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Chinese patients with adult onset leukodystrophy caused by CST3 variants

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Journal of Genetics and Genomics 2024年第7期51卷 778-780页

作者： Yi Zhang zhi-Ying wu Department of Medical Genetics and Center for Rare Diseases Zhejiang Key Laboratory of Rare Diseases for Precision Medicine and Clinical TranslationSecond Affiliated HospitalZhejiang University School of MedicineHangzhouZhejiang 310009China Nanhu Brain-computer Interface Institute HangzhouZhejiang 314050China Department of Neurology Second Affiliated HospitalZhejiang University School of MedicineHangzhouZhejiang 310009China MOE Frontier Science Center for Brain Science and Brain-machine Integration School of Brain Science and Brain MedicineZhejiang UniversityHangzhouZhejiang 310012China

Leukodystrophies represent a group of cerebral white matter disorders that mainly affect axon-glia *** disorders are clinically diverse and display significant genetic *** is challenging to differentiate hereditary leukodystrophies,particularly those that present in adulthood,from acquired leukodystrophies and other genetic disorders,such as multiple sclerosis(MS)or hereditary spastic paraplegia(HSP)(Wei et al.,2021).Over the past few decades,a series of causative genes associated with leukodystrophies have been identified(Kohler et al.,2018).Nevertheless,a significant proportion of patients still lack a precise molecular diagnosis.

关键词： al. patients Kohler

Selection Method for New Energy Output Guaranteed Rates Considering Optimal Energy Storage Configuration

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CSEE Journal of Power and Energy Systems 2024年第2期10卷 539-547页

作者： Xin cheng Tong wu Wei Yao Yan Yang Guangdong Power Grid Corporation Guangzhou 510620China State Key Laboratory of Advanced Electromagnetic Engineering and Technology and School of Electrical and Electronic EngineeringHuazhong University of Science and TechnologyWuhan 430074China

The output of new energy represented by wind power and photovoltaic power features volatility and randomness. It is a practical approach to use the guaranteed rate with statistical characteristics to analyze the output coefficient of new energy. However, there is a lack of analysis and demonstration on the value of the new energy output guaranteed rate. To solve this problem, a selection method for the new energy output guaranteed rate considering optimal energy storage configuration is proposed. First, the influence of the new energy output guaranteed rate on the new energy output coefficient is analyzed. Secondly, with the goal of minimizing the comprehensive costs, an optimal configuration model of energy storage based on the output guaranteed rate of new energy is constructed, and particle swarm optimization is used for the optimization solution. Then, according to the optimal energy storage configuration results under different guaranteed rates, a reasonable new energy output guaranteed rate is determined by maximizing the comprehensive benefits obtained from energy storage. Finally, an example applied to the power grid in an area of Guangdong in Southern China is provided to illustrate the effectiveness of the proposed method.

关键词： Energystorage optimization guaranteed output guaranteed rate new energy consumption

Capillary Property of Entangled Porous Metallic Wire materials and Its Application in Fluid Buffers:Theoretical Analysis and Experimental Study

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Defence Technology（防务技术） 2024年第1期31卷 400-416页

作者： Yu Tang Yiwan wu Hu cheng Rong Liu Engineering Research Center for Metal Rubber School of Mechanical Engineering and AutomationFuzhou UniversityFuzhou 350156China AECC Guizhou Liyang Aviation Power Co. LTDGuiyang 550014China College of Computer and Information Sciences Fujian Agriculture and Forestry UniversityFuzhou 350156China

Strong impact does serious harm to the military industries so it is necessary to choose reasonable cushioning material and design effective buffers to prevent the impact of *** on the capillary property entangled porous metallic wire materials(EPMWM),this paper designed a composite buffer which uses EPMWM and viscous fluid as cushioning materials under the low-speed impact of the recoil force device of weapon equipment(such as artillery,mortar,etc.).Combined with the capillary model,porosity,hydraulic diameter,maximum pore diameter and pore distribution were used to characterize the pore structure characteristics of *** calculation model of the damping force of the composite buffer was *** low-speed impact test of the composite buffer was *** parameters of the buffer under low-speed impact were identified according to the model,and the nonlinear model of damping force was *** test results show that the composite buffer with EPMWM and viscous fluid can absorb the impact energy from the recoil movement effectively,and provide a new method for the buffer design of weapon equipment(such as artillery,mortar,etc.).

关键词： Entangled porous metallic wire materials Capillary property Viscousfluid Low-speed impact Damping force

A Hybrid Signal Processing Method Combining Mathematical Morphology and Walsh Theory for Power Quality Disturbance Detection and Classification

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CSEE Journal of Power and Energy Systems 2024年第2期10卷 584-592页

作者： zhi Ding Tianyao Ji Mengshi Li Q.H.wu School of Electric Power Engineering South China University of Technology(SCUT)Guangzhou 510641China

In this paper, a novel signal processing method combining mathematical morphology (MM) and Walsh theory is proposed, which uses Walsh functions to control the structuring element (SE) and MM operators. Based on the Walsh-MM method, a scheme for power quality disturbances detection and classification is developed, which involves three steps: denoising, feature extraction and morphological clustering. First, various evolution rules of Walsh function are used to generate groups of SEs for the multiscale Walsh-ordered morphological operation, so the original signal can be denoised. Next, the fundamental wave of the denoised signal is suppressed by Hadamard matrix;thus, disturbances can be extracted. Finally, the Walsh power spectrum of the waveform extracted in the previous step is calculated, and the parameters of which are taken by morphological clustering to classify the disturbances. Simulation results reveal the proposed scheme can effectively detect and classify disturbances, and the Walsh-MM method is less affected by noise and only involves simple calculation, which has a potential to be implemented in hardware and more suitable for real-time application.

