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Novel MIP gene mutation causes autosomal-dominant congenital cataract

International Journal of Ophthalmology(English edition) 2024年第3期17卷 454-465页

作者： Jing-lan Ni Hua-ming Wen Xiao-Sheng Huang Qian-Wen Li Jia-Min Cai bao-Jian Fan Jun Zhao The Second Clinical Medical College Jinan UniversityShenzhen 518020Guangdong ProvinceChina Department of Ophthalmology Dongguan Chang’an HospitalDongguan 523843Guangdong ProvinceChina Shenzhen Eye Hospital Affiliated to Jinan University Shenzhen Eye InstituteShenzhen 518040Guangdong ProvinceChina Department of Oral&Maxillofacial Surgery Shenzhen Stomatology Hospital Affiliated to Shenzhen UniversityShenzhen 518040Guangdong ProvinceChina Department of Neurosurgery Massachusetts General Hospital and Harvard Medical SchoolBoston 02114MassachusettsUSA Department of Ophthalmology Shenzhen People’s Hospital(the Second Clinical Medical CollegeJinan Universitythe First Affiliated HospitalSouthern University of Science and Technology)Shenzhen 518020Guangdong ProvinceChina

●AIM:To identify disease-causative mutations in families with congenital cataract.●METHODS:Two Chinese families with autosomaldominant congenital cataract(ADCC)were recruited and underwent comprehensive eye examinations.Gene panel next-generation sequencing of common pathogenic genes of congenital cataract was performed in the proband of each family.Sanger sequencing was used to valid the candidate gene mutations and sequence the other family members for co-segregation analysis.The effect of sequence changes on protein structure and function was predicted through bioinformatics analysis.Major intrinsic protein(MIP)-wildtype and MIP-G29R plasmids were constructed and microinjected into zebrafish single-cell stage embryos.Zebrafish embryonic lens phenotypes were screened using confocal microscopy.●RESULTS:A novel heterozygous mutation(c.85G>A;p.G29R)in the MIP gene was identified in the proband of one family.A known heterozygous mutation(c.97C>T;p.R33C;rs864309693)in MIP was found in the proband of another family.In-silico prediction indicated that the novel mutation might affect the MIP protein function.Zebrafish embryonic lens was uniformly transparent in both wild-type PCS2+MIP and mutant PCS2+MIP.●CONCLUSION:Two missense mutations in the MIP gene in Chinese cataract families are identified,and one of which is novel.These findings expand the genetic spectrum of MIP mutations associated with cataracts.The functional studies suggest that the novel MIP mutation might not be a gain-of-function but a loss-of-function mutation.

关键词： congenital cataract major intrinsic protein missense mutation zebrafish model

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Electrochemical Reduction of Benzo[b]thiophene 1,1-Dioxides with HFIP as Hydrogen Donor

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Chinese Journal of Chemistry 2024年第6期42卷 585-591页

作者： ming-Zhong Guo Mei-Jin Mou Zhuo Chen Shao-Fei Ni ming Li Li-Rong Wen Lin-bao Zhang State Key Laboratory Base of Eco-Chemical Engineering College of Chemistry and Molecular EngineeringQingdao University of Science & TechnologyQingdaoShandong266042 China Department of Chemistry and Key Laboratory for Preparation and Application of Ordered Structural Materials of Guangdong Province Shantou UniversityShantouGuangdong515063 China

A straightforward electrochemical reduction of benzo[b]thiophene 1,1-dioxides with HFIP as the hydrogen donor has been reported in an undivided cell under metal-free conditions.Moreover,the tolerance of various functi... 详细信息

关键词： Electrochemistry Reduction Benzo[b]thiophene 1,1-dioxides Deuterium Hydrogen source

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Temperature inversion enables superior stability for low-temperature Zn-ion batteries

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Journal of Energy Chemistry 2024年第4期91卷 245-253页

作者： Fu-Da Yu Zhe-Jian Yi Rui-Yang Li Wei-Hao Lin Jie Chen Xiao-Yue Chen Yi-ming Xie Ji-Huai Wu Zhang lan lan-Fang Que bao-Sheng Liu Hao Luo Zhen-Bo Wang Engineering Research Center of Environment-Friendly Functional Materials Ministry of EducationInstitute of Materials Physical ChemistryHuaqiao UniversityXiamen 361021FujianChina b2 School of Electronic Engineering Guangxi University of Science and TechnologyLiuzhou 545006GuangxiChina School of Materials Science and Engineering Xiamen University of TechnologyXiamen 361024FujianChina School of Materials Science and Engineering Zhengzhou UniversityZhengzhou 450001HenanChina MIIT Key Laboratory of Critical Materials Technology for New Energy Conversion and Storage State Key Laboratory of Space Power-SourcesSchool of Chemistry and Chemical EngineeringHarbin Institute of TechnologyHarbin 150001HeilongjiangChina College of Materials Science and Engineering Shenzhen UniversityShenzhen 518071GuangdongChina

