constitutive equations for various deformation mechanisms derived based on the data of the current tests.

关键词： Magnesium-lithium alloy superplasticity severe plastic deformation Grain size Grain growth

Role of climatic variability in shaping intraspecific variation of thermal tolerance in Mediterranean water beetles

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Insect science 2024年第1期31卷 285-298页

作者： susana Pallarés David Garoffolo Belén Rodríguez David sánchez-Fernández Department of Zoology University of SevilleSevilleSpain Faculty of Biology Department of Ecology and HydrologyUniversity of MurciaCampus EspinardoMurciaSpain

The climatic variability hypothesis(CVH)predicts that organisms in more thermally variable environments have wider thermal breadths and higher thermal plasticity than those from more stable ***,due to evolutionary trade-offs,taxa with greater absolute thermal limits may have little plasticity of such limits(trade-off hypothesis).The CVH assumes that climatic variability is the ultimate driver of thermal tolerance variation across latitudinal and altitudinal gradients,but average temperature also varies along such *** explored intraspecific variation of thermal tolerance in three typical Mediterranean saline water beetles(families Hydrophilidae and Dytiscidae).For each species,we compared two populations where the species coexist,with similar annual mean temperature but contrasting thermal variability(continental *** population).We estimated thermal limits of adults from each population,previously acclimated at 17,20,or 25℃.We found species-specific patterns but overall,our results agree with the CVH regarding thermal ranges,which were wider in the continental(more variable)*** the two hydrophilid species,this came at the cost of losing plasticity of the upper thermal limit in this population,supporting the trade-off hypothesis,but not in the dytiscid *** results support the role of local adaptation to thermal variability and trade-offs between basal tolerance and physiological plasticity in shaping thermal tolerance in aquatic ectotherms,but also suggest that intraspecific variation of thermal tolerance does not fit a general pattern among aquatic *** such intraspecific variation could lead to inaccurate predictions of the vulnerability of aquatic insects to global warming.

关键词： acclimation capacity aquatic insects climate change heat coma temperature supercooling point thermal plasticity

Ion heat transport in electron cyclotron resonance heated L-mode plasma on the T-10 tokamak

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Plasma science and Technology 2024年第4期26卷 52-60页

作者： V.A.KRUPIn M.R.nURGALIEV A.R.nEMETs I.A.ZEMTsOV s.D.sUnTsOV T.B.MYALTOn D.s.sERGEEV n.A.sOLOVEV D.V.sARYCHEV D.V.RYJAKOV s.n.TUGARInOV n.n.nAUMEnKO national Research Centre“Kurchatov Institute” Moscow 123182Russia Bauman Moscow state Technical University Moscow 105005Russia Institution“Project Center ITER” Moscow 123098Russia JsC“sOLAR” Minsk 220034Belarus

Anomalous ion heat transport is analyzed in the T-10 tokamak plasma heated with electron cyclotron resonance heating(ECRH) in second-harmonic extra-ordinary mode. Predictive modeling with empirical scaling for Ohmical heat conductivity shows that in ECRH plasmas the calculated ion temperature could be overestimated, so an increase of anomalous ion heat transport is required. To study this effect two scans are presented: over the EC resonance position and over the ECRH power. The EC resonance position varies from the high-field side to the low-field side by variation of the toroidal magnetic field. The scan over the heating power is presented with on-axis and mixed ECRH regimes. Discharges with high anomalous ion heat transport are obtained in all considered regimes. In these discharges the power balance ion heat conductivity exceeds the neoclassical level by up to 10 times. The high ion heat transport regimes are distinguished by three parameters: the ratio Te/Ti, the normalized electron density gradient R/■, and the ion–ion collisionality νii~*. The combination of high Te/Ti, high νii~*, and R/■=6-10 results in values of normalized anomalous ion heat fluxes up to 10 times higher than in the low transport scenario.

