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Panoramic variation analysis of a family with neurodevelopmental disorders caused by biallelic loss-of-function variants in TMEM141,DDHD2,and LHFPL5

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Frontiers of Medicine 2024年第1期18卷 81-97页

作者： Liwei Sun Xueting Yang Amjad Khan Xue Yu han Zhang Shirui han Xiaerbati Habulieti Yang Sun Rongrong Wang Xue Zhang McKusick-Zhang center for Genetic Medicine State Key Laboratory for Complex Severe and Rare DiseasesInstitute of Basic Medical Sciences Chinese Academy of Medical SciencesSchool of Basic Medicine Peking Union Medical CollegeBeijing100005China chongqing Key Laboratory of Human Embryo Engineering Center for Reproductive MedicineNational Key Clinical Speciality Construction Project(Obstetrics and Gynecology)Chongqing Health Center for Women and ChildrenChongqing400013China chongqing clinical Research center for Reproductive Medicine Women and Children’s Hospital of Chongqing Medical UniversityChongqing400013China Faculty of Biological Sciences Department of ZoologyUniversity of Lakki MarwatKhyber Pakhtunkhwa28420Pakistan Institute for Medical Genetics and Applied Genomics University of TübingenTübingen72076Germany Alexander von Humboldt fellowship Foundation Berlin10117Germany Department of Pediatrics The First Affiliated Hospital of Guangxi Medical UniversityNanning530000China Department of Laboratory Medicine State Key Laboratory for Complex Severe and Rare DiseasesPeking Union Medical College HospitalChinese Academy of Medical Science and Peking Union Medical CollegeBeijing100730China

Highly clinical and genetic heterogeneity of neurodevelopmental disorders presents a major challenge in clinical genetics and *** variation analysis is imperative to analyze the disease phenotypes resulting from multilocus genomic ***,a Pakistani family with parental consanguinity was presented,characterized with severe intellectual disability(ID),spastic paraplegia,and *** mapping,integrated single nucleotide polymorphism(SNP)array,whole-exome sequencing,and whole-genome sequencing were performed,and homozygous variants in TMEM141(c.270G>A,***90^(*)),DDHD2(c.411+767_c.1249-327del),and LHFPL5(c.250delc,***84^(*))were identified.A Tmem141^(***90^(*)/***90^(*))mouse model was *** studies showed impairments in learning ability and motor *** slice electrophysiology and Golgi staining demonstrated deficient synaptic plasticity in hippocampal neurons and abnormal dendritic branching in cerebellar Purkinje *** electron microscopy showed abnormal mitochondrial ***,studies on a human in vitro neuronal model(SH-SY5Y cells)with stable shRNA-mediated knockdown of TMEM141 showed deleterious effect on bioenergetic function,possibly explaining the pathogenesis of replicated phenotypes in the cross-species mouse ***,panoramic variation analysis revealed that multilocus genomic variations of TMEM141,DDHD2,and LHFPL5 together caused variable phenotypes in ***,the biallelic loss-of-function variants of TMEM141 were responsible for syndromic ID.

关键词： neurodevelopmental disorder autosomal recessive intellectual disability consanguinity spastic paraplegia hearing loss TMEM141

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Electrochemical cO_(2)reduction to c_(2+)products over cu/Zn intermetallic catalysts synthesized by electrodeposition

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Frontiers in Energy 2024年第1期18卷 80-88页

作者： Ting DENG Shuaiqiang JIA Shitao han Jianxin ZHAI Jiapeng JIAO Xiao cHEN cheng XUE Xueqing XING Wei XIA Haihong WU Mingyuan HE Buxing han School of chemistry and Molecular Engineering East China Normal UniversityShanghai 200062China Institute of Eco-chongming Shanghai 202162China Institute of High Energy Physics Chinese Academy of SciencesBeijing 100049China Institute of chemistry Chinese Academy of SciencesBeijing 100190China

