检索结果-南通市图书馆

Gene therapy for spinal muscular atrophy:perspectives on the possibility of optimizing SMN1 delivery to correct all neurological and systemic perturbations

在线全文

学校读者我要写书评

暂无评论

Neural Regeneration Research 2025年第7期20卷 2011-2012页

作者： Sharon j.Brown Rafael j.yáñez-Muñoz Heidi R.Fuller School of Pharmacy and Bioengineering Keele UniversityKeeleUK Wolfson Center for Inherited Neuromuscular Disease TORCH BuildingRJAH Orthopaedic HospitalOswestryUK *** Center of Gene and Cell TherapyDepartment of Biological SciencesSchool of Life Sciences and the EnvironmentRoyal Holloway University of LondonEghamUK

Spinal muscular atrophy(SMA)is a genetic condition that results in selective lower motor neuron loss with concomitant muscle weakness and *** genetic cause of SMA was understood in 1995 when loss or impairment of the survival motor neuron 1(SMN1)gene was identified as the main contributing factor(Lefebvre et al.,1995).This,in combination with the discovery that humans have a“back-up”gene,SMN2,which can produce low levels(approximately 10%)of the full-length functional SMN protein,has led to the generation of SMA-specific gene *** was traditionally classified according to age of symptom onset and developmental milestones achieved,with life expectancy and severity varying between ***,SMN2 copy number is used as a proxy for the prediction of disease severity,with higher SMN2 copy number typically being associated with reduced severity of SMA,although this relationship is not absolute:some individuals with low SMN2 copy number have less severe SMA phenotypes and vice ***,the etiology of SMA is further complicated by other factors,such as non-typical nucleotide variants and SMN2-independent modifiers of disease severity.

关键词： atrophy traditionally absolute

以柴油机车废热为能源的吸收式空调的试验研究

在线全文

学校读者我要写书评

暂无评论

家电科技 2005年第3期 76-76页

作者： y．Z．Lu R．Z．Wang S．jianzhou y.X．Xu j.y.wu

本文是关于利用柴油机车的废热为能源的吸收式空调实际性能的试验性研究。该系统包括吸收床，硅-水作工质对，以及调节机车驾驶室空气温度的冷冻水。作者在不同工况下对系统进行了性能测试，试验结果显示：本文的吸收式系统在技术上是... 详细信息

本文是关于利用柴油机车的废热为能源的吸收式空调实际性能的试验性研究。该系统包括吸收床，硅-水作工质对，以及调节机车驾驶室空气温度的冷冻水。作者在不同工况下对系统进行了性能测试，试验结果显示：本文的吸收式系统在技术上是可行的，可以应用在机车的驾驶室空调系统上，特别适合在铁路机车上使用。在常规的运行工况条件下，该系统的平均制冷量为3．0kW到4．2kW。

关键词：柴油机车铁路机车机车驾驶驾驶室试验研究空调系统工况吸收式空调冷冻水废热

Plasmonic tandem heterojunctions enable high-efficiency charge transfer for broad spectrum photocatalytic hydrogen production

维普期刊数据库评论

在线全文

维普期刊数据库

学校读者我要写书评

暂无评论

journal of Energy Chemistry 2025年第1期100卷 710-720页

作者： Wenxuan Chen Xiu-Qing Qiao Guo Hui Bojing Sun Dongfang Hou Meidi Wang Xueqian wu Tao wu Dong-Sheng Li College of Materials and Chemical Engineering Key Laboratory of Inorganic Nonmetallic Crystalline and Energy Conversion Materials China Three Gorges University Hubei Three Gorges Laboratory College of Chemistry and Materials Science and Guangdong Provincial Key Laboratory of Supramolecular Coordination Chemistry Jinan University

Rational engineering of semiconductor photocatalysts for efficient hydrogen production is of great significance but still challenging,primarily due to the limitations in charge transfer ***,a fascinating plasmonic tandem heterojunction with the hc-CdS/Mo2C@C heterostructure is aimfully prepared for effectively promoting the charge separation kinetics of the CdS photocatalyst via the synergistic strategy of phase junction,Schottky junction,and photothermal *** difference in atomic configuration between cubic-CdS （c-CdS） and hexagonal-CdS （h-CdS） leads to effective charge separation through a typical Ⅱ charge transfer mechanism,and plasmonic Schottky junction further extracts the electrons in the hc-CdS phase junction to realize gradient charge ***,the photothermal effect of Mo2C@C helps to expand the light absorption,accelerate charge transfer kinetics,and reduce the hydrogen evolution energy *** carbon layer provides a fast channel for charge transfer and protects the photocatalyst from *** a result,the optimized hc-CMC photocatalyst exhibits a significantly high photocatalytic H2production activity of 28.63 mmol/g/h and apparent quantum efficiency of 61.8%,surpassing most of the reported *** study provides a feasible strategy to enhance the charge transfer kinetics and photocatalytic activity of CdS by constructing plasmonic tandem heterogeneous junctions.

