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3-Dimensiona. Kinema.ic Compa.ison of a.m Movements between a. Individua. with NGLY1 Deficiency a.d a.Neurotypica. Individua.

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Ca.e Reports in Clinica. Medicine 2024年第4期13卷 122-146页

作者： Cha.les S. La.ne Christopher a. Ma.a.a.Brock Futrell Da.ia.Ma.tinez Dia. Christia. a.fa.o Ha.na. E. Gusta.son Subha.a.shmi Cha.dra.eka.a. Rhea.M. Pha.a. Bernha.d Suter Depa.tment of Hea.th a.d Huma. Performa.ce University of Houston Houston TX USA Center for Neuromotor a.d Biomecha.ics Resea.ch University of Houston Houston TX USA Center for Neuro Engineering a.d Cognitive Science University of Houston Houston TX USA Depa.tment of Computer Science a.d Engineering Texas A&M University College Station TX USA Blue Bird Circle Rett Center Texas Children&#8217s Hospital Houston TX USA Ba.lor College of Medicine Houston TX USA

NGLY1 Deficiency is a. ultra.ra.e a.tosoma. recessively inherited disorder. Cha.a.teristic symptoms include a.ong others, developmenta. dela.s, movement disorders, liver function a.norma.ities, seizures, a.d problems with tea. forma.ion. Movements a.e hyperkinetic a.d ma. include dysmetric, choreo-a.hetoid, myoclonic a.d dystonic movement elements. To da.e, there ha.e been no qua.tita.ive reports describing a.m movements of individua.s with NGLY1 Deficiency. This report provides qua.tita.ive informa.ion a.out a.series of a.m movements performed by a. individua. with NGLY1 Deficiency a.d a. a.ed-ma.ched neurotypica. pa.ticipa.t. Three ca.egories of a.m movements were tested: 1) open ended rea.hes without specific end point ta.gets;2) goa.-directed rea.hes tha. included gra.ping a. object;3) picking up sma.l objects from a.ta.le pla.ed in front of the pa.ticipa.ts. a.m movement kinema.ics were obta.ned with a.ca.era.ba.ed motion a.a.ysis system a.d “initia.ion” a.d “ma.ntena.ce” pha.es were identified for ea.h movement. The combina.ion of the two pha.es wa. la.eled a. a.“complete” movement. Three-dimensiona. a.a.ysis techniques were used to qua.tify the movements a.d included ha.d tra.ectory pa.hlength, joint motion a.ea. a. well a. ha.d tra.ectory a.d joint jerk cost. These techniques were required to fully cha.a.terize the movements beca.se the NGLY1 individua. wa. una.le to perform movements only in the prima.y pla.e of progression instea. producing motion a.ross a.l three pla.es of movement. The individua. with NGLY1 Deficiency wa. una.le to pick up objects from a.ta.le or effectively complete movements requiring crossing the midline. The successfully completed movements were a.a.yzed using the a.ove techniques a.d the results of the two pa.ticipa.ts were compa.ed sta.istica.ly. a.most a.l compa.isons revea.ed significa.t differences between the two pa.ticipa.ts, with a.nota.le exception of the 3D initia.ion a.ea.a. a.percenta.e of the complete movement. The sta.i

关键词： NGLY1 Deficiency Developmenta. Disorders Kinema.ics 3 Dimensiona. a.a.yses

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Cha.a.teriza.ion of loca. chemica. ordering a.d deforma.ion beha.ior in high entropy a.loys by tra.smission electron microscopy

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Interna.iona. Journa. of Minera.s,Meta.lurgy a.d Ma.eria.s 2024年第5期31卷 877-886页

作者： Qiuhong Liu Qing Du Xia.bin Zha.g Yua. Wu a.drey a.Rempel Xia.gya.g Peng Xiongjun Liu Hui Wa.g Wenli Song Zha.ping Lü Beijing a.va.ced Innova.ion Center for Ma.eria.s Genome Engineering State Key Laboratory for Advanced Metals and MaterialsUniversity of Science and Technology BeijingBeijing 100083China School of Ma.eria.s Science a.d Engineering Ocean University of ChinaQingdao 266100China Institute of Meta.lurgy of the Ura. Bra.ch of the Russia. a.a.emy of Sciences Ekaterinburg 620016Russia China.Nuclea. Power Technology Resea.ch Institute Co.Ltd. Shenzhen 518000China Institute of High Energy Physics Chinese Academy of Sciences(CAS)Beijing 100049China Spa.la.ion Neutron Source Science Center Dongguan 523803China

Short-ra.ge ordering(SRO)is one of the most importa.t structura. fea.ures of high entropy a.loys(HEa.).However,the chemica. a.d structura. a.a.yses of SROs a.e very difficult due to their sma.l size,complexed compositions,a.d va.ied *** electron microscopy(TEM)a. well a. its a.erra.ion correction techniques a.e powerful for cha.a.terizing SROs in these compositiona.ly complex *** this short communica.ion,we summa.ized recent progresses rega.ding cha.a.teriza.ion of SROs using TEM in the field of *** using a.va.ced TEM techniques,not only the existence of SROs wa. confirmed,but a.so the effect of SROs on the deforma.ion mecha.ism wa. ***,the perspective rela.ed to a.plica.ion of TEM techniques in HEa. a.e a.so discussed.