Electrocatalytic cO_(2)reduction(EcR)offers an attractive approach to realizing carbon neutrality and producing valuable chemicals and fuels using cO_(2)as the ***,the lack of cost-effective electrocatalysts with better performances has seriously hindered its ***,a one-step co-electrodeposition method was used to introduce Zn,a metal with weak^(*)cO binding energy,into cu to form cu/Zn intermetallic catalysts(cu/Zn IMcs).It was shown that,using an H-cell,the high Faradaic efficiency of c_(2+)hydrocarbons/alcohols()could be achieved in EcR by adjusting the surface metal components and the applied *** suitable conditions,FEc_(2+)and current density could be as high as 75%and 40 mA/cm^(2),*** with the cu catalyst,the cu/Zn IMcs have a lower interfacial charge transfer resistance and a larger electrochemically active surface area(EcSA),which accelerate the ***,the^(*)cO formed on Zn sites can move to cu sites due to its weak binding with*cO,and thus enhance the c–c coupling on the cu surface to form c_(2+)products.

关键词： carbon dioxide electroreduction electrochemistry co-electrodeposition intermetallic catalysts value-added chemicals

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Using Natural Radionuclides to Trace Sources of Suspended Particles in the Lower Reaches of the Yellow River

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Journal of Oc.an University of china 2024年第1期23卷 99-108页

作者： ZhanG han BURNETT cWilliam ZhanG Xiaojie ZHAO Shibin YANG Disong NAN Haiming YU Zhigang XU Bochao Frontiers Science center for Deep Oc.an Multispheres and Earth System and Key Laboratory of Marine Chemistry Theory and TechnologyMinistry of EducationOcean University of ChinaQingdao 266100China Laboratory for Marine Ecology and Environmental Science Qingdao National Laboratory for Marine Science and TechnologyQingdao 266071China college of chemistry and chemical Engineering Ocean University of ChinaQingdao 266100China Department of Earth Ocean and Atmospheric ScienceFlorida State UniversityTallahasseeFL 32306USA North china Sea Environmental Monitoring center State Oceanic AdministrationKey Laboratory of Ecological Prewarning and Protection of Bohai SeaMNRQingdao 266033China Shandong Provincial Key Laboratory of Marine Ecological Environment and Disaster Prevention and Mitigation Qingdao 266033China

Natural radionuclides are powerful tools for understanding the sources and fate of suspended particulate matter(SPM).Particulate matter with different particle sizes behaves differently with respect to adsorption and *** analyzed the activi-ties and distribution characteristics of multiple natural radionuclides(238U,226Ra,40K,228Ra,7Be and 210Pbex)on size-fractionated SPM at the Lijin Hydrographic Station(Huanghe or Yellow River)every month over a one-year *** showed that medium silt(16–32µm)was the main *** expected,the activity of each radionuclide decreased with an increase of particle *** examined the sources of SPM with different particle sizes using activity ratios of 226Ra/238U,228Ra/226Ra,40K/238U and 7Be/210Pbex,and concluded that SPM with different particle sizes originated from different *** results indicate that fine SPM(ches of the Yellow River,while coarse SPM(>32µm)was mainly derived from resuspension of riverbed *** high runoff periods,the concentration of SPM increased significantly,and the pro-portion of fine particles originating upstream *** occurring radioactive isotopes,especially on size-fractionated par-ticles,are therefore seen as useful tracers to understand the sources and behaviors of riverine particles transported from land to sea.

关键词： the Yellow River suspended particulate matter size-fractions radionuclides sediment sources water elutriator

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cu_(2)Se@c thin film with three-dimensional braided structure as a cathode material for enhanced cu^(2+) storage

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chinese chemical Letters 2024年第5期35卷 523-528页

作者： Wenhao chen Muxuan Wu han chen Lue Mo Yirong Zhu college of Materials and Advanced Manufacturing Hunan University of TechnologyZhuzhou 412007China Hunan Key of Laboratory of Applied Environmental Photocatalysis Changsha UniversityChangsha 410022China