It is challenging for aqueous Zn-ion batteries(ZIBs)to achieve comparable low-temperature(low-T)performance due to the easy-frozen electrolyte and severe Zn dendrites.Herein,an aqueous electrolyte with a low freezing point and high ionic conductivity is proposed.Combined with molecular dynamics simulation and multi-scale interface analysis(time of flight secondary ion mass spectrometry threedimensional mapping and in-situ electrochemical impedance spectroscopy method),the temperature independence of the V_(2)O_(5)cathode and Zn anode is observed to be opposite.Surprisingly,dominated by the solvent structure of the designed electrolyte at low temperatures,vanadium dissolution/shuttle is significantly inhibited,and the zinc dendrites caused by this electrochemical crosstalk are greatly relieved,thus showing an abnormal temperature inversion effect.Through the disclosure and improvement of the above phenomena,the designed Zn||V_(2)O_(5)full cell delivers superior low-T performance,maintaining almost 99%capacity retention after 9500 cycles(working more than 2500 h)at-20°C.This work proposes a kind of electrolyte suitable for low-T ZIBs and reveals the inverse temperature dependence of the Zn anode,which might offer a novel perspective for the investigation of low-T aqueous battery systems.

关键词： Aqueous Zn-ion batteries Low-temperature performance Opposite temperature dependence Zndendrite growth Vanadium dissolution

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Failure evaluation and control factor analysis of slope block instability along traffic corridor in Southeastern Tibet

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Journal of Mountain Science 2024年第6期21卷 1830-1848页

作者： LIU Changqing bao Han lan Hengxing YAN Changgen LI Changbo LIU Shijie School of Highway Chang'an UniversityXi'an 710064China State Key Laboratory of Resources and Environmental Information System Institute of Geographic Sciences and Natural Resources ResearchChinese Academy of SciencesBeijing 100101China College of Geological Engineering and Geomatics Chang'an UniversityXi'an 710054China

The instability of slope blocks occurred frequently along traffic corridor in Southeastern Tibet(TCST),which was primarily controlled by the rock mass structures.A rapid method evaluating the control effects of rock mass structures was proposed through field statistics of the slopes and rock mass structures along TCST,which combined the stereographic projection method,modified M-JCS model,and limit equilibrium theory.The instabilities of slope blocks along TCST were then evaluated rapidly,and the different control factors of instability were analyzed.Results showed that the probabilities of toppling(5.31%),planar(16.15%),and wedge(35.37%)failure of slope blocks along TCST increased sequentially.These instability modes were respectively controlled by the anti-dip joint,the joint parallel to slope surface with a dip angle smaller than the slope angle(singlejoint),and two groups of joints inclined out of the slope(double-joints).Regarding the control effects on slope block instability,the stabilization ability of doublejoints(72.7%),anti-dip joint(67.4%),and single-joint(57.6%)decreased sequentially,resulting in different probabilities of slope block instability.Additionally,nearby regional faults significantly influenced the joints,leading to spatial heterogeneity and segmental clustering in the stabilization ability provided by joints to the slope blocks.Consequently,the stability of slope blocks gradually weakened as they approached the fault zones.This paper can provide guidance and assistance for investigating the development characteristics of rock mass structures and the stability of slope blocks.

关键词： Slope engineering Block instability Rapid evaluation method Rock mass structure Control factor

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Optimal V_(2)O_(3) extraction by sustainable vanadate electrolysis in molten salts

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Tungsten 2024年第1期6卷 141-149页

作者： bao-Yan Feng Zhong-Hua Zhao Yong-Zheng Jia Jia-Liang An ming-Yong Wang State Key Laboratory of Advanced Metallurgy University of Science and Technology BeijingBeijing 100083China Beijing Key Laboratory of Green Recovery and Extraction of Rare and Precious Metals University of Science and Technology BeijingBeijing 100083China