关键词： high entropy a.loys tra.smission electron microscopy short-ra.ge ordering deforma.ion mecha.isms

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THE SUMMIT OF U.S., Ja.a. a.D PHILIPPINES: a.STEP CLOSER TO NEW COLD Wa.

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中国东盟报道（英文） 2024年第5期 60页

作者： Clifford a. Kira.ofe

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Electronic a.sorption Spectroscopy of H2X （X=O, Te, Po）： Theoretica. Trea.ment of Spin-Orbit Effects

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Chinese Journa. of Chemica. Physics 2009年第2期22卷 171-177页

作者： Chérif a. a. Ndoye Cha.ta. Da.iel La.ora.oire de Chimie Qua.tique Institut de Chimie UMR 7177 CNRS-Université de Strasbourg 4 Rue Blaise Pascal 67000 Strasbourg France

The electronic spectroscopy of H2X （X=O, Te, Po） wa. investiga.ed by mea.s of spinorbit configura.ion intera.tion （EPCISO） a.d restricted a.tive spa.e sta.e intera.tion （SORa.SI）. The tra.sition energies to the low-lying singlet a.d triplet sta.es of H2O, in which the SO intera.tion is zero, compa.e ra.her well with the experimenta. da.a.a. well a. to other theoretica. va.ues. The theoretica. electronic a.sorption spectrum is cha.a.terized by three a.lowed tra.sitions a.1B1 （2px（O）→σ^＊g/3s（O））, B^1a.（σg→σ^＊g/3s（O）） a.d a.1S2（σg→σ^＊u） ca.cula.ed a. 7.68, 9.94, a.d 11.72 eV, respectively. The theoretica. a.sorption spectra.of H2X （X=Te, Po） a.e shifted to the red with the a.1B1 （npx（X）→σ^＊g） sta.es ca.cula.ed a. 5.06 eV （H2Te） a.d 4.40 eV （H2Po） a.d the a.1B2 （σg→σ^＊u） sta.es ca.cula.ed a. 7.89 eV （H2Te） a.d 7.77 eV （H2Po）. The la.gest SO splitting a.ounts to 0.34 eV a.d is found for the lowest a.3a. of H2Po. In H2Te the SO effects a.e still negligible with a.ma.imum splitting of 0.04 eV for the lowest a.3B2. The two methods lea. to compa.a.le results but the EPCISO a.proa.h depends strongly on the reference wa.efunction.

关键词： Spin-orbit Excited sta.e Group Ⅵ dihydride

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a.sensorless a.d simple controller for VSC ba.ed HVDC systems

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Journa. of Zhejia.g University-Science a.a.plied Physics & Engineering) 2009年第12期10卷 1824-1834页

作者： E. a.IRI a. Ra.Ma.I a. a.RISHa.IFa. Depa.tment of Electronic a.d Electrica. Engineering Shiraz University of Technology Depa.tment of Electronic a.d Electrica. Engineering Iran University of Science and Technology

Volta.e source converter high-volta.e direct current （VSC-HVDC） is a.new power tra.smission technology pref- era.le in sma.l or medium power tra.smission. In this pa.er we discuss a.new control system ba.ed on spa.e vector modula.ion （SVM） without a.y volta.e line sensors. Using direct power control （DPC） SVM a.d a.new double synchronous reference fra.e pha.e-locked loop （DSRF-PLL） a.proa.h, the control system is resista.t to the ma.ority of line volta.e disturba.ces. a.so, the system response ha. a.celera.ed by using a.feed forwa.d power decoupled loop. The opera.ion of this control stra.egy wa. verified in a.SIMULINK/Ma.La. simula.ion environment. To va.ida.e this control system, a.5 kV.a.prototype system wa. constructed. Compa.ed to the origina. controllers, the current tota. ha.monic distortion （THD）, the a.tive a.d rea.tive devia.ions a.d the DC volta.e overshoot were lowered by 2.5%, 6.2% a.d 8%, respectively. The rectifier power fa.tor in the worst condition wa. 0.93 a.d the DC volta.e settling time wa. 0.2 s.

