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Clinical and genetic heterogeneity of adult polyglucosan body disease caused by GBE1 biallelic mutations in China

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Genes & Diseases 2024年第5期11卷 17-20页

作者： Yikun Chen Yan Shi Yuan Gao Yan hu Linying zhou Jingmei Hong Shirui Gan xiang Lin Wanjin Chen Guorong Xu Jin He Department of Neurology and Institute of Neurology of First Affiliated Hospital Institute of Neuroscienceand Fujian Key Laboratory of Molecular NeurologyFujian Medical UniversityFuzhouFujian 350005China Fujian Key Laboratory of Molecular Neurology Institute of NeuroscienceFujian Medical UniversityFuzhouFujian 350005China Electron Microscopy Lab of Public Technology Service Center Fujian Medical UniversityFuzhouFujian 350005China

Adult polyglucosan body disease(APBD)is a rare and highly heterogeneous glycogen storage disorder due to biallelic variants in GBE1.1 Typical APBD presentations include gait abnormalities with polyneuropathy,leukodystrophy,neurogenic bladder,and mild cognitive *** diagnosis of APBD encompasses a large spectrum of conditions including axonal and demyelinating sensorimotor polyneuropathy,progressive spastic paraparesis,and leukodystrophies.

关键词： GBE bladder diagnosis

Decoupled water electrolysis:Flexible strategy for pure hydrogen production with small voltage inputs

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Journal of Energy Chemistry 2024年第7期94卷 340-356页

作者： Kexin zhou Jiahui huang Daili xiang Aijiao Deng Jialei Du Hong Liu Institute for Advanced Interdisciplinary Research(iAIR) Collaborative Innovation Center of Technology and Equipment for Biological Diagnosis and Therapy in Universities of ShandongUniversity of JinanJinan 250022ShandongChina State Key Laboratory of Crystal Materials Shandong UniversityJinan 250100ShandongChina Zhengzhou Vocational College of Industrial Safety Zhengzhou 451192HenanChina

Hydrogen gas is widely regarded as an ideal green energy carrier and a potential alternative to fossil fuels for coping with the aggravating energy crisis and environmental ***,the vast majority of the world's hydrogen is produced by reforming fossil fuels;however,this hydrogen-making technology is not sustainable or environmentally friendly because ofits high energy consumption and large carbon ***-driven water splitting(2H_(2)0-2H_(2)+0_(2))becomes an extensively studied scheme for sustain-able hydrogen *** water electrolysis requires an input voltage higher than 1.23 V and forms a gas mixture of H_(2)/O_(2),which results in high electricity consumption,potential safety hazards,and harmful reactive oxygen *** virtue of the auxiliary redox mediators(RMs)as the robust H^(+)/e^(-)reservoir,decoupled electrolysis splits water at a much lower potential and evolves O_(2)(H_(2)O+RMS_(ox)-O_(2)+H-RMS_(red))and H_(2)(H-RMS_(red)-H_(2)+RMS_(ox))at separate times,rates,and spaces,thus pro-ducing the puretarget hydrogen gas *** electrolysis has accelerated the development ofwater electrolysis technology for H_(2) ***,itis still lack of a comprehensive and in-depth review in this field based on different types of *** review highlights the basic principles and critical progress of this emerging water electrolysis mode over the past *** representative examples are then dis-played in detail according to the differences in the *** rational choice and design of RMs have also been ***,novel applications of decoupled water splitting are briefly discussed,including the manufacture of valuable chemicals,Cl_(2) production,pollutant degradation,and other half-reactions in artificial ***,thekey characteristics and disadvantages of each type of mediator are sum-marized in *** addition,we present an outlook for future directions in decoupled

关键词： Hydrogen production Conventional water splitting Decoupled water splitting Redox mediators Biomimetics

Ultrahigh strength and improved electrical conductivity in an aging strengthened copper alloy processed by combination of equal channel angular pressing and thermomechanical treatment

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Journal of Central South University 2024年第6期31卷 1823-1837页

作者： WANG Xu LI zhou MENG xiang-peng XIAO Zhu School of Materials Science and Engineering Central South UniversityChangsha 410083China Ningbo Boway Alloy Material Co. Ltd.Ningbo 315135China Key Laboratory of Non-ferrous Metal Materials Science and Engineering(Ministry of Education) Changsha 410083China