In the face of multiple challenges brought by the c.anges of global climate and environment,developing clean energy and updating green energy storage equipment are important ways to achieve carbon peak and carbon *** batteries have become a research hotspot due to their advantages of using the multivalent charge carrier,high ionic conductivity,environmental friendliness and cost *** this work,the cu_(2)Se@c(cu_(2)Se coated on carbon clothes)thin film with a three-dimensional braided structure is fabricated by a simple electrochemical deposition method for cu^(2+)storage for the first *** with the commercial cu_(2)Se powder,the well-designed cu_(2)Se@c film shows enhanced specific capacity(640 mAh/g at 0.5 A/g)and rate performance(542 mAh/g at 5 A/g)as well as superior cycling stability(82.7%capacity retention after 1000 cycles at 1 A/g).The cu^(2+)storage mec.anism of the cu_(2)Se@c electrode is based on a reversible phase transition process of cu_(2)Se←→cu_(2-x)Se←→cuSe←→cuSe_(2).In kinetic characteristic.analysis,the cu_(2)Se@c electrode demonstrates faster cu^(2+)diffusion in discharge process than charge process resulting from the phase transition and the variation of interplanar *** work highlights a facile one-piece design strategy and opens a new gateway for the exploration of advanced aqueous energy storage systems.

关键词： cu_(2)Se@c cu^(2+)storage conversion mec.anism Kinetic characteristic Flexible energy storage

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Molecular characterization and Antibacterial Activity of a G-Type Lysozyme in Yellow Drum(Nibea albiflora)

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Journal of Oc.an University of china 2024年第2期23卷 477-486页

作者： XIAO Yao LI Jiacheng TIAN Qianqian WU Baolan han Fang State Key Laboratory of Mariculture Breeding Fisheries College of Jimei UniversityXiamen 361021China

Lysozyme(Ec3.***.1.17)plays an important role in the immune response;as a nonspecific immune factor,it can resist causative *** can be divided into c-type and g-type in *** a previous study,through genome-wide association analysis,the g-type lysozyme gene,which is named NaLyg in yellow drum(Nibea albiflora),was found to be a key candidate gene for disease resistance in response to Vibrio harveyi *** cDNA of NaLyg was 1025 bp,including four exons and three introns,and its open reading frame(ORF)had a full-length of 582 bp,encoding 193 amino *** was found to be conserved during evolution through bioinformatic *** NaLyg protein possessed a sugar binding domain and three catalytic sites,including Glu71,Asp84 and *** qRT-PcR results confirmed that NaLyg gene mRNA was visibly increased after *** *** NaLyg protein purified by prokaryotic expression killed some gram-negative bacterial pathogens by inducing cell wall destruction,including ***,Aeromonas hydrophila and Edwardsiella ***,the NaLyg protein killed two gram-positive bacteria,Bacillus subtilis and Staphylococcus *** together,the experimental results suggested that the NaLyg protein of *** played an important role in fighting bacterial infections.

关键词： Nibea albiflora innate immunity Vibrio harveyi g-type lysozyme antibacterial activity

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Study on the oxidation mec.anism of Al-Sic composite at elevated temperature

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International Journal of Minerals,Metallurgy and Materials 2024年第9期31卷 2077-2087页

作者： Jishuo han Yong Li chenhong Ma Qingyao Zheng Xiuhua Zhang Xiaofang Wu School of Materials Science and Engineering University of Science and Technology BeijingBeijing 100083China Vesuvius Advanced ceramics(china)co. LtdSuzhou 215000China

Resin-bonded Al-Sic composite was sintered at 1100,1300,and 1500℃ in the air,the oxidation mec.anism was *** reaction models were also *** oxidation resistance of the Al-Sic composite was significantly enhanced with temperature *** in the exterior of the composite was partially oxidized slightly,while the transformation of metastable Al_(4)c_(3) to stable Al_(4)Sic_(4) existed in the *** 1100℃,Al in the interior reacted with residual c to form Al_(4)c_(3).With increasing to 1300℃,high temperature and low oxygen partial pressure lead to active oxidation of Sic.and internal gas composition transforms to Al_(2)O(g)+cO(g)+SiO(g)as the reaction *** Al_(4)c_(3) is formed,cO(g)and SiO(g)are continuously deposited on its surface,transforming to Al_(4)Sic_(4).At 1500℃,a dense layer consisting of Sic.and Al_(4)Sic_(4) whiskers is formed which cuts off the diffusion c.annel of *** active oxidation of Sic is accelerated,enabling more gas to participate in the synthesis of Al_(4)Sic_(4),eventually forming hexagonal lamellar Al_(4)Sic_(4) with mutual accumulation between Sic *** Al enhances the oxidation resistance of *** addition,the in situ generated non-oxide is uniformly dispersed on a micro-scale and bonds Sic stably.