Recently,an environmentally friendly electrolysis process of soluble vanadates is proposed and successfully confirmed for V_(2)O_(3) extraction.In this paper,the solubilities of various vanadates(i.e.NaVO_(3),Na_(4)V_(2)O_(7) and Na_(3)VO_(4))in NaCl molten salt are measured.The dependences of V_(2)O_(3) extraction on vanadate form,anode material and molten salt system are studied.A long-term electrolysis is carried out.The result indicates that the solubilities of all vanadates are high and meet electrolytic requirements.Compared to Na_(4)V_(2)O_(7) and Na_(3)VO_(4),NaVO_(3) exhibits larger current efficiency and lower electricity consumption.By using SnO_(2) anode instead of graphite anode,the current efficiency of NaVO_(3) electrolysis can be increased to 58.1%and carbon pollution is avoided at 700℃.The extraction rate of V_(2)O_(3) reaches 81.3%after long-term electrolysis of 51 h.This work optimizes the electrochemical preparation process of V_(2)O_(3) from various vanadates and contributes to the improvement of current efficiency and extraction rate.

关键词： Molten salt electrolysis Vanadate Vanadium trioxide Current efficiency

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On the Preparation of Low-Temperature-Rise and Low-Shrinkage Concrete Based on Phosphorus Slag

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Fluid Dynamics & Materials Processing 2024年第4期20卷 803-814页

作者： Jianlong Jin Jingjing Ding Long Xiong ming bao Peng Zeng China Construction Ready Mixed Concrete Co. Ltd.Wuhan430000China

The effects of different contents of a MgO expansive agent and phosphorus slag on the mechanical properties,shrinkage behavior,and the heat of hydration of concrete were studied.The slump flow,setting time,dry shrinkage,and hydration heat were used as sensitive parameters to assess the response of the considered specimens.As shown by the results,in general,with an increase in the phosphorus slag content,the hydration heat of concrete decreases for all ages,but the early strength displays a downward trend and the dry shrinkage rate increases.The 90-d strength and dry shrinkage of concrete could be improved with a phosphorus residue content between 0%-20%,with the best performances in terms of mechanical properties and shrinkage characteristics being achieved for a content of 20 kg/m^(3).On the basis of these results,it can be concluded that appropriate amounts of phosphorus slag and MgO expansive agent can be used to improve the compressive strength of concrete in the later stage by reducing the hydration heat and dry shrinkage rate,respectively.

关键词： Phosphorus slag MgO expansion agent mass concrete hydration heat

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Sequential enantioselective Ugi-4CR/post-Ugi transformation strategy:a precise construction of structurally diverse azaspiro polycyclic scaffolds

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Science China Chemistry 2024年第2期67卷 576-586页

作者： Xiao-bao Wu Jun-Xiu Shi Yi-ming Ou Hua-Jie Jiang Yi-Jun Fang Qi-ming Wang Quan Gao Jie Yu Department of Applied Chemistry Anhui Province Engineering Laboratory for Green Pesticide Development and ApplicationAnhui Province Key Laboratory of Crop Integrated Pest ManagementAnhui Agricultural UniversityHefei230036China

A privileged strategy has been developed for the precise construction of enantioenriched azaspiro polycyclic scaffolds.Structurally diverse azaspiro polycycles bearing multiple contiguous stereocenters are obtained with excellent results(up to 99:1 e.r.,>95:5 d.r.) via sequential enantioselective four-component Ugi reactions/post-Ugi transformations with substrates containing prerequisite functional groups in the presence of anionic stereogenic-at-cobalt(Ⅲ) complexes.

关键词： asymmetric catalysis post-Ugi transformation anionic stereogenic-at-cobalt(Ⅲ)complex azaspiro polycyclic scaffold one-pot synthesis

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Multi-omics analyses provide insights into the evolutionary history and the synthesis of medicinal components of the Chinese wingnut

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Plant Diversity 2024年第3期46卷 309-320页

作者： Zi-Yan Zhang He-Xiao Xia Meng-Jie Yuan Feng Gao Wen-Hua bao lan Jin Min Li Yong Li College of Life Science and Technology Inner Mongolia Normal UniversityHohhot 010020China Key Laboratory of Biodiversity Conservation and Sustainable Utilization in Mongolian Plateau for College and University of Inner Mongolia Autonomous Region Hohhot 010022China College of landscape and Art Henan Agricultural UniversityZhengzhou 450002China State Key Laboratory of Tree Genetics and Breeding Chinese Academy of ForestryBeijing 100091China