关键词： tokamak L-mode electron cyclotron resonance heating ion heat transport

nanomotion of bacteria to determine metabolic profile

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nanotechnology and Precision Engineering 2024年第1期7卷 1-9页

作者： s.n.Pleskova E.V.Lazarenko n.A.Bezrukov R.n.Kriukov A.V.Boryakov M.E.Dokukin s.I.surodin Research Laboratory of scanning Probe Microscopy Lobachevsky State University of Nizhny NovgorodNizhny Novgorod 603950Russia Department of nanotechnology and Biotechnology Nizhny Novgorod State Technical University n.a.R.E.AlekseevNizhny Novgorod 603950Russia sarov Physics and Technology Institute National Research Nuclear University MEPhISarov 607186Russia

In addition to their visible motion such as swimming(e.g.,with the help ofﬂagella),bacteria can also exhibit nanomotion that is detectable only with highly sensitive instruments,and this study shows that it is possible to detect bacterial nanomotion using an AFM detection *** results show that the nanomotion characteristics depend on the bacterial strain,and that nanomotion can be used to sense the metabolic activity of bacteria because the oscillations are sensitive to the food preferences of the bacteria and the type of surrounding medium.

关键词： nanomotion Bacteria Cantilever Oscillation Atomic force microscopy Metabolism

Optimum Profiles of Endwall Contouring for Enhanced net Heat Flux Reduction and Aerodynamic Performance

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Journal of Harbin Institute of Technology(new series) 2024年第2期31卷 80-92页

作者： Arjun K s Tide P s Biju n Division of Mechanical Engineering School of EngineeringCochin University of Science&TechnologyKalamassery 682022India

successfully utilized non-axisymmetric endwalls to enhance turbine efficiencies(aerodynamic and turbine inlet temperatures)by controlling the characteristics of the secondary flow in a blade *** is accomplished by steady-state numerical hydrodynamics and deep knowledge of the field of *** of the interaction between mainstream and purge flow contributing supplementary losses in the stage,non-axisymmetric endwalls are highly susceptible to the inception of purge flow exit compared to the flat and any advantage rapidly *** conclusions reveal that the supreme endwall pattern could yield a lowering of the gross pressure loss at the design stage and is related to the size of the top-loss location being productively *** has led to diminished global thermal exchange lowered in the passage of the vane *** reverse flow adjacent to the suction side corner of the endwall is migrated farther from the vane surface,as the deviated pressure spread on the endwall accelerates the flow and progresses the reverse flow core still *** depleted association between the tornado-like vortex and the corner vortex adjacent to the suction side corner of the endwall is the dominant mechanism of control in the contoured end *** this publication,we show that the non-axisymmetric endwall contouring by selective numerical shape change method at most prominent locations is advantageous in lowering the thermal load in turbines to augment the net heat flux reduction as well as the aerodynamic performance using multi-objective optimization.

关键词： endwall contouring turbine vane heat transfer phantom cooling coolant injection net heat flux reduction aerodynamic performance

Turbine Passage secondary Flow Dynamics and Endwall Heat Transfer Under Different Inflow Turbulence

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Journal of Harbin Institute of Technology(new series) 2024年第3期31卷 51-62页

作者： Arjun K s Tide P s Biju n Division of Mechanical Engineering School of EngineeringCochin University of Science&TechnologyKalamassery 682022India

This study presents endwall hydrodynamics and heat transfer in a linear turbine cascade at Re 5×105 at low and high intensities of *** are numerically predicted using the standard ssT model and Reθ-γtransition model as well as using the high-resolution LEs *** major secondary flow components,comprising the horseshoe,corner,and passage vortices are recognized and the impact on heat or mass transfer is *** complicated behavior of turbine passage secondary flow generation and establishment are impacted by the perspective of boundary layer attributes and inflow *** passage vortex concerning the latest big leading-edge vane is generated by the enlargement of the circulation developed at the first instance adjacent to the pressure side becomes powerful and mixes with other vortex systems during its migration towards the suction *** study conclusions reveal that substantial enhancements are attained on the endwall surface,for the entire spanwise blade extension on the pressure surface,and in the highly 3-D region close to the endwall on the suction *** forecasted suction surface thermal exchange depicts great conformity with the measurement values and precisely reproduces the enhanced thermal exchange owing to the development and lateral distribution of the secondary flows along the midspan of the blade passage *** impacts of the different secondary flow structures on the endwall thermal exchange are described in depth.

关键词： turbine vane heat transfer endwall turbulence secondary flow