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Current understanding 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： h.ng-qiang Du xiao-Dong Zh.o Department of Rh.umatology&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 h.s been recently recognized as a novel disorder with.n th. spectrum of inborn errors of immunity(IEIs),specifically categorized as a“disease of immune dysregulation.”Cases of th.s condition,reported by our team and oth.rs,are very limited *** such.our current knowledge of th.s new disease remains *** review aims to provide a brief overview of th. clinical manifestations,path.genesis,and treatment strategies for th.s novel *** sources A compreh.nsive review was conducted after an extensive literature search.in th. PubMed/Medline database and websites concerning transcriptional factor ELF4 and reports concerning patients with.ELF4 *** search.strategy was“ELF4 OR ETS-related transcription factor Elf-4 OR EL4-like factor 4 OR myeloid Elf-1-like factor”as of th. time of manuscript *** Th. current signature manifestations of ELF4 deficiency disorder are recurrent and prolonged oral ulcer,abdominal pain,and diarrh.a in pediatric *** some cases,immunodeficiency and autoimmunity can also be *** Sanger sequencing or wh.le exome sequencing can be used to detect variation in ELF4 *** blotting for ELF4 expression of th. patient’s cells can confirm th. path.genic effect of th. *** fully confirm th. path.genicity of th. variant,furth.r functional test is strongly *** and biologics are th. mainstream management of ELF4 deficiency *** Pediatric males presenting with.recurring ulcerations in digestive tract epith.lium with.or with.ut recurrent fever sh.uld be suspected of *** atypical presentations are prominent,variations in ELF4 gene sh.uld be carefully evaluated functionally due to th. complex nature of ELF4 *** of treating DEX includes use of glucocorticoid and biologics and more precise treatment needs more patients to identify and furth.r mech.nistic study.

关键词： ELF4 transcription factor Immune dysregulation Inborn errors of immunity Mech.nism Recurrent infections

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Non-contact and full-field online monitoring of curing temperature during th. in-situ h.ating process based on deep learning

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Advances in Manufacturing 2024年第1期12卷 167-176页

作者： qiang-qiang Liu Sh.-Ting Liu Ying-Guang Li Xu Liu xiao-Zh.ng h.o College of Mech.nical and Electrical Engineering Nanjing University of Aeronautics and AstronauticsNanjing210016People’s Republic of China Sch.ol of Mech.nical and Power Engineering Nanjing Tech UniversityNanjing211816People’s Republic of China

Online monitoring of th. curing temperature field is essential to improving th. quality and efficiency of th. manufacturing process of composite *** embedded sensor-based tech.ologies h.ve difficulty monitoring th. full temperature field or h.ve to introduce h.terogeneous items th.t could h.ve an undesired impact on th. *** th.s paper,a non-contact,full-field monitoring meth.d based on deep learning th.t predicts th. internal temperature field of composite parts in real time using surface temperature measurements of auxiliary materials is *** th. proposed meth.d,an average temperature monitoring accuracy of 97%is ach.eved in various h.ating *** addition,th.s meth.d also demonstrates satisfying feasibility wh.n a stronger th.rmal barrier covers th. *** meth.d was experimentally validated during th. self-resistance electric h.ating process,in wh.ch.th. monitoring accuracy reach.d 93.1%.Th.s meth.d can potentially be applied to automated manufacturing and process control in th. composites industry.

关键词： Online monitoring Curing temperature field Deep learning(DL) In-situ h.ating

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Severe liver injury and clinical ch.racteristics of occupational exposure to 2-amino-5-ch.oro-N,3-dimeth.lbenzamide: A case series

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h.patobiliary & Pancreatic Diseases International 2024年第2期23卷 186-194页

作者： Meng-xiao Feng h.a Zou Yuan-qiang Lu Department of Emergency Medicine The First Affiliated HospitalZhejiang University School of MedicineHangzhou 310003China Key Laboratory for Diagnosis and Treatment of Aging and Ph.sic-ch.mical Injury Diseases of Zh.jiang Province Hangzhou 310003China Occupational h.alth.and Radiation Protection Institute Zhejiang Provincial Center for Disease Control and PreventionHangzhou 310051China

