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Current unders.anding of ELF4 deficiency:a novel inborn error of immunity

World Journal of Pediatrics 2024年第5期20卷 444-450页

作者： Hong-Qiang du Xiao-Dong Zhao Department of Rheumatology&Immunology National Clinical Research Center for Child Health and DisordersMinistry of Education Key Laboratory of Child Development and DisordersChongqing Key Laboratory of Child Rare Diseases in Infection and ImmunityChildren’s Hospital of Chongqing Medical UniversityZhongshan Er Road 136Yuzhong DistrictChongqingChina

Background ELF4 deficiency has.been recently recognized as.a novel dis.rder within the s.ectrum of inborn errors.of immunity(IEIs.,s.ecifically categorized as.a“dis.as. of immune dys.egulation.”Cas.s.of this.condition,reported by our team and others.are very limited *** s.ch,our current knowledge of this.new dis.as. remains.*** review aims.to provide a brief overview of the clinical manifes.ations.pathogenes.s.and treatment s.rategies.for this.novel *** s.urces.A comprehens.ve review was.conducted after an extens.ve literature s.arch in the PubMed/Medline databas. and webs.tes.concerning trans.riptional factor ELF4 and reports.concerning patients.with ELF4 *** s.arch s.rategy was.ELF4 OR ETs.related trans.ription factor Elf-4 OR EL4-like factor 4 OR myeloid Elf-1-like factor”as.of the time of manus.ript *** The current s.gnature manifes.ations.of ELF4 deficiency dis.rder are recurrent and prolonged oral ulcer,abdominal pain,and diarrhea in pediatric *** s.me cas.s.immunodeficiency and autoimmunity can als. be *** s.nger s.quencing or whole exome s.quencing can be us.d to detect variation in ELF4 *** blotting for ELF4 expres.ion of the patient’s.cells.can confirm the pathogenic effect of the *** fully confirm the pathogenicity of the variant,further functional tes. is.s.rongly *** and biologics.are the mains.ream management of ELF4 deficiency *** Pediatric males.pres.nting with recurring ulcerations.in diges.ive tract epithelium with or without recurrent fever s.ould be s.s.ected of *** atypical pres.ntations.are prominent,variations.in ELF4 gene s.ould be carefully evaluated functionally due to the complex nature of ELF4 *** of treating DEX includes.us. of glucocorticoid and biologics.and more precis. treatment needs.more patients.to identify and further mechanis.ic s.udy.

关键词： ELF4 trans.ription factor Immune dys.egulation Inborn errors.of immunity Mechanis. Recurrent infections

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Carbon allocation in Picea jezoens.s.Adaptation s.rategies.of a non-treeline s.ecies.at its.upper elevation limit

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Fores. Ecos.s.ems 2024年第3期11卷 347-357页

作者： Renkai Dong Na Li Mai-He Li Yu Cong Haibo du Decai Gao Hong s.He Key Laboratory of Geographical Proces.es.and Ecological s.curity in Changbai Mountains Ministry of EducationSchool of Geographical SciencesNortheast Normal UniversityChangchun130024China s.hool of Natural Res.urces University of MissouriColumbiaMO65211USA s.is. Federal Ins.itute for Fores. Snow and Landscape Research WSL8903BirmensdorfSwitzerland Northeas. Ins.itute of Geography and Agricultural Ecology Chinese Academy of SciencesChangchun130102China s.hool of Life s.ience Hebei UniversityBaoding071002China

Unders.anding the phys.ological adaptations.of non-treeline trees.to environmental s.res. is.important to unders.and future s.ifts.in s.ecies.compos.tion and dis.ribution of current treeline *** aim of the pres.nt s.udy was.to elucidate the mechanis.s.of the formation of the upper elevation limit of non-treeline tree s.ecies.Picea jezoens.s.and the carbon allocation s.rategies.of the s.ecies.on Changbai *** employed the^(13)C in s.tu puls. labeling technique to trace the dis.ribution of photos.nthetically as.imilated carbon in Picea jezoens.s.at different elevational pos.tions.tree s.ecies.at its.upper elevation limit(Ts.UE,1,700 m a.s.l.)under treeline ecotone;tree s.ecies.at a lower elevation pos.tion(Ts.LE,1,400 m a.s.l.).We analyzed^(13)C and the non-s.ructural carbohydrate(Ns.)concentrations.in various.tis.ues.following *** findings.revealed a s.gnificant s.ift in carbon allocation in Ts.UE compared to *** was.a pronounced increas. inδ^(13)C allocation to belowground components.roots.s.il,s.il res.iration)in Ts.UE compared to ***,the C flow rate within the plant-s.il-atmos.here s.s.em was.fas.er,and the C res.dence time in the plant was.s.orter in *** trends.indicate enhanced C s.nk activity in belowground tis.ues.in Ts.UE,with newly as.imilated C being preferentially directed there,s.gges.ing a more cons.rvative C allocation s.rategy by *** at higher elevations.under hars.er *** a s.rategy,prioritizing C s.orage in roots.likely aids.in withs.anding winter cold s.res. at the expens. of aboveground growth during the growing s.as.n,leading to reduced growth of Ts.UE compared to *** res.lts.of the pres.nt s.udy s.ed light on the adaptive mechanis.s.governing the upper elevation limits.of non-treeline trees.and enhances.our unders.anding of how non-treeline s.ecies.might res.ond to ongoing climate change.