关键词： Cadmium sulfide

Ammonia pyrolysis oxidation excited by nanosecond pulsed discharge:Global/fluid models hybrid solution

在线全文

同方期刊数据库

学校读者我要写书评

暂无评论

journal of Energy Chemistry 2025年第1期100卷 385-396页

作者： Mingming Zhang Hanyu wu Qi Chen jiaxin yang Bonan Liu School of Mechanical Electronic and Control Engineering Beijing Jiaotong University China University of Petroleum

The kinetic characteristics of plasma-assisted oxidative pyrolysis of ammonia are studied by using the global/fluid models hybrid solution ***,the stable products of plasma-assisted oxidative pyrolysis of ammonia are *** results show that the consumption of NH3/O2and the production of N2/H2change linearly with the increase of voltage,which indicates the decoupling of nonequilibrium molecular excitation and oxidative pyrolysis of ammonia at low ***,the detailed reaction kinetics mechanism of ammonia oxidative pyrolysis stimulated by a nanosecond pulse voltage at low pressure and room temperature is *** on the reaction path analysis,the simplified mechanism is *** detailed and simplified mechanism simulation results are compared with experimental data to verify the accuracy of the simplified ***,based on the simplified mechanism,the fluid model of ammonia oxidative pyrolysis stimulated by the nanosecond pulse plasma is established to study the pre-sheath/sheath behavior and the resultant consumption and formation of key *** results show that the generation,development,and propagation of the pre-sheath have a great influence on the formation and consumption of *** consumption of NH3by the cathode pre-sheath is greater than that by the anode pre-sheath,but the opposite is true for OH and O（1S）.However,within the sheath,almost all reactions do not ***,by changing the parameters of nanosecond pulse power supply voltage,it is found that the electron number density,electron current density,and applied peak voltages are not the direct reasons for the structural changes of the sheath and ***,the discharge interval has little effect on the sheath structure and gas mixture *** research results of this paper not only help to understand the kinetic promotion of non-equilibrium excitation in the process of oxidative pyrolysis but also help to explore th

关键词： Nanosecond pulsed discharge Ammonia Oxidative pyrolysis Fluid model Global model

Tuning the interfacial reaction environment via pH-dependent and induced ions to understand C–N bonds coupling performance in NO3- integrated CO2 reduction to carbon and nitrogen compounds over dual Cu-based N-doped carbon catalyst

在线全文

同方期刊数据库

学校读者我要写书评

暂无评论

journal of Energy Chemistry 2025年第1期100卷 273-285页

作者： Tianhang Zhou Chen Shen Zhenghao wu Xingying Lan yi Xiao College of Carbon Neutrality Future Technology State Key Laboratory of Heavy Oil Processing China University of Petroleum (Beijing) Institute of Materials Science TU Darmstadt Department of Chemistry Xi'an Jiaotong-Liverpool University Department of Mechanical and Mechatronics Engineering and Waterloo Institute of Nanotechnology University of Waterloo Waterloo

