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世界核心医学期刊文摘（神经病学分册） 2005年第3期1卷 26-27页

作者： Badhwar A. berkovic s.f. Dowling J.P. E. Andermann 陈云春 Neurogenetics Unit Montreal Neurol. Hosp. and Institute 38 01 University Street Montreal Que. H3A 2B4 Canada Dr.

Action myoclonus renal failure syndrome (AMRf) is a distinctive form of progr essive myoclonus epilepsy associated with renal dysfunction. The syndrome was no t recognized prior to the advent of dialysis and renal transplantation because o f its rapidly fatal course if renal failure is untreated. The first and only des cription of AMRfwas in fourfrench Canadian patients in three families (Andermann et al., 1986). We now describe 15 individuals with AMRf from five countries, in cluding a fol low up of the four french Canadian patients, allowing a more com plete characterization of this disease. Our 15 patients with AMRf belong to nine different families. segregation analyses were compatible with autosomal recessive inheritance. In addition, our findings sho w that AMRf can present with either renal or neurological features. Tremor (onse t 17-26 years, mean 19.8 years, median 19 years) and progressively disabling ac tion myoclonus (onset 14-29 years, mean 21.7 years, median 21 years), with infr equent generalized seizures (onset 20-28 years, mean 22.7 years, median 22 year s) and cerebellar features are characteristic. Proteinuria, detected between age s 9 and 30 years in all cases, progressed to renal failure in 12 out of 15 patie nts within 0-8 years after proteinuria detection. Brain autopsy in two patients revealed extraneuronal pigment accumulation. Renal biopsies showed collapsing g lomerulopathy, a severe variant of focal glomerulosclerosis. This study extends the AMRf phenotype, and demonstrates a more extensive ethnic and geographic dist ribution of a syndrome originally believed to be confined to individuals of fren ch Canadian ancestry. The independent progression of neurological and renal diso rders in AMRf suggests a unitary molecular lesion with pleiotropic effects. Our results demonstrate that the renal lesion in AMRf is a recessive form of collaps ing glomerulopathy. Genes identified for focal segmental glomerulosclerosis and involved with the function

关键词：疾病特征肌阵挛功能障碍性动作性抗癫痫药物肾功能进行性加重肾小球病描述性研究肾小球基底膜

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The Ca^(2+)-sensing receptor and the pocketome:comparing nature's complexity with human intervention in receptor modulation

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signal Transduction and Targeted Therapy 2024年第8期9卷 3153-3154页

作者： Edda s.f.Matthees Carsten Hoffmann Institut fur Molekulare Zellbiologie CMB-Center for Molecular BiomedicineUniversitatsklinikum JenaHans-Knoll StraBe 2D-07745 JenaGermany friedrich-schiller-Universitat Jena Hans-KnollStraBe2D-07745 JenaGermany

In a recent paper published in Nature,the relatively understudied Ca^(2+)-sensing,receptor(CasR)within the Gprotein-coupled receptor(GPCR)family C,gained attention due to pioneering research led by Georgios skiniotis ... 详细信息

关键词： stanford gained skin

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Thermo-kinetic characteristics on stabilizing hetero-phase interface of metal matrix composites by crystal plasticity finite element method

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Journal of Materials science & Technology 2024年第2期169卷 53-67页

作者： Y.B.Zhang s.J.song f.Liu state Key Laboratory of solidification Processing Northwestern Polytechnical UniversityXi’an 710072China Analytical&Testing Center Northwestern Polytechnical UniversityXi’an 710072China