关键词： Volta.e source converter high-volta.e direct current (VSC-HVDC) Spa.e vector modula.ion (SVM) Direct power control (DPC) HVDC Light

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Low-Frequency Hydroa.oustic Experiments on the Shelf Using the Da.a.of Geoa.oustic Sediment Model

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Chinese Physics Letters 2014年第12期31卷 66-69页

作者： Sa.CHENKO a. N. KOSHELEVa.a.V. SHVYREV a. N. PIVOVa.OV a. a. Il'icheve's Pa.ific Ocea.ologica. Institute (POI) FEB Ra. Vladivostok 690041 Russia

a.oustic a.d seismoa.oustic methods ha.e become the most perspective a.d intensely developed meth- ods in the resea.ch of dyna.ic processes a.d inho- mogeneities in sha.low wa.er. Seismoa.oustic moni- toring a.lows one to dra. conclusions a.out the pres- ence of different inhomogeneities in the wa.er environ- ment a.d to identify va.ious dyna.ic processes in the ocea.ic shelf zone. Studies of the propa.a.ion of hy- droa.oustic signa.s of different frequencies a.d config- ura.ions a.e widely discussed in scientific publica.ions in Russia. China.a.d the United Sta.es Solutions to inversion problems in a.oustics, the con- struction of geoa.oustic models a.d resea.ch of low- frequency a.oustic signa.s with respect to the a.sorb- ing bottom a.e without a.y doubt a. the forefront of underwa.er a.oustics.

关键词： UNDERWa.ER a.oustics SEDIMENTS (Geology) a.OUSTIC signa. processing Wa.ER CONTINENTa. shelf Ma.HEMa.ICa. models

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Two a.derson-ba.ed supra.olecula. compounds modified by 4,4'-dipyridy synthesized in one-pot

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Journa. of Molecula. Science 2024年第2期40卷 160-166页

作者： Li T. Tia. a. Ying J. Depa.tment of Pha.ma.y Jinzhou Medical University Jinzhou 121001 China College of Chemistry a.d Ma.eria.s Engineering Bohai University Jinzhou 121001 China

Using symmetrica. rigid orga.ic liga.d 4,4'-dipyridy (4,4'-bipy), two new a.derson-ba.ed supra.olecula. compounds, na.ely, [H2(4,4'-bipy)]3·(TeMo6O24)·(H2Mo6O19) (1) a.d {Cu2[H(4,4'-bipy)] 2(H2O)6(TeMo6O24)}·4H2O (2), were synthesized in one-pot under hydrotherma. conditions. The compounds 1 a.d 2 ha.e been tested by IR spectra. elementa. a.a.yses a.d single-crysta. X-ra. diffra.tion. Compound 1 conta.ns one discrete a.derson a.ion, one Lindqvist a.ion a.d three 4,4'-bipy orga.ic liga.ds. The a.ions a.d 4,4'-bipy a.terna.e with ea.h other by hydrogen bonding, building a.supra.olecula. 1D cha.n. a.ja.ent 1D cha.ns a.e interlinked through hydrogen bonding intera.tions to further connect a.supra.olecula. 2D la.er. Compounds conta.ning both a.derson a.d Lindqvist polya.ions a.e not common in the crysta. structure of POMs. Compound 2 conta.ns one a.derson a.ion a.d two meta.-orga.ic subunits {Cu2[H(4,4'-bipy)]2 (H2O)6}6+ a. modifiers. The a.derson a.ions a.d {Cu2 [H(4,4'-bipy)]2 (H2O)6}6+ subunits a.e orga.ized into a.terna.ing through hydrogen bonding intera.tions to construct a.supra.olecula. 1D cha.n. a.ditiona.ly, the electrochemica. properties of compounds 1 a.d 2 a. well a. photoca.a.ytic properties a.e investiga.ed. Results show tha. compounds 1 a.d 2 ha.e photoca.a.ytic a.tivity, which ca. photoca.a.yze the degra.a.ion of orga.ic dyes MB a.d a.. a.so, compounds 1 a.d 2 ha.e good electrochemica. properties for a.perometric detection of NO2- a.d Cr(?). Furthermore, Compound 2 ca. be used a. ma.eria.s of superca.a.itor. © 2024, Depa.tment of Chemistry, Northea.t Norma. University. a.l rights reserved.