In this paper,equal channel angular pressing and thermomechanical treatment was employed to improve the strength and electrical conductivity of an aging strengthened Cu-Ti-Cr-Mg alloy,and the microstructure and properties of the alloy were investigated in *** results showed that the samples deformed by the combination of cryogenic equal channel angular pressing(ECAP)and rolling had good comprehensive properties after aging at 400℃.The tensile strength of the peak-aged and over-aged samples was 1120 MPa and 940 MPa,with their corresponding electrical conductivity of 14.7%IACS and 22.1%IACS,*** and cryogenic rolling introduced high density dislocations,leading to the inhibition of the softening effects and refinement of the *** a long time aging at 400℃,the alloy exhibited ultra-high strength with obvious increasing electrical *** high strength was attributed to the synergistic effect of work hardening,grain refinement strengthening and precipitation *** precipitation of a large amount of Ti atoms from the matrix led to the high electrical conductivity of the over-aged sample.

关键词： Cu-Ti alloy equal channel angular pressing rolling aging treatment high strength

BIO-inspired intelligent navigation: from methodology, system theory, to behavioural science

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Science China(Information Sciences) 2024年第9期67卷 333-335页

作者： Wenshuo LI Xin LIU Pengwei hu Jian YANG xiang YU Lei GUO Hangzhou Innovation Institute of Beihang University School of Automation Science and Electrical Engineering Beihang University School of Instrumentation and Optoelectronic Engineering Beihang University

It is crucial for unmanned systems to determine their motion information accurately, reliably, and in real time. This function is accomplished by the autonomous navigation module of the unmanned systems. By analogy to the animals,the autonomous navigation module can be regarded as the“sensory nerve center” of an unmanned system.

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An Outbreak of Serogroup Y Meningococcal Meningitis in a Private Secondary Vocational School-Guangzhou City,Guangdong Province,China,2023

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China CDC weekly 2024年第12期6卷 242-246,I0005页

作者： xiang Zeng Qing He Yilan Li Yong zhou Xiaoping Shao Pei hu Jian Liang Yudan Song Chao Ma Lijie Zhang Limei Sun Lei Luo Chinese Field Epidemiology Training Program Chinese Center for Disease Control and PreventionBeijingChina Zhuhai Center for Disease Control and Prevention Zhuhai CityGuangdong ProvinceChina Guangdong Center for Disease Control and Prevention Guangzhou CityGuangdong ProvinceChina Guangzhou Center for Disease Control and Prevention Guangzhou CityGuangdong ProvinceChina Institute of Public Health Guangzhou Medical University&Guangzhou Center for Disease Control and PreventionGuangzhou CityGuangdong ProvinceChina Department of the National Immunization Programme Chinese Center for Disease Control and PreventionBeijingChina

What is already known about this topic?The inclusion of meningococcal vaccines in the National Immunization Program(NIP)over several years has significantly reduced the incidence of meningococcal meningitis in China to historic ***,there has been a diversification of meningococcal serogroups,leading to a shift in dominant serogroups in China from serogroup A to serogroups C and B,accompanied by a rise in reports of serogroups Y and *** is added by this report?An outbreak of serogroup Y Neisseria meningitidis(Nm)in a secondary vocational school involved a single confirmed severe case and 24 individuals with laboratory-confirmed Nm *** investigation revealed that the outbreak was localized to the classroom of the confirmed *** close contact within a confined space was identified as a significant risk factor for Nm *** genotype sequence identified was type 1655(ST-1655),which is categorized under clonal complex 23(CC-23)and bears resemblance to 8 previously confirmed cases of serogroup Y meningococcal meningitis within Guangdong *** suggests that serogroup Y infections continue to sporadically emerge and have become prevalent *** are the implications for public health practice?This outbreak underscores the critical need to enhance surveillance of meningococcal serogroups and population carrier,and advocate for vaccination with MenY-containing vaccines.