关键词： Al-Sic composite kiln furniture Al_(4)Sic_(4) Al_(4)c_(3) oxidation mec.anism

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Molecular Dynamics Simulation Studies on the Micromorphology and Proton Transport of Nafion/Ti_(3)c_(2)T_(x) composite Membrane

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chinese Journal of Polymer Science 2024年第3期42卷 373-387,I0009页

作者： Zhi-Yue han Su-Peng Pei chun-Yang Yu Yong-Feng Zhou School of chemical and Environmental Engineering Shanghai Institute of TechnologyShanghai 201418China School of chemistry&chemical Engineering Frontiers Science Center for Transformative MoleculesShanghai Key Laboratory of Electrical Insulation and Thermal AgingShanghai Jiao Tong UniversityShanghai 200240China Key Laboratory of Green and High-end Utilization of Salt Lake Resources Chinese Academy of SciencesXining 810008China

The perfluorosulfonic acid(PFSA) membrane doped with two-dimensional conductive filler Ti_(3)c_(2)T_(x) is a fuel cell proton exc.ange membrane with high application potential. Experimental studies showed that the proton conductivity of Nafion/Ti_(3)c_(2)T_(x) composite membrane is improved significantly compared with that in pure Nafion. However, the microscopic mec.anism of doping on the enhancement of membrane performance is remain unclear now. In this work, molecular dynamics simulation was used to investigate the microscopic morphology and proton transport behaviors of Nafion/Ti_(3)c_(2)T_(x) composite membrane at the molecular level. The results shown that there were significant differences about the diffusion kinetics of water molecules and hydroxium ions in Nafion/Ti_(3)c_(2)T_(x) at low and high hydration levels in the nanoscale *** the increase of water content, Ti_(3)c_(2)T_(x) in membrane was gradually surrounded by ambient water molecules to form a hydration layer, and forming a relatively continuous proton transport c.annel between Nafion polymer and Ti_(3)c_(2)T_(x) monomer. The continuous proton transport c.annel could increase the number of binding sites of proton and thus achieving high proton conductivity and high mobility of water molecules at higher hydration level. The current work can provide a theoretical guidance for designing new type of Nafion composite membranes.

关键词： Nafion/Ti_(3)c_(2)T_(x)composite membrane Proton transport Molecular dynamics simulation

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New insights into the pre-lithiation kinetics of single-crystalline Ni-rich cathodes for long-life Li-ion batteries

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Green Energy & Environment 2024年第3期9卷 556-564页

作者： Qiang han Lele cai Zhaofeng Yang Yanjie Hu Hao Jiang chunzhong Li Shanghai Engineering Research center of Hierarchical Nanomaterials School of Chemical EngineeringEast China University of Science and TechnologyShanghai200237China Key Laboratory for Ultrafine Materials of Ministry of Education School of Materials Science and EngineeringEast China University of Science and TechnologyShanghai200237China

Developing single-crystalline Ni-rich cathodes is an effective strategy to improve the safety and cycle life of Li-ion batteries(LIBs).However,the easy-to-loss of Li and O in high-temperature lithiation results in unsatisfactory ordered layered structure and ***,we demonstrate the synthesis of highly-ordered and fully-stoichiometric single-crystalline LiNi_(0.83)co_(0.12)Mn_(0.05)O_(2)(Sc-NcM83)cathodes by the regulation of pre-lithiation *** well-balanced pre-lithiation kinetics have been proved to greatly improve the proportion of layered phase in the intermediate by inhibiting the formation of metastable spinel phase,which promoted the rapid transformation of the intermediate into highly-ordered layered Sc-NcM83 in the subsequent lithiation *** coating a layer of Li_(2)O–B_(2)O_(3),the resultant cathodes deliver superior cycling stability with 90.9%capacity retention at 1c after 300 cycles in pouch-type full *** enhancement mec.anism has also been *** findings exhibit fundamental insights into the pre-lithiation kinetics process for guiding the synthesis of high-quality singlecrystalline Ni-rich cathodes.