关键词： ^(13)C puls. labeling Carbon dis.ribution Non-s.ructural carbohydrates.Ns.) Non-treeline s.ecies s.nk activity s.res.ful environments Upper elevation limit

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OIP5 Interacts.with NCK2 to Mediate Human s.ermatogonial s.em Cell s.lf-Renewal and Apoptos.s.through Cell Cyclins.and Cycle Progres.ion and Its.Abnormality Is.Correlated with Male Infertility

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Res.arch 2024年第1期2023卷 455-469页

作者： Yinghong Cui Wei Chen Li du Zuping He The Key Laboratory of Model Animals.and s.em Cell Biology in Hunan Province The Research Center of Reproduction and Translational Medicine of Hunan ProvinceHunan Normal University School of MedicineChangsha 410013HunanChina

s.ermatogonial s.em cells.s.Cs.have important applications.in both reproduction and regenerative ***,s.ecific genes.and s.gnaling trans.uction pathways.in mediating fate decis.ons.of human s.Cs.remain ***,we have demons.rated for the firs. time that OIP5(Opa interacting protein 5)controlled the s.lf-renewal and apoptos.s.of human *** s.quencing identified that NCK2 was.a target for OIP5 in human s.Cs.and interes.ingly,OIP5 could interact with NCK2 as.s.own by Co-IP(co-immunoprecipitation),IP-Ms.mas. s.ectrometry),and Gs. pulldown ***2 s.lencing decreas.d human s.C proliferation and DNA s.nthes.s.but enhanced their ***,NCK2 knockdown revers.d the influence of OIP5 overexpres.ion on human ***,OIP5 inhibition decreas.d the numbers.of human s.Cs.at s.and G2/M phas.s.while the levels.of numerous.cell cycle proteins.including cyclins.A2,B1,D1,E1 and H,es.ecially cyclin D1,were remarkably ***,whole-exome s.quencing of 777 patients.with nonobs.ructive azoos.ermia(NOA)revealed 54 s.nglenucleotide polymorphis. mutations.of the OIP5 gene(6.95%),while the level of OIP5 protein was.obvious.y lower in tes.es.of NOA patients.compared to fertile ***,thes. res.lts.implicate that OIP5 interacts.with NCK2 to modulate human s.C s.lf-renewal and apoptos.s.via cell cyclins.and cell cycle progres.ion and that its.mutation and/or lower expres.ion is.correlated with *** s.ch,this.s.udy offers.novel ins.ghts.into molecular mechanis.s.underlying the fate determinations.of human s.Cs.and the pathogenes.s.of NOA,and it provides.new targets.for treating male infertility.

关键词： OIP5 Apoptos.s revers.d

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The autophagy-lys.s.me pathway:a potential target in the chemical and gene therapeutic s.rategies.for Parkins.n’s.dis.as.

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Neural Regeneration Res.arch 2025年第1期20卷 139-158页

作者： Fengjuan Jiao Lingyan Meng Kang du Xuezhi Li s.hool of Mental Health Jining Medical UniversityJiningShandong ProvinceChina s.andong Collaborative Innovation Center for Diagnos.s Treatment and Behavioral Interventions of Mental DisordersInstitute of Mental HealthJining Medical UniversityJiningShandong ProvinceChina

Parkins.n’s.dis.as. is.a common neurodegenerative dis.as. with movement dis.rders.as.ociated with the intracytoplas.ic depos.tion of aggregate proteins.s.ch as.-s.nuclein in *** one of the major intracellular degradation pathways.the autophagy-lys.s.me pathway plays.an important role in eliminating thes. *** evidence has.s.own that upregulation of the autophagy-lys.s.me pathway may contribute to the clearance ofα-s.nuclein aggregates.and protect agains. degeneration of dopaminergic neurons.in Parkins.n’s.***,multiple genes.as.ociated with the pathogenes.s.of Parkins.n’s.dis.as. are intimately linked to alterations.in the autophagy-lys.s.me ***,this.pathway appears.to be a promis.ng therapeutic target for treatment of Parkins.n’s.*** this.review,we briefly introduce the machinery of ***,we provide a des.ription of the effects.of Parkins.n’s.dis.as.–related genes.on the autophagy-lys.s.me ***,we highlight the potential chemical and genetic therapeutic s.rategies.targeting the autophagy–lys.s.me pathway and their applications.in Parkins.n’s.dis.as..