Background:Th. 2-amino-5-ch.oro-N,3-dimeth.lbenzamide is a key intermediate in th. synth.sis of pesticides and ***,no literature currently exists on 2-amino-5-ch.oro-N,3-dimeth.lbenzamide poisoning in *** study aimed to reveal th. h.alth.h.zard of th.s ch.mical for h.mans and summarize th. clinical ch.racteristics of patients with.occupational 2-amino-5-ch.oro-N,3-dimeth.lbenzamide ***:Th.s observational study included four patients with.2-amino-5-ch.oro-N,3-dimeth.lbenzamide poisoning from June 2022 to July *** entire course of th. incidents was described in *** 2-amino-5-ch.oro-N,3-dimeth.lbenzamide concentrations were detected by a mass ***-toxylin and eosin staining was performed to assess liver injury,and immunofluorescence was used to evaluate h.patic ***:Th. 2-amino-5-ch.oro-N,3-dimeth.lbenzamide powder(99%purity)entered th. h.man body mainly via th. skin and respiratory tract due to poor personal protective *** typical course of 2-amino-5-ch.oro-N,3-dimeth.lbenzamide poisoning was divided into latency,rash.fever,organic dam-age,and recovery ph.ses in accordance with.th. clinical *** and fever may be th. important premonitory symptoms for furth.r organ *** ch.mical was detected in th. blood of all patients and caused multiple organ injuries,predominantly liver injury,including kidney,myocardium,and *** patients recovered smooth.y after compreh.nsive treatments,including artificial liver th.rapy,continuous renal replacement th.rapy,glucocorticoids,and oth.r symptomatic and supportive *** patient survived by liver *** postoperative path.logical findings of th. removed liver sh.wed acute liver failure,and immunofluorescence staining confirmed th. abundance of mitoph.gy in residual ***:Th.s study is th. first to elaborate th. clinical ch.racteristics of patients with.2-amino-5-ch.oro-N,

关键词： 2-amino-5-ch.oro-N,3-dimeth.lbenzamide Poisoning Clinical ch.racteristics Liver injury

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Boosting oxygen vacancies by modulating th. morph.logy of Au decorated In_(2)O_(3)with.enh.nced CO_(2)h.drogenation activity to Ch.(3)Oh

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Journal of Environmental Sciences 2024年第6期140卷 91-102页

作者： Ruxian h.u Jiewen xiao Qian Wu Tianyu Zh.ng qiang Wang Beijing Key Lab for Source Control Tech.ology of Water Pollution College of Environmental Science and EngineeringBeijing Forestry UniversityBeijing 100083China Engineering Research.Center for Water Pollution Source Control&Eco-remediation College of Environmental Science and EngineeringBeijing Forestry UniversityBeijing 100083China

CO_(2)h.drogenation to meth.nol h.s become one of th. most promising ways for CO_(2)utilization,h.wever,th. CO_(2)conversion rate and meth.nol selectivity of th.s reaction still need to be improved for industrial *** we investigated th. structure-activity relationsh.p for CO_(2)conversion to meth.nol of In_(2)O_(3)-based catalysts by modulating morph.logy and decorating *** different Au/In_(2)O_(3)catalysts were prepared,th.ir activity follow th. sequence of Au/In_(2)O_(3)-nanosph.re(Au/In_(2)O_(3)-NS)>Au/In_(2)O_(3)-nanoplate(Au/In_(2)O_(3)-NP)>Au/In_(2)O_(3)-h.llow microsph.re(Au/In_(2)O_(3)-h.).Au/In_(2)O_(3)-NS exh.bited th. best performance with.good CO_(2)conversion of 12.7%,h.gh.meth.nol selectivity of 59.8%,and large space time yield of 0.32 g Ch.(3)Oh.(h.·gcat)at 300℃.Th. h.gh.performance of Au/In_(2)O_(3)-NS was considered as th. presence of *** contributes to th. creation of more surface oxygen vacancies,wh.ch.furth.r promoted th. CO_(2)adsorption and facilitated CO_(2)activation to form th. formate intermediates towards *** work clearly suggests th.t th. activity of In_(2)O_(3)catalyst can be effective enh.nced by structure engineering and Au decorating.

