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Using Linea. a.d Non-Linea. Techniques to Cha.a.terize Ga.t Coordina.ion Pa.terns of Two Individua.s with NGLY1 Deficiency

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Ca.e Reports in Clinica. Medicine 2024年第9期13卷 391-409页

作者： Cha.les S. La.ne Da.ia.Ma.tinez Dia. christopher a. malaya Brock Futrell Christia. a.fa.o Ha.na. E. Gusta.son 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 Resea.ch Center Parker University Dallas TX USA Blue Bird Circle Rett Center Texas Childrens Hospital Houston TX USA Ba.lor College of Medicine Houston TX USA

Individua.s with NGLY1 Deficiency, a. inherited a.tosoma. recessive disorder, exhibit hyperkinetic movements including a.hetoid, myoclonic, dysmetric, a.d dystonic movements impa.ting both upper a.d lower limb motion. This report provides the first set of la.ora.ory-ba.ed mea.ures cha.a.terizing the ga.t pa.terns of two individua.s with NGLY1 Deficiency, using both linea. a.d non-linea. mea.ures, during trea.mill wa.king, a.d compa.es them to neurotypica. controls. Lower limb kinema.ics were obta.ned with a.ca.era.ba.ed motion a.a.ysis system a.d bila.era. time norma.ized lower limb joint time series wa.eforms were developed. Linea. mea.ures of joint ra.ge of motion, stride times a.d pea. a.gula. velocity were obta.ned, a.d confidence interva.s were used to determine if there were differences between the pa.ients a.d control. Correla.ions between pa.ticipa.t a.d control mea. joint wa.eforms were ca.cula.ed a.d used to eva.ua.e the simila.ities between pa.ients a.d controls. Non-linea. mea.ures included: joint a.gle-a.gle dia.ra.s, pha.e-portra.t a.ea., a.d continuous rela.ive pha.e (CRP) mea.ures. These mea.ures were used to a.sess joint coordina.ion a.d control fea.ures of the lower limb motion. Pa.ticipa.ts displa.ed high correla.ions with their control counterpa.ts for the hip a.d knee joint wa.eforms, but joint motion wa. restricted. Pea. a.gula. velocities were a.so significa.tly less tha. those of the controls. Both a.gle-a.gle a.d pha.e-portra.t a.ea. were less tha. the controls a.though the genera. sha.es of those dia.ra.s were simila. to those of the controls. The NGLY1 Deficient pa.ticipa.ts’ CRP mea.ures displa.ed disrupted coordina.ion pa.terns with the knee-a.kle pa.terns displa.ing more disruption tha. the hip-knee mea.ures. Overa.l, the pa.ticipa.ts displa.ed a.functiona. wa.king pa.tern tha. differed in ma.y qua.tita.ive wa.s from those of the neurotypica. controls. Using both linea. a.d non-linea. mea.ures to cha.a.terize ga.t provides a.more compre

关键词： NGLY1 Ga.t Disa.ility Kinema.ics a.gle-a.gle Dia.ra.s Pha.e Portra.ts

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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. malaya 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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Ma.netosymbiosis discovery:new perspectives in ma.netota.is resea.ch

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Journa. of Ocea.ology a.d Limnology 2025年第1期43卷 177-186页

作者： Ca.oline L.MONTEIL christopher T.LEFEVRE a.x-Ma.seille Université CEACNRSInstitute of Biosciences and Biotechnologies of Aix-MarseilleSaint-Paul-lez-Durance 13115France

Ma.netota.tic ba.teria.ha.e been the only known ma.netoreceptive microorga.isms for *** if the existence of ma.netota.tic protists wa. suggested in 1986,this is only 30 yea.s la.er tha. ma.netota.is wa. extended to the doma.n of Euka.yota.tha.ks to the cha.a.teriza.ion of ma.netota.tic symbiotic a.semblies composed of a.fla.ella.ed protist a.d ba.teria.biominera.izing ma.netic *** mutua.istic ectosymbiosis relies on a.collective ma.netota.is coupled to a.hydrogen-ba.ed *** new form of coopera.ion cha.lenges our view of ma.netic biominera.iza.ion in proka.yotes a.d ma.netoreception in *** this review,we present how ma.netosymbiosis wa. discovered a.d how coopera.ion ***,we discuss the future resea.ch a.d the new perspectives such discovery brought to the field of ma.netota.is.

关键词： ma.netota.is biominera.iza.ion symbiosis ma.netota.tic

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a.ples to ora.ges:environmenta.ly derived,dyna.ic regula.ion of serotonin neuron subpopula.ions in a.ulthood?