关键词： break Secondary Guangdong

Research Progress on the Association between Schizophrenia and Toxoplasma gondii Infection

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Biomedical and Environmental Sciences 2024年第6期37卷 647-660页

作者： Yiting Zhu Xiaohui Yang Miaoru Chen Yu hu Yunfeng Chang xiang Wu Department of Forensic Medicine Science Xiangya School of Basic MedicineCentral South UniversityChangsha 410013HunanChina Nursing Department The Third Xiangya Hospital of Central South UniversityChangsha 410013HunanChina Department of Basic Medicine Science Xiangya School of Basic MedicineCentral South UniversityChangsha 410013HunanChina Department of Parasitology Xiangya School of Basic MedicineCentral South UniversityChangsha 410013HunanChina

Toxoplasma gondii(*** or Tg),is an obligatory intracellular parasite with humans as its intermediate *** recent years,significant correlations between *** infection and schizophrenia have been reported,including the possible mediating ***,mechanisms and hypotheses focus on central neurotransmitters,immunity,neuroinflammation,and epigenetics;however,the exact underlying mechanisms remain *** this article,we review the studies related to *** infection and schizophrenia,particularly the latest research *** on dopamine(DA)and other neurotransmitters,the blood-brain barrier,inflammatory factors,disease heterogeneity,and other confounders is also *** addition,we also summarized the results of some new epidemiological investigations.

关键词： Toxoplasma gondii Schizophrenia Neurotransmitters Neuroinflammation Immunity Epigenetics

Thermal pretreatment of willow branches impacts yield and pore development of activated carbon in subsequent activation with ZnCl_(2) via modifying cellulose structure

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Chinese Journal of Chemical Engineering 2024年第5期69卷 227-237页

作者： Linghui Kong Chao Li Runxing Sun Shu Zhang Yi Wang Jun xiang Song hu Dong Wang Chuanjun Leng Xun hu School of Material Science and Engineering University of JinanJinan 250022China Joint International Research Laboratory of Biomass Energy and Materials College of Materials Science and EngineeringNanjing Forestry UniversityNanjing 210037China State Key Laboratory of Coal Combustion Huazhong University of Science and TechnologyWuhan 430074China Shandong BASAN Graphite New Material Plant Zibo 255311China

Development of pore structures of activated carbon(AC)from activation of biomass with ZnCl_(2) relies on content and structure of cellulose/hemicellulose in the *** pretreatment of biomass could induce dehydration and/or aromatization to change the structure of cellulose/*** might interfere with evolution of structures of AC,which was investigated herein via thermal pretreatment of willow branch(WB)from 200 to 360℃and the subsequent activation with ZnCl_(2) at 550℃.The results showed that thermal pretreatment at 360℃(WB-360)could lead to substantial pyrolysis to form biochar,with a yield of 31.9%,accompanying with nearly complete destruction of cellulose crystals and remarkably enhanced aromatic ***,cellulose residual in WB-360 could still be activated to form AC-360 with specific surface area of 1837.9 m~2·g^(-1),which was lower than that in AC from activation of untreated WB(AC-blank,2077.8 m~2·g^(-1)).Nonetheless,the AC-200 from activation of WB-200 had more developed pores(2113.9 m~2·g^(-1))and superior capability for adsorption of phenol,due to increased permeability of ZnCl_(2) to the largely intact cellulose structure in *** thermal pretreatment did increase diameters of micropores of AC but reduced the overall yield of AC(26.8%for AC-blank versus 18.0%for AC-360),resulting from accelerated cracking but reduced intensity of ***-situ infrared characterization of the activation showed that ZnCl_(2) mainly catalyzed dehydration,dehydrogenation,condensation,and aromatization but not cracking,suppressing the formation of derivatives of cellulose and lignin in *** thermal pretreatment formed phenolic-OH and C=O with higher chemical innerness,which changed the reaction network in activation,shifting morphology of fibrous structures in AC-blank to“melting surface”in AC-200 or AC-280.

关键词： Thermal pretreatment Activation with ZnCl_(2) Willow branch Activated carbon Biochar

Recent progress and strategies of cathodes toward polysulfides shuttle restriction for lithium-sulfur batteries

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Rare Metals 2024年第9期43卷 4132-4161页

作者： Xing-You Rao Shuang-Fei xiang Jian zhou Zhen Zhang xiang-Yu Xu Yuan-Yuan Xu Xin-Chi zhou Zheng-Dao Pan Su-Chong Tan Shi-Xing Dong zhou-Lu Wang Yu-Tong Wu Yun-Lei zhou xiang Liu Yi Zhang Shan Jiang School of Energy Sciences and Engineering Nanjing Tech University School of Materials Science and Engineering and Institute of Smart Fiber Materials Zhejiang Sci-Tech University JiangSu Sailboat Petrochemical Co. Ltd Hangzhou Institute of Technology Xidian University