Chinese wingnut(Pterocarya stenoptera)is a medicinally and economically important tree species within the family Juglandaceae.However,the lack of high-quality reference genome has hindered its in-depth research.In this study,we successfully assembled its chromosome-level genome and performed multiomics analyses to address its evolutionary history and synthesis of medicinal components.A thorough examination of genomes has uncovered a significant expansion in the Lateral Organ Boundaries Domain gene family among the winged group in Juglandaceae.This notable increase may be attributed to their frequent exposure to flood-prone environments.After further differentiation between Chinese wingnut and Cyclocarya paliurus,significant positive selection occurred on the genes of NADH dehydrogenase related to mitochondrial aerobic respiration in Chinese wingnut,enhancing its ability to cope with waterlogging stress.Comparative genomic analysis revealed Chinese wingnut evolved more unique genes related to arginine synthesis,potentially endowing it with a higher capacity to purify nutrient-rich water bodies.Expansion of terpene synthase families enables the production of increased quantities of terpenoid volatiles,potentially serving as an evolved defense mechanism against herbivorous insects.Through combined transcriptomic and metabolomic analysis,we identified the candidate genes involved in the synthesis of terpenoid volatiles.Our study offers essential genetic resources for Chinese wingnut,unveiling its evolutionary history and identifying key genes linked to the production of terpenoid volatiles.

关键词： Genome Medicinal components Metabolome Pterocarya stenoptera Transcriptome

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Degradation analysis and doping modification optimization for high-voltage P-type layered cathode in sodium-ion batteries

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Journal of Energy Chemistry 2024年第2期89卷 1-9,I0002页

作者： bao Zhang Yi Zhao minghuang Li Qi Wang Lei Cheng Lei ming Xing Ou Xiaowei Wang National Engineering Laboratory for High-Efficiency Recovery of Refractory Nonferrous Metals School of Metallurgy and EnvironmentCentral South UniversityChangsha 410083HunanChina Zhejiang Power New Energy Co. LTDZhuji 311800ZhejiangChina

Advancing high-voltage stability of layered sodium-ion oxides represents a pivotal avenue for their progress in energy storage applications.Despite this,a comprehensive understanding of the mechanisms underpinning their structural deterioration at elevated voltages remains insufficiently explored.In this study,we unveil a layer delamination phenomenon of Na_(0.67)Ni_(0.3)Mn_(0.7)O_(2)(NNM)within the 2.0-4.3 V voltage,attributed to considerable volumetric fluctuations along the c-axis and lattice oxygen reactions induced by the simultaneous Ni^(3+)/Ni^(4+)and anion redox reactions.By introducing Mg doping to diminished Ni-O antibonding,the anion oxidation-reduction reactions are effectively mitigated,and the structural integrity of the P2 phase remains firmly intact,safeguarding active sites and precluding the formation of novel interfaces.The Na_(0.67)Mg_(0.05)Ni_(0.25)Mn_(0.7)O_(2)(NMNM-5)exhibits a specific capacity of100.7 mA h g^(-1),signifying an 83%improvement compared to the NNM material within the voltage of2.0-4.3 V.This investigation underscores the intricate interplay between high-voltage stability and structural degradation mechanisms in layered sodium-ion oxides.

关键词： Soidum ion batteries Layer cathode materials P-type High-voltage performance Degradation analysis

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Accumulated-state-error-based event-triggered sampling scheme and its application to H∞ control of sampled-data systems

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Science China(Information Sciences) 2024年第6期67卷 328-341页

作者： Xian-ming ZHANG Qing-Long HAN bao-Lin ZHANG Xiaohua GE Dawei ZHANG School of Science Computingand Engineering TechnologiesSwinburne University of Technology College of Automation and Electronic Engineering Qingdao University of Science & Technology School of Mathematics Shandong University

This paper is concerned with event-triggered H∞control of sampled-data systems. Its novelties lie in three aspects:（i） A novel accumulated-state-error-based event-triggered scheme is introduced by comparing the integral of the state error from tk to t with the system state sampled at tk. This condition works well due to the fact that the so-called Zeno behaviour does not occur.（ii） A novel Lyapunov functional is constructed to establish a criterion to ensure some certain H∞performance of the closed-loop system. This Lyapunov functional is dependent on the integral of the state error involved in the event-triggered scheme.（iii） Under the event-triggered sampling scheme, suitable state-feedback controllers can be designed rather than be given a priori. Moreover, a self-triggered implementation of the proposed event-triggered sampling scheme is presented as well. Finally, a batch reactor model and an inverted pendulum system are given to demonstrate the effectiveness of the proposed method.

关键词： event-triggered sampling self-triggered implementation $H_\infty$ control looped-functional

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