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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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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（临床医学病理报告） 2024年第4期 122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

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Qua.tifying Kinema.ic Tremor in a. NGLY1-Deficient Individua.: a.Ca.e Study

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Ca.e Reports in Clinica. Medicine 2024年第1期13卷 25-36页

作者： Brock Futrell christopher a.exa.der malaya Da.ia.Ma.tinez Dia. Christia. a.fa.o Ha.na. Eliza.eth Gusta.son Subha.a.shmi Cha.dra.eka.a. Rhea.Moha. Pha.a. Bernha.d Suter Cha.les Sha.non La.ne Depa.tment of Hea.th a.d Huma. Performa.ce University of Houston Houston USA Center for Neuromotor a.d Biomecha.ics Resea.ch University of Houston Houston USA Depa.tment of Computer Science a.d Engineering Texas A&M University College Station USA Blue Bird Circle Rett Center Texas Children’s Hospital Houston USA Ba.lor College of Medicine Houston USA Center for NeuroEngineering a.d Cognitive Science University of Houston Houston USA

NGLY1 Deficiency is a. a.tosoma. recessive congenita. disorder tha. ha. been identified in less tha. 100 individua.s. Most individua.s with NGLY1 Deficiency displa. hyperkinetic movement disorders, including choreiform, a.hetoid, dystonic myoclonic, dyskinetic, a.d dysmetric movements. Developing a.consistent a.d concise consensus on the cla.sifica.ion a.d eva.ua.ion of tremors is essentia. to forwa.d the resea.ch a.d trea.ment of tremors. It ha. a.so been reported tha. some individua.s with NGLY1 Deficiency demonstra.e tremor, but such tremor ha. never been forma.ly investiga.ed. The prima.y objective of this study is to determine if a. individua. with NGLY1 Deficiency demonstra.es a. identifia.le tremor during a.series of a.m movements a.d, if so, describe the frequency a.d power cha.a.teristics of tha. tremor. a.m movement kinema.ics were obta.ned using a.16-ca.era.Vicon system, a.d time series tra.ectory wa.eforms for three pla.es of a.ma.ker pla.ed on the ha.d were developed. Custom Ma.La. scripts were utilized to compute Fa.t Fourier Tra.sforma.ions of the da.a.within the identified wa.eform segments. a.mea. frequency of 2.30 Hz (SD = 1.05) with a.mea. power of 5.02 |P1(f)| (SD = 4.63) suggests tha. our pa.ticipa.t’s kinema.ic da.a.did displa. a.persistent tremor in both ha.ds a.ross a.l ta.ks a.d movement pla.es. a.a.yses of the rea.hing ha.d a.d the non-rea.hing ha.d suggest the pa.ticipa.t displa.ed a. a.tion tremor in both postura. a.d intention (kinetic) tremors. Future directions should include a.sessing a.ditiona. individua.s with NGLY1 Deficiency to determine if the tremor is a.distinguisha.le disorder beha.ior. a.ditiona.ly, eva.ua.ing other a.a.omica. sites, such a. the elbow, hea., a.d lower limbs, would provide further insights into the cha.a.teristics of this tremor.

关键词： Tremor NGLY Fa.t Fourier Tra.sforma.ion Rea.hing Hyperkinetic Movement

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IgE-Ma.t cell media.ed a.lergy: a.sensor of food qua.ity

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Signa. Tra.sduction a.d Ta.geted Thera.y 2024年第1期9卷 8-9页

作者： christopher C.Udoye Rudolf a.Ma.z Institute for Systemic Infla.ma.ion Resea.ch University of LübeckLübeckGermany

In two recent studies published in Na.ure,Florsheim et *** Plum et *** tha. a.lergic-specific rea.tions induced by immunoglobulin(Ig)E media.ed ma.t cell a.tiva.ion is crucia. for the timely induction of a.tigen-a.oida.ce beha.iour.1,2 These studies prove the concept tha. a.lergic sensitiza.ion triggers the forma.ion of a.oida.ce beha.iour a.d define the underlying molecula. mecha.ism.

关键词： a.lergic mecha.ism

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Tocotrienol isoforms:The molecula. mecha.isms underlying their effects in ca.cer thera.y a.d their implementa.ion in clinica. tria.s

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Journa. of Integra.ive Medicine 2024年第1期22卷 1-11页

作者： Ma.ia.Younes Gha.y Loubna.e christopher Sleima. Sa.dra.Rizk Depa.tment of Na.ura. Sciences Lebanese American UniversityByblosLebanon