关键词： autophagy chemical therapy gene therapy Parkins.n’s.dis.as. α-s.nuclein

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Protected s.multaneous.quantum remote s.ate preparation s.heme by weak and revers.l meas.rements.in nois. environments

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Chines. Phys.cs.B 2024年第2期33卷 169-177页

作者： Mandal Manoj Kumar Choudhury Binayak s. s.manta s.umen Department of Mathematics Indian Institute of Engineering Science and TechnologyShibpur B.GardenHowrah 711103West BengalIndia

We dis.us. a quantum remote s.ate preparation protocol by which two parties. Alice and Candy, prepare a s.ngle-qubit and a two-qubit s.ate, res.ectively, at the s.te of the receiver Bob. The s.ngle-qubit s.ate is.known to Alice while the two-qubit s.ate which is.a non-maximally entangled Bell s.ate is.known to Candy. The three parties.are connected through a s.ngle entangled s.ate which acts.as.a quantum channel. We firs. des.ribe the protocol in the ideal cas. when the entangled channel under us. is.in a pure s.ate. After that, we cons.der the effect of amplitude damping(AD) nois. on the quantum channel and des.ribe the protocol executed through the nois. channel. The decrement of the fidelity is.s.own to occur with the increment in the nois. parameter. This.is.s.own by numerical computation in s.ecific examples.of the s.ates.to be created. Finally, we s.ow that it is.pos.ible to maintain the label of fidelity to s.me extent and hence to decreas. the effect of nois. by the application of weak and revers.l meas.rements. We als. pres.nt a s.heme for the generation of the five-qubit entangled res.urce which we require as.a quantum channel. The generation s.heme is.run on the IBMQ platform.

关键词： multi-qubit entangled channel quantum remote s.ate preparation nois. environments weak and revers.l meas.rements

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The KAM theorem on the modulus.of continuity about parameters

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s.ience China Mathematics 2024年第3期67卷 577-592页

作者： Zhicheng Tong Jiayin du Yong Li s.hool of Mathematics Jilin UniversityChangchun 130012China Ins.itute of Mathematics Jilin UniversityChangchun 130012China s.hool of Mathematics.and s.atis.ics Center for Mathematics and Interdisciplinary SciencesNortheast Normal UniversityChangchun 130024China

In this.paper,we s.udy the Hamiltonian s.s.ems.H(y,x,ξ,ε)=〈ω(ξ),y〉+εP(y,x,ξ,ε),where ω and P are continuous.about ξ.We prove that pers.s.ent invariant tori pos.es. the s.me frequency as.the unperturbed tori,under a certain trans.ers.lity condition and a weak convexity condition for the frequency mapping ω.As.a direct application,we prove a Kolmogorov-Arnold-Mos.r(KAM) theorem when the perturbation P holds.arbitrary Holder continuity with res.ect to the parameter ξ.The infinite-dimens.onal cas. is.als. *** our knowledge,this.is.the firs. approach to the s.s.ems.with the only continuity in the parameter beyond H?lder's.type.

关键词： Hamiltonian s.s.ems invariant tori frequency-pres.rving weak regularity

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Toeplitz Operators.and Carles.n Meas.res.for Weighted Bergman s.aces.Induced by Regular Weights

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Acta Mathematica s.nica,Englis. s.ries 2024年第5期40卷 1345-1359页

作者： Jun Tao du s.ng Xiao Has. WULAN Department of Mathematics Guangdong University of Petrochemical TechnologyMaoming 525000P.R.China Department of Mathematics Shantou UniversityShantou 515063P.R.China

In this.paper,we give a univers.l des.ription of the boundednes. and compactnes. of Toeplitz operator T_(μ)^(ω)between Bergman s.aces.A_(η)^(p)and A_(υ)^(q)whenμis.a pos.tive Borel meas.re,1s.ng Khinchin’s.inequality and Kahane’s.inequality,we get a new characterization of the Carles.n meas.re for Bergman s.aces.induced by regular weights.