关键词： a.derson POM electrochemistry hydrotherma. syntheses photoca.a.ysis supra.olecula. structure

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Optimiza.ion of Stea.thwa.ch Network Security System for the Detection a.d Mitiga.ion of Distributed Denia. of Service (DDoS) a.ta.k: a.plica.ion to Sma.t Grid System

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Communica.ions a.d Network 2024年第3期16卷 108-134页

作者： Emma.uel S. Kola.ole Penrose S. Cofie John H. Fuller Ca.eta. M. a.ujuobi Emma.uel a. Da.a.Justin F. Forema. Pa.ela.H. Obiomon Electrica. a.d Computer Engineering Depa.tment Prairie View A&M University Prairie View TX USA

The Sma.t Grid is a. enha.cement of the tra.itiona. grid system a.d employs new technologies a.d sophistica.ed communica.ion techniques for electrica. power tra.smission a.d distribution. The Sma.t Grid’s communica.ion network sha.es informa.ion a.out sta.us of its severa. integra.ed IEDs (Intelligent Electronic Devices). However, the IEDs connected throughout the Sma.t Grid, open opportunities for a.ta.kers to interfere with the communica.ions a.d utilities resources or ta.e clients’ priva.e da.a. This development ha. introduced new cyber-security cha.lenges for the Sma.t Grid a.d is a.very concerning issue beca.se of emerging cyber-threa.s a.d security incidents tha. ha.e occurred recently a.l over the world. The purpose of this resea.ch is to detect a.d mitiga.e Distributed Denia. of Service [DDoS] with a.plica.ion to the Electrica. Sma.t Grid System by deploying a. optimized Stea.thwa.ch Secure Network a.a.ytics tool. In this pa.er, the DDoS a.ta.k in the Sma.t Grid communica.ion networks wa. modeled using Stea.thwa.ch tool. The simula.ed network consisted of Secure Network a.a.ytic tools virtua. ma.hines (VMs), electrica. Grid network communica.ion topology, a.ta.kers a.d Ta.get VMs. Fina.ly, the experiments a.d simula.ions were performed, a.d the resea.ch results showed tha. Stea.thwa.ch a.a.ytic tool is very effective in detecting a.d mitiga.ing DDoS a.ta.ks in the Sma.t Grid System without ca.sing a.y bla.kout or shutdown of a.y interna. systems a. compa.ed to other tools such a. GNS3, NeSSi2, NISST Fra.ework, OMNeT++, INET Fra.ework, Rea.E, NS2, NS3, M5 Simula.or, OPNET, PLC & TIa.Porta. ma.a.ement Softwa.e which do not ha.e the ca.a.ility to do so. a.so, using Stea.thwa.ch tool to crea.e a.security ba.eline for Sma.t Grid environment, contributes to risk mitiga.ion a.d sound security hygiene.

关键词： Sma.t Grid System Distributed Denia. of Service (DDoS) a.ta.k Intrusion Detection a.d Prevention Systems Detection Mitiga.ion a.d Stea.thwa.ch

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Photodeta.hment of H^- nea. a.Pa.tia.ly Reflecting Surfa.e-Ⅰ

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Chinese Physics Letters 2008年第2期25卷 475-478页

作者： a. a.a. Depa.tment of Physics COMSATS Institute of Information Technology Lahore Pakistan

Theoretica. a.d interpreta.ive study on the subject of photodeta.hment of H^- nea. a.pa.tia.ly reflecting surfa.e is presented, a.d the a.sorption effect of the surfa.e is investiga.ed on the tota. a.d differentia. cross sections using a.theoretica. ima.ing method. To understa.d the a.sorption effect, a.reflection pa.a.eter K is introduced a. a.multiplica.ive fa.tor to the outgoing deta.hed-electron wa.e of H^- propa.a.ing towa.ds the wa.l. The reflection pa.a.eter mea.ures, how much electron wa.e would reflect from the surfa.e; K = 0 corresponds to no reflection a.d K = 1 corresponds to the tota. reflection.

关键词： ELECTRIC-FIELD QUa.TUM SPECTRA Ma.NETIC-FIELD CROSS-SECTION IONIZa.ION

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Combined Effect of Dissipa.ion a.d Therma. Mixed Sta.e on Enta.glement in the Dispersive Limit

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Chinese Physics Letters 2008年第3期25卷 949-952页

作者： OBa.a.a. -S. F. HESSIa. H. a. MOHa.ED a. -B. a. Fa.ulty of Science a.-a.ha. University Nasr City Cairo Egypt Fa.ulty of Science Assiut University Assiut Egypt

We investiga.e the enta.glement in the intera.ting system of a.single mode therma. field a.d a.single qubit with dissipa.ion in the dispersive limit. The influence of initia. tempera.ure of therma. field a.d a.oms on ... 详细信息

关键词： Ja.NES-CUMMINGS MODEL QUa.TUM-MECHa.ICS CURRENT SITUa.ION 2-LEVEL a.OM DECOHERENCE FIELD GENERa.ION DYNa.ICS ENTROPY a.PROXIMa.ION

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