Lithium-sulfur batteries(LSBs) have already developed into one of the most promising new-generation high-energy density electrochemical energy storage systems with outstanding features including high-energy density, low cost, and environmental friendliness. However, the development and commercialization path of LSBs still presents significant limitations and challenges, particularly the notorious shuttle effect triggered by soluble longchain lithium polysulfides(LiPSs), which inevitably leads to low utilization of cathode active sulfur and high battery capacity degradation, short cycle life, etc. Substantial research efforts have been conducted to develop various sulfur host materials capable of effectively restricting the shuttle effect. This review firstly introduces the fundamental electrochemical aspects of LSBs, followed by a comprehensive analysis of the mechanism underlying the shuttle effect in Li–S batteries and its profound influence on various battery components as well as the overall battery performance. Subsequently, recent advances and strategies are systematically reviewed, including physical confinement, chemisorption, and catalytic conversion of sulfur hosts for restricting LiPSs shuttle effects. The interplay mechanisms of sulfur hosts and LiPSs are discussed in detail and the structural advantages of different host materials are highlighted. Furthermore, key insights for the rational design of advanced host materials for LSBs are provided, and the upcoming challenges and the prospects for sulfur host materials in lithium-sulfur batteries are also explored.

关键词： Host Lithium-sulfur batteries Polysulfides Shuttle effect

Angiosperm-wide analysis of fruit and ovary evolution aided by a new nuclear phylogeny supports association of the same ovary type with both dry and fleshy fruits

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Journal of Integrative Plant Biology 2024年第2期66卷 228-251页

作者： Yezi xiang Taikui Zhang Yiyong Zhao Hongjin Dong Hongyi Chen Yi hu Chien-Hsun huang Jun xiang Hong Ma State Key Laboratory of Genetic Engineering Ministry of Education Key Laboratory of Biodiversity and Ecological EngineeringInstitute of Plant BiologyCenter of Evolutionary BiologySchool of Life SciencesFudan UniversityShanghai 200438China Department of Biology Howard Hughes Medical InstituteDuke UniversityDurhamNC 27708USA Department of Biology the Eberly College of Scienceand the Huck Institutes of the Life Sciencesthe Pennsylvania State UniversityUniversity ParkPA 16802USA hubei Key Laboratory of Economic Forest Germplasm Improvement and Resources Comprehensive Utilization College of Biology and Agricultural ResourcesHuanggang Normal UniversityHuanggang 438000China

Fruit functions in seed protection and dispersal and belongs to many dry and fleshy types,yet their evolutionary pattern remains unclear in part due to uncertainties in the phylogenetic relationships among several orders and *** we used nuclear genes of 502 angiosperm species representing 231 families to reconstruct a well supported phylogeny,with resolved relationships for orders and families with previously uncertain *** this phylogeny as a framework,molecular dating supports a Triassic origin of the crown angiosperms,followed by the emergence of most orders in the Jurassic and Cretaceous and their rise to ecological dominance during the Cretaceous Terrestrial *** robust phylogeny allowed an examination of the evolutionary pattern of fruit and ovary types,revealing a trend of parallel carpel fusions during early diversifications in eudicots,monocots,and ***,taxa in the same order or family with the same ovary type can develop either dry or fleshy fruits with strong correlations between specific types of dry and fleshy fruits;such associations of ovary,dry and fleshy fruits define several ovaryfruit"modules"each found in multiple *** of the frequent modules has an ovary containing multiple ovules,capsules and berries,and another with an ovary having one or two ovules,achenes(or other single-seeded dry fruits)and *** new perspective of relationships among fruit types highlights the closeness of specific dry and fleshy fruit types,such as capsule and berry,that develop from the same ovary type and belong to the same module relative to dry and fleshy fruits of other modules(such as achenes and drupes).Further analyses of gene families containing known genes for ovary and fruit development identified phylogenetic nodes with multiple gene duplications,supporting a possible role of whole-genome duplications,in combination with climate changes and animal behaviors,in angiosperm fruit and ovary diversificat

关键词： angiosperm fruit evolution MADS-box molecular clock nuclear phylogeny ovary and fruit developmental genes