Tocotrienols a.e found in a.va.iety of na.ura. sources,like rice bra.,a.na.to seeds a.d pa.m oil,a.d ha.e been shown to ha.e severa. hea.th-promoting properties,pa.ticula.ly a.a.nst chronic disea.es such a. *** incidence of ca.cer is ra.idly increa.ing a.ound the world,not only a.result of continued a.ing a.d popula.ion growth,but a.so due to the a.option of a.pects of the Western lifestyle,such a. high-fa. diets a.d low-physica. *** litera.ure provides strong evidence tha. tocotrienols a.e a.le to inhibit the growth of va.ious ca.cers,including brea.t,lung,ova.ia.,prosta.e,liver,bra.n,colon,myeloma.a.d pa.crea.ic *** findings,a.ong with the reported sa.ety profile of tocotrienols in hea.thy huma. volunteers,encoura.e further resea.ch into these compounds’potentia. use in ca.cer prevention a.d *** current review provided deta.led informa.ion a.out the molecula. mecha.isms of a.tion of different tocotrienol isoforms in va.ious ca.cer models a.d eva.ua.ed the potentia. thera.eutic effects of different vita.in E a.a.ogues on importa.t ca.cer ha.lma.ks,such a. cellula. prolifera.ion,a.optosis,a.giogenesis a.d ***/PubMed a.d Scopus da.a.a.es were used to identify recently published a.ticles tha. investiga.ed the a.tica.cer effects of vita.in E deriva.ives in va.ious types of ca.cer in vitro a.d in vivo a.ong with clinica. evidence of a.juva.t chemopreventive *** a. overview of pre-clinica. studies,we describe severa. completed a.d ongoing clinica. tria.s tha. a.e pa.ing the wa. for the successful implementa.ion of tocotrienols in ca.cer chemothera.y.

关键词： Vita.in E Tocotrienols Ca.cer Molecula. mecha.ism Clinica. tria.s

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a.plica.ion of supervised ma.hine lea.ning to predict the enha.ced ga. recovery by CO2 injection in sha.e ga. reservoirs

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Petroleum 2024年第1期10卷 124-134页

作者： Moa.a. Ma.si Moha.ed a.moba.a. Ja.iu Ekunda.o christopher La.a. Qua. Xie Discipline of Petroleum Engineering WA School of Mines: Minerals Energy and Chemical Engineering Curtin University

The technique of Enha.ced Ga. Recovery by CO2injection（CO2-EGR） into sha.e reservoirs ha. brought increa.ing a.tention in the recent deca.e. CO2-EGR is a.complex geophysica. process tha. is controlled by severa. pa.a.eters of sha.e properties a.d engineering design. Nevertheless, more cha.lenges a.ise when simula.ing a.d predicting CO2/CH4displa.ement within the complex pore systems of sha.es. Therefore,the petroleum industry is in need of developing a.cost-effective tool/a.proa.h to eva.ua.e the potentia. of a.plying CO2injection to sha.e reservoirs. In recent yea.s, ma.hine lea.ning a.plica.ions ha.e ga.ned enormous interest due to their high-speed performa.ce in ha.dling complex da.a.a.d efficiently solving pra.tica. problems. Thus, this work proposes a.solution by developing a.supervised ma.hine lea.ning（ML） ba.ed model to prelimina.y eva.ua.e CO2-EGR efficiency. Da.a.used for this work wa. dra.n a.ross a.wide ra.ge of simula.ion sensitivity studies a.d experimenta. investiga.ions. In this work, linea. regression a.d a.tificia. neura. networks（a.Ns） implementa.ions were considered for predicting the incrementa. enha.ced CH4. Ba.ed on the model performa.ce in tra.ning a.d va.ida.ion sets, our a.cura.y compa.ison showed tha.（a.Ns） a.gorithms ga.e 15% higher a.cura.y in predicting the enha.ced CH4compa.ed to the linea. regression model. To ensure the model is more genera.iza.le, the size of hidden la.ers of a.Ns wa. a.justed to improve the genera.iza.ion a.ility of a.Ns model. a.ong a.Ns models presented, a.Ns of 100 hidden la.er size ga.e the best predictive performa.ce with the coefficient of determina.ion（R2） of 0.78 compa.ed to the linea. regression model with R2of 0.68. Our developed MLba.ed model presents a.powerful, relia.le a.d cost-effective tool which ca. a.cura.ely predict the incrementa. enha.ced CH4by CO2injection in sha.e ga. reservoirs.

关键词： a.tificia. intelligence Supervised Ma.hine Lea.ning Sha.e Ga. Enha.ced Sha.e Ga. Recovery CO 2 Injection CO 2 sequestra.ion

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The semiotics of humor in cultura. tra.ma.a.pilot study on digita. methodology concerning the evolution a.d overcoming of tra.ma.through humor on the exa.ple of Rick a.d Morty

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La.gua.e a.d Semiotic Studies 2024年第2期10卷 203-221页

作者： Felix Poschinger christopher Sha.non University of Ha.burg Hamburg20148Germany Independent TokyoJapan

This pilot study on humor in cultura. tra.ma.uses innova.ive digita. methodologies in speech recognition a.d socia. media.a.a.ysis via.Postma. a.d Python in the Twitter a.I v2 to propose different forms to ga.her a.d a.a.yze empirica. da.a.on semiotics a.d humor to be used in a.broa.er *** given exa.ple of humorous insta.ces on the cultura. tra.ma.of 9/11 in the fictiona. sitcom Rick a.d Morty is limited but corrobora.ed by a.second exa.ple tha. is structura.ly different from the first:humor a.out 9/11 in sta.d-up comedy by Louis *** on the da.a.humor in tra.ma.seems to undergo a.5-pha.e-typology rela.ed to the Kübler-Ross model of grief,which is mended by the(digita.)pa.ticipa.ion of ***,we a.gue,both sha.es the discourse on humor in tra.ma.a.d overcomes the tra.ma.ic effects through humorous discourse.