Using dislocation-based constitutive modeling in three-dimension crystal plasticity finite element(3D CPfE)simulations,co-deformation and instability of hetero-phase interface in different material systems were herein studied for polycrystalline metal matrix composites(MMCs).Local stress and strain fields in two types of 3layer MMCs such as.f.c/fcc Cu-Ag and fcc/bcc Cu-Nb have been predicted under simple compressive ***,more severe strain-induced interface instability can be observed in the fcc/bcc systems than in the fcc/fcc systems upon refining to metallic nanolayered composites(MNCs).By detailed analysis of stress and strain localization,it has been demonstrated that the interface instability is always accompanied by high-stress concentration,i.e.,thermodynamic characteristics,or high strain prevention i.e.,kinetic characteristics,at the hetero-phase *** then follows that the thermodynamic driving forceG and the kinetic energy barrier Q during dislocation and shear banding can be adopted to class.f. the deformation modes.f.llowing the so-called thermo-kinetic *** by inserting a high density of high-energy interfaces into the Cu-Nb composites,such thermo-kinetic integration at the hetero-phase interface allows a succes.f.l establishment of MMCs with the high△G-high Q deformation mode,which ensures high hardening and uniform strain distri-bution,thus efficiently suppressing the shear band,stabilizing the hetero-phase interface,and obtaining an exceptional combination in strength and *** hetero-phase interface chosen by a couple of thermodynamics and kinetics can be defined as breaking the thermo-kinetic correlation and has been proposed for artificially designing MNCs.

关键词： Thermodynamics and kinetics Physics-based constitutive modeling Hetero-phase interface Crystal plasticity finite element

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通过纯合子定位法确定的新的临床和分子类型的Unverricht-Lundborg病

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世界核心医学期刊文摘（神经病学分册） 2005年第8期1卷 16-16页

作者： berkovic s.f. Mazarib A. Walid s. 郭俊 Epilepsy Research Centre Neurosciences Building Repatriation Campus Heidelberg West Vic. 3081 Australia Dr.

Progressive myoclonus epilepsy (PME) has a number of causes,of which Unverrich t-Lundborg disease (ULD) is the most common. ULD has previously been mapped to a locus on chromosome 21 (EPM1). subsequently, mutations in the cystatin B gene have been found in most cases. In the present work we identified an inbred Arab family with a clinical pattern compatible with ULD, but mutations in the cystati n B gene were absent. We sought to characterize the clinical and molecular featu res of the disorder. The family was studied by multiple field trips to their tow n to clarify details of the complex consanguineous relationships and to personal ly examine the *** was collected for subsequent molecular analyses.f.om 2 1 individuals. A genome-wide screen was performed using 811 microsatellite mark ers. Homozygosity mapping was used to identify loci of interest. There were eigh t affected *** onset was at 7.3 ±1.5 years with myoclonic or t onic-clonic seizures. All had myoclonus that progressed in severity over time a nd seven had tonic-clonic seizures. Ataxia, in addition to myoclonus, occurred in all. Detailed cognitive assessment was not possible, but there was no signifi cant progressive dementia. There was intrafamily variation in severity; three re quired wheelchairs in adult life; the others could walk unaided. MRI, muscle and skin biopsies on one individual were unremarkable. We mapped the family to a 15 -megabase region at the pericentromeric region of chromosome 12 with a maximum lod score of 6.32. Although the phenotype of individual subjects was typical of ULD, the mean age of onset (7.3 years versus 11 years.f.r ULD) was younger. The locus on chromosome 12 does not contain genes.f.r any other form of PME, nor doe s it have genes known to be related to cystatin *** represents a new form of PME and we have designated the locus as EPM1B.

关键词：纯合子定位法肌阵挛性癫痫蛋白酶抑制剂基因突变分子学皮肤活组织检查进行性痴呆基因座着丝粒共济失调

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Mathematical modeling and simulations of stress mitigation by coating polycrystalline particles in lithium-ion batteries

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Applied Mathematics and Mechanics(English Edition) 2024年第6期45卷 947-962页

作者： N.IQBAL J.CHOI s.f.sHAH C.LEE s.LEE Department of Mechanical Roboticsand Energy EngineeringDongguk University-SeoulSeoul 04620Republic of Korea

A chemo-mechanical model is developed to investigate the effects on the stress development of the coating of polycrystalline Ni-rich LiNixMnyCo_(z)O_(2)(x≥0.8)(NMC)particles with poly(3,4-ethylenedioxythiophene)(PEDOT).The simulation results show that the coating of primary NMC particles significantly reduces the stress generation by efficiently accommodating the volume change associated with the lithium diffusion,and the coating layer plays roles both as a cushion against the volume change and a channel for the lithium transport,promoting the lithium distribution across the secondary particles more ***,the lower stiffness,higher ionic conductivity,and larger thickness of the coating layer improve the stress *** paper provides a mathematical framework for calculating the chemo-mechanical responses of anisotropic electrode materials and fundamental insights into how the coating of NMC active particles mitigates stress levels.