关键词： Hadamard matrix mathematical morphology morphological clustering power quality disturbance Walsh theory

Wettability regulation from superhydrophilic to superhydrophobic via nanosecond laser ablation

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Science China(Technological Sciences) 2024年第6期67卷 1829-1841页

作者： CHEN zhi wu zhicheng ZHANG GuoJun LI DongHui HAN FengLin State Key Laboratory of Precision Manufacturing for Extreme Service Performance College of Mechanical and Electrical EngineeringCentral South UniversityChangsha410083China Guangdong Provincial Key Laboratory of Manufacturing Equipment Digitization Guangdong HUST Industrial Technology Research InstituteDongguan523808China China Harzone Industry Corp. Ltd.Wuhan430299China

Metal surfaces play a crucial role in numerous applications,from self-cleaning and anti-icing to anti-fogging and oil-water *** regulation of their wettability is essential to enhance their performance in these *** paper proposes a multi-state regulation method for metal surface wettability,leveraging nanosecond laser *** creating non-uniform microstructures on a metal surface,the contact area between the solid and liquid phases can be increased,resulting in the attainment of superhydrophilic properties(contact angle(CA),ranging from 4.6°to 8.5°).Conversely,the construction of uniform microstructures leads to a decreased solid-liquid contact area,thereby rendering the metal surface hydrophilic(CA=12.2°–53°).Furthermore,through heat treatment on a surface with uniform microstructures,organic matter adsorption can be promoted while simultaneously reducing surface *** process results in the metal surface acquiring hydrophobic properties(CA=92.1°–133.5°),facilitated by the“air cushion effect.”Building on the hydrophobic surface,stearic acid modification can further reduce surface energy,ultimately bestowing the metal surface with superhydrophobic properties(CA=150.1°–152.7°,and sliding angle=3.8°).Performance testing has validated the durability and self-cleaning effectiveness of the fabricated superhydrophobic surface while also highlighting the excellent anti-fog performance of the superhydrophilic *** findings strongly indicate the immense potential of these surfaces in various engineering applications.

关键词： nanosecond laser ablation surface wettability superhydrophobic superhydrophilic

Perforated gastric ulcer causing mediastinal emphysema: A case report

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World Journal of Clinical Cases 2024年第4期12卷 859-864页

作者： zhi-cheng Dai Xun-wu Gui Feng-He Yang Hao-Yuan Zhang Wen-Feng Zhang Department of General Surgery Mengcheng County First People's HospitalMengcheng 233500Anhui ProvinceChina Department of Medical Imaging Mengcheng County First People's HospitalMengcheng 233500Anhui ProvinceChina

BACKGROUND Mediastinal emphysema is a condition in which air enters the mediastinum between the connective tissue spaces within the pleura for a variety of *** can be spontaneous or secondary to chest trauma,esophageal perforation,medi-cally induced factors,*** common symptoms are chest pain,tightness in the chest,and respiratory *** mediastinal emphysema patients have mild symptoms,but severe mediastinal emphysema can cause respiratory and circulatory failure,resulting in serious *** SUMMARY A 75-year-old man,living alone,presented with sudden onset of severe epigastric pain with chest tightness after drinking *** to the remoteness of his residence and lack of neighbors,the patient was found by his nephew and brought to the hospital the next morning after the disease *** tomography(CT)showed free gas in the abdominal cavity,mediastinal emph-ysema,and subcutaneous *** gastrointestinal angiography showed that the esophageal mucosa was intact and the gastric antrum was ***,we chose to perform open gastric perforation repair on the patient under thoracic epidural anesthesia combined with intravenous *** operative incision of the muscle layer of the patient's abdominal wall was made,and a large amount of subperitoneal gas was *** a continued incision of the peritoneum revealed the presence of a perforation of approx-imately 0.5 cm in the gastric antrum,which we repaired after pathological ***,the patient received high-flow oxygen and cough *** CT was performed on the first and sixth postoperative days,and the mediastinal and subcutaneous gas was gradually *** After gastric perforation,a large amount of free gas in the abdominal cavity can reach the mediastinum through the loose connective tissue at the esophageal hiatus of the diaphragm,and upper gastrointestinal angiography can clarify the site of *** pat

关键词： Gastric ulcer Perforated Mediastinal emphysema Case report

Si-wu-Tang attenuates liver fibrosis via regulating lncRNA H19-dependent pathways involving cytoskeleton remodeling and ECM deposition