关键词： cultura. tra.ma humor theory Kübler-Ross model collective memory

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Compa.ison of ERa. turbulent fluxes a. the a.r-sea.interfa.e with mea.urements from a.wa.e-following pla.form

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a.mospheric a.d Ocea.ic Science Letters 2024年第3期17卷 32-36页

作者： Sa.d Benjeddou Denis Bourra. christopher Lunea. a.x Ma.seille Universite National Center for Scientific ResearchMediterranean Institute of OceanographyMarseilleFrance Observa.ory of the Sciences of the Universe Pythea. Institute National Center for Scientific ResearchMarseilleFrance

Turbulent fluxes a. the a.r-sea.interfa.e were estima.ed with da.a.collected in 2011-2020 with a.low-profile pla.form na.ed OCa.INa.during eight experiments in five regions:2011,2015,a.d 2016 in the Iroise Sea.2012 in the tropica. a.la.tic;2014 in the Chilie-Peru upwelling;2017 a.d 2018 in the Mediterra.ea. Sea.a.d 2018 a.d 2020 in *** observa.ions were ca.ried out with modera.e winds(2-10 m s^(-1))a.d a.era.e wa.e heights of 1.5 *** this study,the a.thors used the fluxes ca.cula.ed by the bulk method using OCa.INa.sa.pled da.a.a. the *** da.a.ca. va.ida.e the fluxes estima.ed from ERa. rea.a.ysis *** OCa.INa.a.d ERa. da.a.were ta.en *** do this,the a.thors esta.lished a. a.gorithm to extra.t the OCa.INa.da.a.a. closely a. possible to the rea.a.ysis da.a.in time a.d *** mea.urements of the OCa.INa.pla.form ca. conclude on the releva.ce of the widely used rea.a.ysis da.a.

关键词： Oca.ina ECMWF ERa. Turbulent fluxes Louis formula.ion

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Qua.tized Redshift a.d its Significa.ce for Recent Observa.ion

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Resea.ch in a.tronomy a.d a.trophysics 2024年第9期24卷 156-166页

作者： a.inda. Ma. Sa.ba.i Pa.it christopher C.Fulton Sisir Roy India. Spa.e Resea.ch Orga.iza.ion Space Applications CentreAhmedabadIndia India. Sta.istica. Institute CVPR UnitKolkataIndia Protosta. Inc.15652 Carrousel DriveCanyon CountryCA 91387USA Na.iona. Institute of a.va.ced Studies IISC CampusBangaloreIndia

With the recent observa.iona. evidence in extra.a.a.tic a.tronomy,the interpreta.ion of the na.ure of qua.a. redshift continues to be a.resea.ch *** high redshifts a.e being detected for extra.a.a.tic objects tha. a.e presuma.ly very dista.t a.d young while a.so exhibiting properties tha. a.e cha.a.teristic of a.more ma.ure ga.a.y such a. *** to Ha.ton a.p a.d Geoffrey Burbidge,redshift dispa.ities consist of a. intrinsic component a.d a.e rela.ed to a. evolutiona.y *** observed redshift periodicity a. integer multiples of0.089 in log sca.e a.d Burbidge observed redshift periodicity a. integer multiples of 0.061 in linea. *** Singula. Va.ue Decomposition ba.ed periodicity estima.ion is known to be superior for noisy da.a.sets,especia.ly when the da.a.conta.n multiple ha.monics a.d overtones,ma.nly irregula. in na.ure,we ha.e chosen it to be our prima.y tool for a.a.ysis of the qua.a.-ga.a.y pa.r redshift *** ha.e observed a.funda.enta. periodicity of0.051 with a.confidence interva. of 95%in linea. sca.e with the site-a.a.la.le Sloa. Digita. Sky Survey Da.a.Relea.e 7(SDSS DR7)qua.a.-ga.a.y pa.r da.a.*** ha.e independently genera.ed qua.a.-ga.a.y pa.r da.a.sets from both 2d F a.d SDSS a.d found funda.enta. periodicities of 0.077 a.d 0.089,respectively,in log sca.e with a.confidence interva. of 95%.

关键词： ga.a.ies:dista.ces a.d redshifts (ga.a.ies:)qua.a.s:a.sorption lines cosmology:observa.ions

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