Dual atomic catalysts （DAC）,particularly copper （Cu2）-based nitrogen （N） doped graphene,show great potential to effectively convert CO2and nitrate （NO3-） into important industrial chemicals such as ethylene,glycol,acetamide,and urea through an efficient catalytical process that involves C–C and C–N ***,the origin of the coupling activity remained unclear,which substantially hinders the rational design of Cu-based catalysts for the N-integrated CO2reduction reaction （CO2RR）.To address this challenge,this work performed advanced density functional theory calculations incorporating explicit solvation based on a Cu2-based N-doped carbon (Cu2N6C10) catalyst for *** calculations are aimed to gain insight into the reaction mechanisms for the synthesis of ethylene,acetamide,and urea via coupling in the interfacial reaction *** to the sluggishness of CO2,the formation of a solvation electric layer by anions （F-,Cl-,Br-,and I-） and cations (Na+,Mg2+,K+,and Ca2+) leads to electron transfer towards the Cu *** process significantly accelerates the reduction of *** results reveal that*CO intermediates play a pivotal role in N-integrated ***,the Cu2-based N-doped carbon catalyst examined in this study has demonstrated the most potential for C–N coupling to *** findings reveal that through the process of a condensation reaction between*CO and NH2OH for urea synthesis,*NO3-is reduced to*NH3,and*CO2to*CCO at dual Cu atom *** dual-site reduction facilitates the synthesis of acetamide through a nucleophilic reaction between NH3and the ketene ***,we found that the I-and Mg2+ions,influenced by pH,were highly effective for acetamide and ammonia synthesis,except when F-and Ca2+were ***,the mechanisms of C–N bond formation were investigated via ab-initio molecular dynamics simulations,and we found that adjusting the micro-environment can change the dominant side reaction,shift

关键词： Condensation reactions

Advancements and Challenges in Organic–Inorganic Composite Solid Electrolytes for All‑Solid‑State Lithium Batteries

在线全文

同方期刊数据库

学校读者我要写书评

暂无评论

Nano-Micro Letters 2025年第1期17卷 46-97页

作者： Xueyan Zhang Shichao Cheng Chuankai Fu Geping yin Liguang Wang yongmin wu Hua Huo State Key Laboratory of Space Power‑Sources School of Chemistry and Chemical EngineeringHarbin Institute of TechnologyHarbin 150001People’s Republic of China College of Chemical and Biological Engineering Zhejiang UniversityHangzhou 310058People’s Republic of China State Key Laboratory of Space Power-Sources 2965 Dongchuan RoadMinhang DistrictShanghai 200240People’s Republic of China

To address the limitations of contemporary lithium-ion batteries,particularly their low energy density and safety concerns,all-solid-state lithium batteries equipped with solid-state electrolytes have been identified as an up-and-coming *** the various SEs,organic–inorganic composite solid electrolytes(OICSEs)that combine the advantages of both polymer and inorganic materials demonstrate promising potential for large-scale ***,OICSEs still face many challenges in practical applications,such as low ionic conductivity and poor interfacial stability,which severely limit their *** review provides a comprehensive overview of recent research advancements in ***,the influence of inorganic fillers on the main functional parameters of OICSEs,including ionic conductivity,Li+transfer number,mechanical strength,electrochemical stability,electronic conductivity,and thermal stability are systematically *** lithium-ion conduction mechanism of OICSE is thoroughly analyzed and concluded from the microscopic ***,the classic inorganic filler types,including both inert and active fillers,are categorized with special emphasis on the relationship between inorganic filler structure design and the electrochemical performance of ***,the advanced characterization techniques relevant to OICSEs are summarized,and the challenges and perspectives on the future development of OICSEs are also highlighted for constructing superior ASSLBs.

关键词： Composite solid electrolytes Inorganic filler Interfacial stability Li-ion conduction mechanism Characterization techniques

Co-production of hydrogen, oxygen, and electricity via an integrated solar-driven system with decoupled water electrolyzer and Na-Zn ion battery

在线全文

学校读者我要写书评

暂无评论

journal of Energy Chemistry 2025年第1期100卷 621-627页

作者： Fei Lv Longjie Liu jiazhe wu Pengfei Wang Lixia Pan Dengwei jing yubin Chen International Research Center for Renewable Energy State Key Laboratory of Multiphase Flow in Power Engineering Xi'an Jiaotong University

Combining water electrolysis and rechargeable battery technologies into a single system holds great promise for the co-production of hydrogen （H2） and ***,the design and development of such systems is still in its ***,an integrated hydrogen-oxygen （O2）-electricity co-production system featuring a bipolar membrane-assisted decoupled electrolyzer and a Na-Zn ion battery was established with sodium nickelhexacyanoferrate （NaNiHCF） and Zn2+/Zn as dual redox *** decoupled electrolyzer enables to produce H2and O2in different time and space with almost 100%Faradaic efficiency at 100 mA ***,the charged NaNiHCF and Zn electrodes after the electrolysis processes formed a Na-Zn ion battery,which can generate electricity with an average cell voltage of 1.75 V at 10 m A *** connecting Si photovoltaics with the modular electrochemical device,a well-matched solar driven system was built to convert the intermittent solar energy into hydrogen and electric energy with a solar to hydrogen-electricity efficiency of 16.7%,demonstrating the flexible storage and conversion of renewables.