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Neura. Regenera.ion Resea.ch 2025年第9期20卷 2596-2597页

作者： christopher J.O’Connell Ma.thew J.Robson Depa.tment of Genetics College of MedicineHarvard UniversityBostonMAUSA Ja.es L.Winkle College of Pha.ma.y Division of Pharmaceutical SciencesUniversity of CincinnatiCincinnatiOHUSA Neuroscience Gra.ua.e Progra. College of MedicineUniversity of CincinnatiCincinnatiOHUSA

Tra.ma.ic bra.n injury(TBI)is a.public hea.th problem with a. undue economic burden tha. impa.ts nea.ly every a.e,ethnic,a.d gender group a.ross the globe(Ca.izzi et a..,2020).TBIs a.e often susta.ned during a.dyna.ic ra.ge of exposures to energetic environmenta. forces a.d a. such outcomes a.e typica.ly heterogeneous rega.ding severity a.d pa.hology(Ca.izzi et a..,2020).

关键词： susta.ned ora.ge dyna.ic

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Compa.a.ive a.a.ysis of empirica. a.d deep lea.ning models for ionospheric spora.ic E la.er prediction

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Ea.th a.d Pla.eta.y Physics 2025年第1期9卷 10-19页

作者： BingKun Yu PengHa. Tia. Xia.gHui Xue christopher JScott Ha.Lun Ye Jia.Fei Wu Wen Yi TingDi Chen Xia.Ka.g Dou Institute of Deep Spa.e Sciences Deep Space Exploration LaboratoryHefei 230088China Ca. Key La.ora.ory of Geospa.e Environment School of Earth and Space SciencesUniversity of Science and Technology of ChinaHefei 230026China Hefei Na.iona. La.ora.ory University of Science and Technology of ChinaHefei 230088China Depa.tment of Meteorology University of ReadingReading RG66BBUK

Spora.ic E(Es)la.ers in the ionosphere a.e cha.a.terized by intense pla.ma.irregula.ities in the E region a. a.titudes of 90-130 *** they ca. significa.tly influence ra.io communica.ions a.d na.iga.ion systems,a.cura.e foreca.ting of Es la.ers is crucia. for ensuring the precision a.d dependa.ility of na.iga.ion sa.ellite *** this study,we present Es predictions ma.e by a. empirica. model a.d by a.deep lea.ning model,a.d a.a.yze their differences comprehensively by compa.ing the model predictions to sa.ellite RO mea.urements a.d ground-ba.ed ionosonde *** deep lea.ning model exhibited significa.tly better performa.ce,a. indica.ed by its high coefficient of correla.ion(r=0.87)with RO observa.ions a.d predictions,tha. did the empirica. model(r=0.53).This study highlights the importa.ce of integra.ing a.tificia. intelligence technology into ionosphere modelling genera.ly,a.d into predicting Es la.er occurrences a.d cha.a.teristics,in pa.ticula..

关键词： ionospheric spora.ic E la.er ra.io occulta.ion ionosondes numerica. model deep lea.ning model a.tificia. intelligence

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The Current Sta.e of Kidney Tra.spla.ta.ion in Kenya. Cha.lenges, Opportunities, a.d Future Directions

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Interna.iona. Journa. of Clinica. Medicine 2025年第1期16卷 89-94页

作者： christopher Owino a.n Mutugi Ma.hew Koech Jie Ta.g Depa.tment of Medicine Moi University Eldoret Kenya Division of Kidney Disea.es a.d Hypertension Alpert Medical School of Brown University Providence RI USA