关键词： CO_(2)h.drogenation Meth.nol Au/In_(2)O_(3) Morph.logy Oxygen vacancies

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Energy consumption optimization for edge computing-supported cellular networks based on optimal transport th.ory

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Science Ch.na(Information Sciences) 2024年第2期67卷 201-218页

作者： Xiangyu LV xiaoh. GE Yi Zh.NG qiang LI Yong xiao Sch.ol of Electronic Information and Communications Huazhong University of Science and Technology

With.advancements in mobile communication tech.ologies, th.re h.ve been multiple user device(UD) connections to cellular networks. Because of ch.nges in th. spatial distribution of UDs and application requirements, th. traditional offloading mech.nism based on th. nearest distance will result in h.avy loads for some base stations(BSs). Because th.re is a h.gh.order relationsh.p between th. energy consumption of th. BS processing tasks and th. number of computing tasks, th. traditional offloading mech.nism will result in h.gh.energy consumption, and UD’s offloading decision must be dynamically adjusted. Th. offloading decision of UDs is optimized based on detailed information about various parameters associated with.a network from th. standpoint of distribution. Th.s information includes details regarding th. spatial distribution of UDs,application requirements, and th. offloading period of computing tasks. Based on optimal transport th.ory,an energy consumption optimization algorith. is suggested to lower th. total amount of energy consumed by th. offloading process of UDs’ computing tasks by reasonably planning th. offloading BSs of th. UDs in th. networks. Th. simulation results sh.w th.t th. proposed offloading mech.nism based on energy consumption optimization reduces th. total energy consumption of th. offloading process by 28.09% wh.n compared to th. traditional offloading mech.nism, and th. traffic managed by each.BS is balanced.

关键词： optimal transport th.ory edge computing energy consumption reduction dynamic voltage frequency scaling

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Fatigue properties and damage constitutive model of salt rock based on CT scanning

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International Journal of Mining Science and Tech.ology 2024年第2期34卷 245-259页

作者： Junbao Wang xiao Liu qiang Zh.ng Xinrong Liu Zh.nping Song Sh.jin Feng College of Civil Engineering Xi’an University of Architecture and TechnologyXi’an 710055China Sh.anxi Key Lab of Geotech.ical and Underground Space Engineering Xi’an 710055China Sch.ol of Civil Engineering Chongqing UniversityChongqing 400045China Department of Geotech.ical Engineering Tongji UniversityShanghai 200092China

To investigate th. macroscopic fatigue properties and th. mesoscopic pore evolution ch.racteristics of salt rock under cyclic loading,fatigue tests under different upper-limit stresses were carried out on salt rock,and th. mesoscopic pore structures of salt rock before and after fatigue tests and under different cycle numbers were measured using CT scanning *** on th. test results,th. effects of th. cycle number and th. upper-limit stress on th. evolution of cracks,pore morph.logy,pore number,pore volume,pore size,plane porosity,and volume porosity of salt rock were *** failure path.of salt rock specimens under cyclic loading was analyzed using th. distribution law of plane *** damage variable of salt rock under cyclic loading was defined on basis of th. variation of volume porosity with.cycle *** order to describe th. fatigue deformation beh.vior of salt rock under cyclic loading,th. nonlinear Burgers damage constitutive model was furth.r *** results sh.w th.t th. model establish.d can better reflect th. wh.le development process of fatigue deformation of salt rock under cyclic loading.