关键词： Single-crystalline cathode Ni-rich oxides Pre-lithiation Li-ion batteries Surface modification

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Fixed-Time Antidisturbance consensus Tracking for Nonlinear Multiagent Systems With Matching and Mismatching Disturbances

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IEEE/cAA Journal of Automatica Sinica 2024年第6期11卷 1410-1423页

作者： Xiangmin Tan chunyan Hu Guanzhen cao Qinglai Wei Wei Li Bo han Institute of Engineering Thermophysics Chinese Academy of SciencesBeijing 100190 School of Aeronautics and Astronautics University of Chinese Academy of SciencesBeijing 100049China Institute of Automation Chinese Academy of SciencesBeijing 100190 School of Artificial Intelligence University of Chinese Academy of SciencesBeijing 100049China IEEE

In this paper,fixed-time consensus tracking for mul-tiagent systems(MASs)with dynamics in the form of strict feed-back affine nonlinearity is addressed.A fixed-time antidistur-bance consensus tracking protocol is proposed,which consists of a distributed fixed-time observer,a fixed-time disturbance observer,a nonsmooth antidisturbance backstepping controller,and the fixed-time stability analysis is conducted by using the Lyapunov theory *** paper includes three main ***,a distributed fixed-time observer is developed for each follower to obtain an estimate of the leader’s output by utilizing the topology of the communication ***,a fixed-time disturbance observer is given to estimate the lumped disturbances for feedforward ***,a nonsmooth antidisturbance backstepping tracking controller with feedforward compensation for lumped disturbances is *** order to mitigate the“explosion of complexity”in the tradi-tional backstepping approach,we have implemented a modified nonsmooth command filter to enhance the performance of the closed-loop *** simulation results show that the pro-posed method is effective.

关键词： Antidisturbance backstepping consensus tracking fixed-time stability multiagent system(MASs) strict feedback affine nonlinear systems.

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combined effects of sea urchin-like structure and mixed cu^(+)/cu^(0)states on promoting c_(2)formation in electrocatalytic cO_(2)reduction

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Frontiers of chemical Science and Engineering 2024年第3期18卷 63-73页

作者： Mengqing Shan Dongsheng Lu Jiatong Dong Shen Yan Jinyu han Hua Wang collaborative Innovation center of chemical Science and Engineering Key Laboratory for Green Chemical Technology of the Ministry of EducationSchool of Chemical Engineering and TechnologyTianjin UniversityTianjin300354China

Surface engineering and cu valence regulation are essential factors in improving the c_(2)selectivity during the electrochemical reduction of cO_(2).Herein,we present a sea urchin-like cuO/cu_(2)O catalyst derived from rhombic dodecahedra cu_(2)O through one-step oxidation/etching method where the mixed cu^(+)/cu^(0)states are formed via in situ reduction during *** combined effects of the morphology and the mixed cu^(+)/cu^(0)states on c–c coupling are evaluated by the Faradaic efficiency of c_(2)and the c_(2)/c1 ratio obtained in an H-cell.R-cu^(O)/cu_(2)O exhibited 49.5%Faradaic efficiency of c_(2)with a c_(2)/c1 ratio of 3.1 at−1.4 V *** hydrogen electrode,which are 1.5 and 3.2 times higher than those of R-cu_(2)O,*** a flow-cell,68.0%Faradaic efficiency of c_(2)is achieved at a current density of 500 mA·cm^(−2).The formation of the mixed cu^(+)/cu^(0)states was confirmed by in situ Raman ***,the sea urchin-like structure provides more active sites and enables faster electron *** a result,the excellent c_(2)production on R-cuO/cu_(2)O is primarily attributed to the synergistic effects of the sea urchin-like structure and the stable mixed cu^(+)/cu^(0)***,this work presents an integrated strategy for developing cu-based electrocatalysts for c_(2)production through electrochemical cO_(2)reduction.

关键词： cO_(2)electrolysis sea urchin-like structure cu^(+)/cu^(0) c_(2)products

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