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Chinese Journal of Natural Medicines 2024年第1期22卷 31-46页

作者： QU Jiaorong XUE Xiaoyong WANG zhixing MA zhi JIA Kexin LI Fanghong ZHANG Yinhao wu Ruiyu ZHOU Fei ZHAO Piwen LI Xiaojiaoyang School of Life Sciences Beijing University of Chinese MedicineBeijing 100029China School of Chinese Materia Medica Beijing University of Chinese MedicineBeijing 100029China State Key Laboratory of Medical Molecular Biology Department of Molecular Biology and BiochemistryInstitute of Basic Medical SciencesChinese Academy of Medical Sciences and Peking Union Medical CollegeBeijing 100005China

Liver fibrosis is a dynamic wound-healing response characterized by the agglutination of the extracellular matrix(ECM).Si-wu-Tang(SWT),a traditional Chinese medicine(TCM)formula,is known for treating gynecological diseases and liver *** previous studies demonstrated that long non-coding RNA H19(H19)was markedly upregulated in fibrotic livers while its deficiency markedly reversed ***,the mechanisms by which SWT influences H19 remain ***,we established a bile duct ligation(BDL)-induced liver fibrosis model to evaluate the hepatoprotective effects of SWT on various cells in the *** results showed that SWT markedly improved ECM deposition and bile duct reactions in the ***,SWT relieved liver fibrosis by regulating the transcription of genes involved in the cytoskeleton remodeling,primarily in hepatic stellate cells(HSCs),and influencing cytoskeleton-related angiogenesis and hepatocellular *** modulation collectively led to reduced ECM *** extensive bioinformatics analyses,we determined that H19 acted as a miRNA sponge and mainly inhibited miR-200,miR-211,and let7b,thereby regulating the above cellular regulatory ***,SWT reversed H19-related miRNAs and signaling pathways,diminishing ECM deposition and liver ***,these protective effects of SWT were diminished with the overexpression of H19 in *** conclusion,our study elucidates the underlying mechanisms of SWT from the perspective of H19-related signal networks and proposes a potential SWT-based therapeutic strategy for the treatment of liver fibrosis.

关键词： Liver fibrosis LncRNA H19 Si-wu-Tang miRNAs Cytoskeleton ECM deposition

Fault-tolerant identity-based encryption from SM9

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Science China(Information Sciences) 2024年第2期67卷 104-117页

作者： Xiaohong LIU Xinyi HUANG Zhaohui cheng Wei wu Fujian Provincial Key Laboratory of Network Security and Cryptology College of Computer and Cyber SecurityFujian Normal University Artificial Intelligence Thrust Information HubThe Hong Kong University of Science and Technology (Guangzhou) Olym Information Security Technology Ltd. Center for Applied Mathematics of Fujian Province School of Mathematics and StatisticsFujian Normal University

This paper initiates the formal study of attribute-based encryption within the framework of SM9,the Chinese National Cryptography Standard for Identity-Based Cryptography, by presenting two new faulttolerant identity-based encryption(FIBE) schemes. Our first scheme uses the same private-key/ciphertext structure as the original SM9 algorithm and operates in a small attribute universe. As a result, it can be effectively and smoothly integrated into the information systems using SM9. In the random oracle model,we prove that our scheme is ciphertext-indistinguishable against fuzzy selective-identity and chosen-plaintext attacks under the(k + 3)-DBDHI assumption. Our second design is a large universe FIBE scheme based on SM9 that is ciphertext-indistinguishable against chosen-plaintext attacks in the random oracle model under the(f, g)-GDDHE assumption. Finally, we compare the communication and computing costs of our schemes to those of other classical ones. The comparison shows that our schemes have comparable performance as others. We believe that our findings will accelerate the applications of SM9 in modern information systems such as cloud computing and blockchain.

关键词： attribute-based encryption identity-based encryption fault-tolerant SM9

Recent advances in post-chiroptical manipulation of supramolecular aggregates assembled with molecular modules

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Science China Chemistry 2024年第9期67卷 2842-2863页

作者： Yanling Shen Wanhua wu zhipeng Yu cheng Yang Key Laboratory of Green Chemistry&Technology of Ministry of Education College of Chemistry InstitutionSichuan UniversityChengdu 610064China

Recently,various supramolecular assembly systems have been identified where the pre-existing chiral supramolecular structure can be manipulated through environmental adjustments,such as temperature,solvent,and *** changes result in diverse microscopic morphologies during the assembly process and induce chiroptical variations,particularly in circular dichroism(CD)and circularly polarized luminescence(CPL)*** chiroptical properties serve as direct indicators of the chiral supramolecular structure and hold promise for applications in sensing,molecular recognition,asymmetric synthesis,and material *** review focuses on research published in the last five years,highlighting instances where assembled structures aggregated by small organic molecular modules demonstrate in-situ chiroptical changes through external condition *** categorizes and discusses different modes of regulation,aiming to offer insights and guidelines for exploring dynamic changes in assembly processes and designing in-situ adjustable structures of assembly modules.

关键词： supramolecular assemblies post-chiroptical manipulation in-situ structural changes

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