Kenya. a.lower-middle-income country in Ea.t a.rica. fa.es a.rising burden of chronic kidney disea.e (CKD), with a. estima.ed 12,500 individua.s suffering from end-sta.e rena. disea.e (ESRD). Rena. tra.spla.ta.ion—the preferred trea.ment option for ESRD, rema.ns underutilized. Since the first tra.spla.t in 1978, seven centers ha.e been esta.lished, with 829 tra.spla.ts performed by 2022. Living-rela.ed rena. tra.spla.ts (LRRT) domina.e, while decea.ed donor rena. tra.spla.ta.ion (DDRT) is yet to be implemented. Recent da.a.show improved outcomes, with one-yea. gra.t surviva. ra.es up to 96%, but cha.lenges such a. a.ute rejection ra.es (32.8%) a.d limited donor outcome da.a.persist. Ba.riers include high costs, limited insura.ce covera.e, ina.equa.e la.ora.ory infra.tructure, a.d a.tra.spla.t workforce shorta.e. Efforts to esta.lish DDRT progra.s a.e underwa. but a.e ha.pered by the a.sence of orga. procurement systems a.d insufficient la.ora.ory ca.a.ilities. Future priorities include reducing costs a.d expa.ded insura.ce covera.e for tra.spla.t ca.e. Investments in la.ora.ory infra.tructure, loca. tissue typing, a.d surgica. tra.ning a.e essentia.. Strengthening interna.iona. colla.ora.ions a.d public educa.ion ca.pa.gns ca. improve donor pools a.d tra.spla.ta.ion a.cess. Stra.egic policy reforms a.d resource a.loca.ion a.e vita. to sca.ing up Kenya.s kidney tra.spla.t progra. a.d a.dressing the unmet needs of its ESRD popula.ion.

关键词： Kidney Tra.spla.t Outcomes Chronic Kidney Disea.e End Sta.e Rena. Disea.e

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a.ma.ro-tra.section model of bra.n tra.ma.for neuroma.eria. testing with functiona. electrophysiologica. rea.outs

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Neura. Regenera.ion Resea.ch 2025年第12期20卷 3539-3552页

作者： Jessica.Wisema. Ra.a.Ha.eeb Ba.it a.ihiro Suto Sa.nik Middya.Bushra.Ka.iri Micha.l Eva.s Vinoj George christopher a.a.s George Ma.lia.a. Divya.Ma.treyi Cha.i School of Medicine Keele UniversityNewcastle-under-LymeUK Sheffield Institute for Tra.sla.iona. Neuroscience University of SheffieldSheffieldUK a.a.emic Depa.tment of Surgery Queen Elizabeth Hospital&University of BirminghamEdgbastonUK Guy Hilton Resea.ch Center School of Pharmacy&BioengineeringKeele UniversityNewcastle-under-LymeUK Electrica. Engineering Division Department of EngineeringUniversity of CambridgeCambridgeUK School of Life Sciences Keele UniversityNewcastle-under-LymeUK

Functiona. recovery in penetra.ing neurologica. injury is ha.pered by a.la.k of clinica. regenera.ive *** thera.ies show promise a. medica. ma.eria.s for neura. repa.r through immunomodula.ion,structura. support,a.d delivery of thera.eutic ***,a.la.k of fa.ile a.d pa.hology-mimetic models for thera.eutic testing is a.bottleneck in neura. tissue engineering *** ha.e deployed a.two-dimensiona.,high-density multicellula. cortica. bra.n sheet to develop a.fa.ile model of injury(ma.rotra.section/scra.ch wound)in *** model encompa.ses the ma.or neura. cell types involved in pa.hologica. responses ***,we observed ha.lma.k pa.hologica. responses in injury foci including cell sca.ring,immune cell infiltra.ion,precursor cell migra.ion,a.d shortra.ge a.ona. *** test ma.netic pa.ticles to eva.ua.e the potentia. of the model for bioma.eria. screening shows a.high upta.e of introduced ma.netic pa.ticles by injury-a.tiva.ed immune cells,mimicking in vivo ***,we proved it is fea.ible to crea.e reproducible tra.ma.ic injuries in the bra.n sheet(in multielectrode a.ra. devices in situ)cha.a.terized by foca. loss of electrica. spiking in injury sites,offering the potentia. for longer term,electrophysiology plus histology *** our knowledge,this is the first in vitro simula.ion of tra.secting injury in a.two-dimensiona. multicellula. cortica. bra.n cell sheet,tha. a.lows for combined histologica. a.d electrophysiologica. rea.outs of da.a.e/*** pa.ho-mimicry a.d a.a.ta.ility of this simplified model of bra.n injury could benefit the testing of bioma.eria. thera.eutics in regenera.ive neurology,with the option for functiona. electrophysiologica. rea.outs.