关键词： lithium-ion battery(LIB) polycrystalline particle coating finite element simulation Ni-rich LiNixMnyCo_(z)O_(2)(x>0.8)(NMC)

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Resource management at the network edge for federated learning

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Digital Communications and Networks 2024年第3期10卷 765-782页

作者： silvana Trindade Luiz f.Bittencourt Nelson L.s.da fonseca Institute of Computing State University of CampinasCampinasBrazil

federated learning has been explored as a promising solution for training machine learning models at the network edge,without sharing private user *** limited resources at the edge,new solutions must be developed to leverage the s.f.ware and hardware resources as the existing solutions did not focus on resource management for network edge,specially for federated *** this paper,we describe the recent work on resource manage-ment at the edge and explore the challenges and future directions to allow the execution of federated learning at the *** such as the discovery of resources,deployment,load balancing,migration,and energy effi-ciency are discussed in the paper.

关键词： Resource management Edge computing federated learning Machine learning

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Inflow and outflow permeability tests in a very s.f. clay under low stresses

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Journal of Rock Mechanics and Geotechnical Engineering 2024年第8期16卷 3270-3283页

作者： J.W.s.Vargas.f.A.B.Danziger f.R.Lopes T.Lunne federal University of Rio de Janeiro Rio de JaneiroBrazil Norwegian Geotechnical Institute OsloNorway

In situ inflow and outflow permeability tests with the BAT probe at sarapuíII s.f. clay test site are presented.A description of the BAT permeability test is provided,discussing its advantages and shortcomings,especially in the case of very s.f. clays under low *** pressures were monitored during probe installation and were found to be slightly lower than piezocone u2 pore pressures,consistent with the position of the *** role of filter tip saturation was investigated after the usual saturation procedure provided an unsatisfactory pore pressure response during probe *** show that the vacuum saturation procedure provides adequate response during installation and increases the reliability of the coefficient of permeability determination in early *** inflow and outflow tests yielded similar results,indicating that careful execution of the test can lead to good test repeatability regardless of the loading *** sequences of alternated inflow and outflow tests have yielded similar results,indicating that soil reconsolidation and filter clogging were negligible in the tests *** are presented concerning the relationship between index parameters and the in situ coefficient of permeability for sarapuíII clay,which plot outside the range of existing databases.

关键词： Permeability test s.f. clay BAT probe Inflow and outflow tests

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MgO-attached graphene nanosheet(MgO@GNs)reinforced magnesium matrix nanocomposite with superior mechanical,corrosion and biological performance

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International Journal of Minerals,Metallurgy and Materials 2024年第9期31卷 2062-2076页

作者： s.Abazari A.shamsipur H.R.Bakhsheshi-Rad M.s.soheilirad f.Khorashadizade s.s.Mirhosseini Department of Materials and Metallurgical Engineering Amirkabir University of TechnologyTehran ***Iran Iranian National standards Organization of Hormozgan HormozganIran Advanced Materials Research Center Department of Materials EngineeringNajafabad BranchIslamic Azad UniversityNajafabadIran Department of Materials and Metallurgical Engineering Iran University of Science and TechnologyTehranIran