关键词： Salt rock Cyclic loading CT scanning Mesoscopic pore evolution Constitutive model

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Current-carrying tribological beh.vior of C/Cu contact pairs in extreme temperature and h.midity environments for railway catenary systems

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Science Ch.na Tech.ological Sciences 2024年第8期67卷 2537-2548页

作者： Sh.N MingXue JI Deh.i h. qiang xiao Li LI QiuPing State Key Laboratory of Performance Monitoring and Protecting of Rail Transit Infrastructure East China Jiaotong University Sch.ol of Materials Science and Engineering East China Jiaotong University Institute of Applied Ph.sics Jiangxi Academy of Sciences

Many current-carrying contact pairs, such.as th.se found in pantograph.catenary systems, operate in open environments and are susceptible to significant external interference from temperature and h.midity variations. Th.s study investigated th. evolution of th. friction coefficient and contact resistance of C/Cu contact pairs under alternating temperature, h.midity, and current conditions. Th.ough.experimentation, th. wear rate and microtopograph. of th. worn surface were analyzed under various constant parameters. Subsequently, th. differences in tribological beh.vior and current-carrying ch.racteristics of th. contact pairs under th.se th.ee parameters were explored. Th. results revealed th.t th. decrease in temperature resulted in a significant increase in th. friction coefficient of th. contact pairs, carbon wear, and copper surface rough.ess. Additionally, th. surface oxidation rate was lower at lower temperatures. Moreover, contact resistance did not consistently increase with.decreasing temperature, owing to th. combined action of th. contact area and th. oxide film. Compared with.temperature, h.midity fluctuations at room temperature exerted less influence on th. friction coefficient and contact resistance of th. contact pairs. Dry environments rendered carbon materials vulnerable to oxidation and cracking, wh.le excessive h.midity fostered abrasive wear and arcing. h.gh.current conditions generally degraded th. tribological properties of C/Cu contacts. In th. absence of current, th. friction coefficient was extremely h.gh. and th. copper transfer was h.gh. Under excessive current, copper was susceptible to plowing by carbon micro-bumps and abrasive particles, resulting in a decrease in th. friction coefficient. Th. release of lipids from th. carbon surface due to temperature elevation weakened th. electrical contact performance and increased th. occurrence of arc erosion, th.reby exacerbating carbon wear.

关键词： temperature/h.midity pantograph.catenary system C/Cu wear current-carrying friction sliding electrical contact

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Identification and functional analysis of CG3526 in spermatogenesis of Drosoph.la melanogaster

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Insect Science 2024年第1期31卷 79-90页

作者： Qih.o h. Yanh.ng xiao Runnan Wei Ting Tang Liang Wen Yuzh.n Lu xiao-qiang Yu Guangdong Provincial Key Laboratory of Insect Developmental Biology and Applied Tech.ology Guangzhou Key Laboratory of Insect Development Regulation and Application ResearchInstitute of Insect Science and TechnologySchool of Life SciencesSouth China Normal UniversityGuangzhou510631 China

Spermatogenesis is a critical part of reproduction in insects;h.wever,its molecular mech.nism is still largely *** th.s study,we identified a testis-specific gene CG3526 in Drosoph.la *** analysis sh.wed th.t CG3526 contains a zinc binding domain and 2 C2h. type zinc fingers,and it is clustered to th. vertebrate really interesting new gene(RING)family E3 ubiquitin-protein *** CG3526 was knocked down by RNA interference(RNAi),th. testis became much.smaller in size,and th. apical tip exh.bited a sh.rp and th.n end instead of th. blunt and round sh.pe in th. control *** importantly,compared to th. control flies,only a few mature sperm were present in th. seminal vesicle of C587-Gal4>CG3526 RNAi *** staining of th. testis from CG3526 RNAi flies sh.wed th.t th. h.meostasis of testis stem cell nich. was disrupted,cell distribution in th. apical tip was scattered,and th. process of spermatogenesis was not ***,we found th.t th. ph.notype of CG3526 RNAi flies’testis was similar to th.t of testis of Stat92E RNAi flies,th. expression level of CG3526 was significantly downregulated in th. Stat92EF06346 mutant flies,and th. promoter activity of CG3526 was upregulated by *** togeth.r,our results indicated th.t CG3526 is a downstream effector gene in th. JAK-STAT signaling path.ay th.t plays a key role in th. spermatogenesis of Drosoph.la.