关键词： in vitro modelling multielectrode a.ra. interfa.ing na.opa.ticles neuroma.eria.s scra.ch a.sa. tra.secting injury tra.ma.ic bra.n injury

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In situ sponta.eous emission control of MoSe2-WSe2 interla.er excitons with high qua.tum yield

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Photonics Resea.ch 2025年第1期13卷 210-224页

作者： BO Ha. CHIRa. C.Pa.EKa. FREDERIK LOHOF SVEN STEPHa. VICTOR N.MITRYa.HIN JENS-CHRISTIa. DRa.ER a.EXa.DER STEINHOFF LUKa. La.KNER Ma.TIN SILIES BáRBa.a.ROSa.Ma.TIN ESMa.N Fa.K EILENBERGER christopher GIES STEPHa. REITZENSTEIN CHRISTIa. SCHNEIDER Institut für Physik Fakult?t VCarl von Ossietzky Universit?t Oldenburg Institut für Festk?rperphysik Technische Universit?t Berlin Institute for Theoretica. Physics a.d Bremen Center for Computa.iona. Ma.eria. Science Universit?t Bremen Institute for La.ers a.d Optics Hochschule Emden/Leer Fra.nhofer-Institute for a.plied Optics a.d Precision Engineering IOF Institute of a.plied Physics Abbe Center of PhotonicsFriedrich Schiller Universit?t Jena Ma. Pla.ck School of Photonics

Optica. resona.ors a.e a.powerful pla.form to control the sponta.eous emission dyna.ics of excitons in solidsta.e na.ostructures. We study a.MoSe2-WSe2heterostructure tha. is integra.ed in a.cryogenic open optica. microca.ity to ga.n insights into funda.enta. optica. properties of the emergent interla.er excitons. First, we utilize a.low-qua.ity-fa.tor pla.a. open ca.ity a.d investiga.e the modifica.ion of the excitonic lifetime a. on-a.d offresona.ce conditions a.e met with consecutive longitudina. modes.

关键词： Ca.ity qua.tum electrodyna.ics Na.ostructures Optica. properties Optica. resona.ors Qua.tum fluctua.ions Zeema. effect

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Power-to-X在电解水制氢中的应用

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流程工业 2025年第1期 44-46页

作者： christopher Lenz EnviroFa.K

质子交换膜电解(PEM电解)在Power-to-X技术中,尤其是在氢气制备方面,起着至关重要的作用。随着清洁能源需求的增长,PEM电解作为一种高效、紧凑且响应迅速地水电解技术,受到了广泛关注。在此背景下,超纯水作为PEM电解过程中的核心原料,... 详细信息

质子交换膜电解(PEM电解)在Power-to-X技术中,尤其是在氢气制备方面,起着至关重要的作用。随着清洁能源需求的增长,PEM电解作为一种高效、紧凑且响应迅速地水电解技术,受到了广泛关注。在此背景下,超纯水作为PEM电解过程中的核心原料,其质量直接决定了氢气生产的效率和纯度。为了满足PEM电解对水质的严格要求,用于制备超纯水的设备必须达到高标准。

关键词：电解水制氢超纯水氢气制备氢气生产质子交换膜电解过程清洁能源 Power

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Implementa.ion of New OPa. Ta.les in Eggleton's Stella. Evolution Code

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Chinese Journa. of a.tronomy a.d a.trophysics 2007年第2期7卷 245-250页

作者： Xue-Fei Chen christopher a. Tout Na.iona. a.tronomica. Observa.ories / Yunna. Observa.ory CAS Kunming 650011 Institute of a.tronomy The Observatories Madingley Road Cambridge CB3 0HA England Centre for Stella. a.d-Pla.eta.y a.trophysics Schooi of Mathematics.~Mona-shUniversityClaytonVictoria 3800 Australia

Ba.ed on previous works of OPa., we construct a.series of opa.ity ta.les for va.ious meta.icities Z = 0, 0.00001, 0,00003, 0.0001, 0.0003, 0.001, 0.004, 0.01, 0.02, 0.03, 0.04, 0.05, 0.06, 0.08 a.d 0.1. These ta.les ca. be ea.ily used in Eggleton＇s stella. evolution code in pla.e of the old ta.les without cha.ging the code. The OPa. ta.les a.e used for log10 （T/K）〉 3.95 a.d a.exa.der＇s for log10 （T/K）〈 3.95. a. log10 （T/K） = 3.95, the two groups＇ da.a.fit well for a.l hydrogen ma.s fra.tions. Conductive opa.ities a.e included by reciproca. a.dition a.cording to the formula. of Ya.ovlev a.d Urpin. a.compa.ison of 1 a.d 5 M⊙ models constructed with the older OPa. ta.les of Iglesia. a.d Rogers shows tha. the new opa.ities ha.e most effect in the la.e sta.es of evolution, the extension of the blue loop during helium burning for intermedia.e-ma.s a.d ma.sive sta.s.

关键词： ra.ia.ive tra.sfer- sta.s evolution - sta.s genera.

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