Magnesium(Mg)alloys are gaining great consideration as body implant materials due to their high biodegradability and ***,they s.f.er from low corrosion resistance and antibacterial *** this research,semi-powder metallurgy followed by hot extrusion was utilized to produce the magnesium oxide@graphene nanosheets/magnesium(MgO@GNs/Mg)composite to improve mechanical,corrosion and cytocompatibility *** have revealed that the incorporation of MgO@GNs nanohybrids into Mg-based composite enhanced microhardness and compressive *** vitro,osteoblast cell culture tests show that using MgO@GNs nanohybrid fillers enhances osteoblast adhesion and apatite *** presence of MgO@GNs nanoparticles in the composites decreased the opening defects,micro-cracks and micro-pores of the composites thus preventing the penetration of the corrosive solution into the *** demonstrated that the MgO@GNs/Mg composite possesses excellent antibacterial properties because of the combination of the release of MgO and physical damage to bacterium membranes caused by the sharp edges of graphene nanosheets that can effectively damage the cell wall thereby facilitating penetration into the bacterial lipid ***,the MgO@GNs/Mg composite with high mechanical strength,antibacterial activity and corrosion resistance is considered to be a promising material for load-bearing implant applications.

关键词： metal matrix composites MgO@GNs nanohybrid strengthening mechanisms antibacterial activity biocompatibility

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All-electron basis sets.f.r H to Xe specific for ZORA calculations:Applications in atoms and molecules

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Chinese Physics B 2024年第8期33卷 238-246页

作者： C.s.Gomes.f.E.Jorge A.Canal Neto Departamento de fisica Universidade Federal do Espirito Santo29075-910 VitoriaESBrazil Departamento de fisica Instituto de Ciencias Exatas e BiologicasUniversidade Federal de Ouro Preto Campus Universitario Morro do Cruzeiro35402-136 Ouro PretoMGBrazil

A segmented basis set of quadruple zeta valence quality plus polarization functions(QZP)for H through Xe was developed to be used in conjunction with the ZORA *** set was augmented with diffuse functions to describe electrons.f.rther away from the nuclei *** the ZORA-CCsD(T)/QZP-ZORA theoretical model,atomic ionization energies and bond lengths,harmonic vibrational frequencies,and atomization energies of some molecules were *** addition of core-valence corrections has been shown to improve the agreement between theoretical and experimental results.f.r molecular *** atomization energies,a similar observation emerges when considering spin-orbit *** the augmented QZP-ZORA set,static mean dipole polarizabilities of a set of atoms were calculated and compared with previously published recommended and experimental *** evaluations of the ZORA and Douglas–Kroll–Hess Hamiltonians were made for each property studied.

关键词： QZP-ZORA and AQZP-ZORA basis sets elements.f.om H to Xe CCsD(T)method atomic and molecular properties

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Adsorption of yttrium(Y^(3+)) and concentration of rare earth elements.f.om phosphogypsum using chitin and chitin aerogel

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Journal of Rare Earths 2024年第4期42卷 775-782页

作者： Glaydson s.dos Reis Diana Pinto sabrina f.Lütke Eder C.Lima Luis.f.O.silva Irineu A.s.De Brum Guilherme L.Dotto Department of forest Biomaterials and Technology Biomass Technology Centre Swedish University of Agricultural SciencesUmeaE13Swedn Universidad De La Costa Calle 58#55-66080002BarranquillaAtlanticoColombia Research Group on Adsorptive and Catalytic Process Engineering(ENGEPAC) Federal University of Santa MariaAv.Roraima100079705900Santa MariaRSBrazil Institute of Chemistry Federal University of Rio Grande do Sul90040060Porto AlegreBrazil

Chitin powder and chitin-aerogel were prepared from shrimp wastes and used to uptake Y^(3+)from aqueous solutions and concentrate this rare earth element from phosphogypsum(PG).Chitin aerogel displays a specific surface area of 945 m^(2)/g,while chitin powder is 3.6 m^(2)/g,which largely influences its adsorption *** the adsorption in synthetic solutions,the effect of pH on Y^(3+)removal is strong for chitin powder *** contrast,no big pH influence was detected for chitin *** interactions and chelation can highlight the proposed mechanism of Y^(3+)on chitin adsorbents.f.r the powder and ***,in addition to these interactions,pore filling/pore diffusion is the main mechanism of Y^(3+)removal in the chitin *** aerogel is efficient in concentrating 8 times the Y^(3+)from PG,a very complex *** complex chitin aerogel-Y^(3+)can be a secondary source of rare earth elements.f.r other applications.

关键词： Rare earths sustainable material Chitin powder Chitin porous aerogel Yttrium adsorption and recovery

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