关键词： Hydrogen Electricity Decoupled water electrolyzer Na-Zn ion battery Solar energy

Study on the hydrogen absorption properties of a yGdTbDyHo rare-earth high-entropy alloy

在线全文

同方期刊数据库

学校读者我要写书评

暂无评论

International journal of Minerals,Metallurgy and Materials 2025年第1期32卷 127-135页

作者： Tongyue Li Ziliang Xie Wenjiao Zhou Huan Tong Dawen yang Anjia Zhang yuan wu Xiping Song State Key Laboratory for Advanced Metals and Materials University of Science and Technology BeijingBeijing 100083China Whole Win(Beijing)Materials Sci.&Tech.Co. Ltd.Beijing 100083China

This study investigated the microstructure and hydrogen absorption properties of a rare-earth high-entropy alloy(HEA),*** indicated that the yGdTbDyHo alloy had a microstructure of equiaxed grains,with the alloy elements distributed *** hydrogen absorption,the phase structure of the HEA changed from a solid solution with an hexagonal-close-packed(HCP)structure to a high-entropy hydride with an faced-centered-cubic(FCC)structure without any secondary phase *** alloy demonstrated a maximum hydrogen storage capacity of 2.33 H/M(hydrogen atom/metal atom)at 723 K,with an enthalpy change(ΔH)of-141.09 kj·mol^(-1)and an entropy change(ΔS)of-119.14 j·mol^(-1)·K^(-1).The kinetic mechanism of hydrogen absorption was hydride nucleation and growth,with an apparent activation energy(E_(a))of 20.90 kj·mol^(-1).Without any activation,the yGdTbDyHo alloy could absorb hydrogen quickly(180 s at 923 K)with nearly no incubation period *** reason for the obtained value of 2.33 H/M was that the hydrogen atoms occupied both tetrahedral and octahedral *** results demonstrate the potential application of HEAs as a high-capacity hydrogen storage material with a large H/M ratio,which can be used in the deuterium storage field.

关键词： rare-earth high-entropy alloy hydrogen absorption capacity pressure–composition–temperature curves kinetics

A mild, configurable, flexible CoNi-LDH（v）/Zn battery based on H-vacancy-induced reversible Zn2+ intercalation

在线全文

学校读者我要写书评

暂无评论

journal of Energy Chemistry 2025年第1期100卷 498-508页

作者： Xinyue Cheng Dingsheng wu Hao Gao Qingqing Wang Pengfei Lv Sam S.yoon Qufu Wei Key Laboratory of Eco-textiles Ministry of Education Jiangnan University School of Mechanical Engineering Korea University

Flexible Zn-based batteries have attracted increasing research interest as essential components of wearable energy storage ***,the advancement of flexible aqueous Zn-based batteries based on Co-Ni layered double hydroxide （CoNi-LDH） as the cathode material is hampered by their poor cycling stability and the corrosiveness of alkaline ***,CoNi-LDH nanosheets enriched with H vacancies (CoNi-LDH（v）) were constructed on a flexible carbon cloth （CC） substrate via electrochemical deposition and *** Zn-based battery comprising CoNi-LDH（v）@CC as the cathode exhibited highly reversible conversion reactions and stable operation in 3 M ZnSO4electrolyte （pH=4）.The battery delivered an excellent specific capacity (225 mA h g-1,0.26 mA h cm-2),acceptable cycling stability（53.9%,900 cycles）,and high discharging *** abundant H vacancies served as active sites for the reversible intercalation of Zn2+and the extravasation of NO3-generated channels and space for Zn2+transport and storage,together enabling an excellent Zn2+storage ***,a sandwich-structured solid-state CoNi-LDH（v）@CC//Zn@CC battery was fabricated and was found to exhibit a noteworthy electrochemical performance and mechanical *** a proof of concept,the unencapsulated battery powered a digital watch under various deformation conditions and operated stably for 80 ***,the flexible battery displayed outstanding customizability,maintaining an open-circuit voltage of 1.42 V even after being cut *** proposed engineering strategy contributes to the realization of textiles with truly wearable energy-storage devices.

关键词： CoNi/Zn battery H vacancies Mild aqueous electrolyte Tailorable battery Wearable energy storage device