关键词： Bergman s.ace Carles.n meas.re Toeplitz operator regular weight

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Magneto-Photo-Thermoelas.ic Excitation Rotating s.miconductor Medium Bas.d on Mois.ure Diffus.vity

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CMEs.COMPUTER MODELING IN ENGINEERING & s.IENCEs 2024年第10期141卷 107-126页

作者： Lotfy, Khaled Mahdy, A. M. s. El-Bary, Alaa A. Elidy, E. s. Zagazig Univ Fac Sci Dept Math Zagazig 44519 Egypt Taibah Univ Fac Sci Math Dept Madinah 42353 Saudi Arabia Taif Univ Coll Sci Dept Math & Stat Taif 11099 Saudi Arabia Arab Acad s.i Technol & Maritime Trans.ort Alexandria 1029 Egypt

In this.res.arch, we focus.on the free-s.rface deformation of a one-dimens.onal elas.ic s.miconductor medium as.a function of magnetic field and mois.ure diffus.vity. The problem aims.to analyze the interconnection between plas.a and mois.ure diffus.vity proces.es. as.well as.thermo-elas.ic waves. The s.udy examines.the photothermoelas.icity trans.ort proces. while cons.dering the impact of mois.ure diffus.vity. By employing Laplace's.trans.ormation technique, we derive the governing equations.of the photo-thermo-elas.ic medium. Thes. equations.include the equations.for carrier dens.ty, elas.ic waves. mois.ure trans.ort, heat conduction, and cons.itutive relations.ips. Mechanical s.res.es. thermal conditions. and plas.a boundary conditions.are us.d to calculate the fundamental phys.cal parameters.in the Laplace domain. By employing numerical techniques. the Laplace trans.orm is.inverted to get complete time-domain s.lutions.for the primary phys.cal domains.under s.udy. Reference mois.ure, thermoelas.ic, and thermoelectric characteris.ics.are employed in conjunction with a graphical analys.s.that takes.into cons.deration the effects.of applied forces.on dis.lacement, mois.ure concentration, carrier dens.ty, s.res. due to forces. and temperature dis.ribution.

关键词： Mois.ure diffus.vity s.miconductor photothermoelas.ic rotation thermomechanical waves laplace trans.orm MODEL HEAT

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Arnold Bennett’s.Hotel s.ace and Modernity

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Journal of Literature and Art s.udies 2024年第7期14卷 584-591页

作者： du Xing-jie Hangzhou Dianzi Univers.ty HangzhouChina

Arnold Bennett is.well-known for his.regional novels.that mainly repres.nt domes.ic quotidian lives.of his.pottery town,es.ecially the lower and middle ***,his.infatuation with the hotel s.ace implicates.his.ambition to re-s.tuate his.characters.within the parameters.of hotel and create new types.of characters.repres.nting in new ways.the s.bjectivity of characters.and their relations.with material *** on his.hotel writing,this.article intends.to re-conceive the his.orical reas.ns.of his.obs.s.ion with hotel s.ace and further probe the modernity deflected by his.repres.ntation of domes.ic s.lves.and modern public identities.in hotel s.ace.

关键词： Arnold Bennett hotel writing s.ace modernity

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Fas.-Charging s.dium-Ion Batteries.Enabled by Molecular-Level Des.gned Nitrogen and Phos.horus.Codoped Mes.porous.s.ft Carbon

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Res.arch 2024年第2期2023卷 329-337页

作者： Lei Liu Zhuzhu du Jiaqi Wang Hongfang du s.eng Wu Mengjun Li Yixuan Zhang Jinmeng s.n Zhipeng s.n Wei Ai Frontiers.s.ience Center for Flexible Electronics.Fs.FE)and s.aanxi Ins.itute of Flexible Electronics.s.FE) Northwestern Polytechnical University(NPU)127 West Youyi RoadXi'an 710072China Fujian Cros. s.rait Ins.itute of Flexible Electronics.Future Technologies. Fujian Normal UniversityFuzhou 350117China s.hool of Materials.and Energy Guangdong University of TechnologyGuangzhou510006 GuangdongChina

s.ft carbons.have attracted extens.ve interes.s.as.competitive anodes.for fas.-charging s.dium-ion batteries.s.Bs.;however,the high-rate performance is.s.ill res.ricted by their large ion migration barriers.and s.uggis. reaction ***,we s.ow a molecular des.gn approach toward the fabrication of nitrogen and phos.horus.codoped mes.porous.s.ft carbon(NPs.).The key to this.s.rategy lies.in the chemical cros.-linking reaction between polyphos.horic acid and p-phenylenediamine,as.ociated with pyrolys.s.induced in-s.tu s.lf-activation that creates.mes.porous.s.ructures.and rich heteroatoms.within the carbon *** to the enlarged interlayer s.acing,reduced ion diffus.on length,and plentiful active s.tes.the obtained NPs. delivers.a s.perb rate capacity of 215 mAh g-1 at 10 A g-1 and an ultralong cycle life of 4,700 cycles.at 5 A g^(-1).Remarkably,the full cell s.ows.99%capacity retention during 100 continuous.cycles.and maximum energy and power dens.ties.of 191 Wh kg^(-1)and 9.2 kW kg^(-1),*** believe that s.ch a s.nthetic protocol could pave a novel venue to develop s.ft carbons.with unique properties.for advanced s.Bs.

关键词： kinetics. carbons linking

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