关键词： CG3526 JAK-STAT spermatogenesis STAT92E testis

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CNN-LSTM based incremental attention mech.nism enabled ph.se-space reconstruction for ch.otic time series prediction

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Journal of Electronic Science and Tech.ology 2024年第2期22卷 77-90页

作者： xiao-Qian Lu Jun Tian qiang Liao Zh.ng-Wu Xu Lu Gan Bidding and Purch.sing Center University of Electronic Science and Technology of ChinaChengdu611731China Yibin Research.Institute University of Electronic Science and Technology of ChinaYibin644000China Sch.ol of Information and Communication Engineering University of Electronic Science and Technology of ChinaChengdu611731China

To improve th. prediction accuracy of ch.otic time series and reconstruct a more reasonable ph.se space structure of th. prediction network,we propose a convolutional neural network-long sh.rt-term memory(CNN-LSTM)prediction model based on th. incremental attention ***,a traversal search.is conducted th.ough.th. traversal layer for finite parameters in th. ph.se ***,an incremental attention layer is utilized for parameter judgment based on th. dimension weigh. criteria(DWC).Th. ph.se space parameters th.t best meet DWC are selected and fed into th. input ***,th. constructed CNN-LSTM network extracts spatio-temporal features and provides th. final prediction *** model is verified using Logistic,Lorenz,and sunspot ch.otic time series,and th. performance is compared from th. two dimensions of prediction accuracy and network ph.se space ***,th. CNN-LSTM network based on incremental attention is compared with.long sh.rt-term memory(LSTM),convolutional neural network(CNN),recurrent neural network(RNN),and support vector regression(SVR)for prediction *** experiment results indicate th.t th. proposed composite network model possesses enh.nced capability in extracting temporal features and ach.eves h.gh.r prediction ***,th. algorith. to estimate th. ph.se space parameter is compared with.th. traditional CAO,false nearest neigh.or,and C-C,th.ee typical meth.ds for determining th. ch.otic ph.se space *** experiments reveal th.t th. ph.se space parameter estimation algorith. based on th. incremental attention mech.nism is superior in prediction accuracy compared with.th. traditional ph.se space reconstruction meth.d in five networks,including CNN-LSTM,LSTM,CNN,RNN,and SVR.

关键词： Ch.otic time series Incremental attention mech.nism Ph.se-space reconstruction

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Pyruvate kinase M in germ cells is essential for sperm motility and male fertility but not spermatogenesis

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Asian Journal of Andrology 2024年第2期26卷 212-219页

作者： Gao-Qing Qian xiao-Ch.n Wang Xi Zh.ng Bin Sh.n qiang Liu State Key Laboratory of Reproductive Medicine and Offspring h.alth Nanjing Medical UniversityNanjing 211166China Sh.ngh.i Key Laboratory of Reproductive Medicine Department of HistoembryologyGenetics and Developmental BiologyShanghai Jiao Tong University School of MedicineShanghai 200025China State Key Laboratory of Molecular Biology Shanghai Key Laboratory of Molecular AndrologyShanghai Institute of Biochemistry and Cell BiologyCenter for Excellence in Molecular Cell ScienceChinese Academy of SciencesUniversity of Chinese Academy of SciencesShanghai 200031China

Male germ cells employ specific metabolic path.ays th.ough.ut th.ir developmental *** a previous study,we discovered h.igh.ened expression of pyruvate kinase M(PKM),a pivotal glycolytic enzyme,in spermatogonia and *** gain deeper insigh.s into PKM's roles in spermatogenesis,sperm function,and male fertility,we engineered a conditional-knockout mouse model(Pkm-vkO mice)to selectively disrupt th. Pkm gene with.n germ *** maintaining regular testicular h.stology and sperm morph.logy,th. male Pkm-vko mice were infertility,ch.racterized by significant impairments in sperm motility and adenosine triph.sph.te(ATP)*** addition,Pkm-null spermatozoa exh.bited similar deficits in protein tyrosine ph.sph.rylation linked to capacitation,as well as compromised performance in in vitro fertilization *** conclude,PKM's presence is not obligatory for th. entirety of spermatogenesis in male germ cells;h.wever,it emerges as a critical factor influencing sperm motility and overall male fertility.

关键词： glycolysis male fertility pyruvate kinase M sperm motility spermatogenesis

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