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PACS gene family-related neurological diseases: limited genotypes and diverse phenotypes

World Journal of Pediatrics 2024年第1期20卷 82-91页

作者： Han zhang Kai Gao Shuang Wang yue-Hua zhang Zhi-xian Yang Ye Wu Yu-Wu Jiang Department of Pediatrics Peking University First Hospital.No.1 Xi'an Men StreetWest DistrictBeijing 100034China Bejing Key Laboratory of Molecular Diagnosis and Study on Pediatric Genetic Diseases BeijingChina Children Epilepsy Center Peking University First HospitalBeijingChina Key Laboratory for Neuroscience Ministry of Education/National Health and Family Planning CommissionPeking UniversityBeijingChina Center of Epilepsy Beijing Institute for Brain DisordersBeijingChina

Background The PACS gene family has been demonstrated to be related to intracellular vesicular *** phenotypic manifestations caused by the pathogenic variants of PACS include epilepsy,intellectual disability/developmental delay,and malformations,such as facial *** We identified seven new cases with pathogenic or likely pathogenic PACS variants using next-generation *** information obtained from these patients was analyzed along with that obtained from previously reported *** With the inclusion of the newly diagnosed cases in this study,103 cases with PACS gene family-related neurological diseases were reported,of which 43 were PACS2-related cases and the remaining were PACSI-related *** patients had seizures,which have been reported to be effectively controlled by several types of anti-seizure medications(ASMs).The most efficacious and frequently prescribed ASMs included sodium valproate(43.3%,13/30),oxcarbazepine/carbamazepine(26.7%,8/30),and levetiracetam(20%,6/30).Almost all patients had intellectual disability/developmental *** most common pathogenic missense variants were PACSI ***203Trp and PACS2 *** addition,we report a patient carrying a likely pathogenic copy number variation(CNV)(de novo heterozygous deletion of chr14:105821380-106107443,286 kilobase,destroyed part of the furin-binding region domain and the protein structure after it)with more severe and refractory late-onset *** The clinical phenotypes of the different PACS heterozygous missense variants were *** pathogenic variant sites of PACSI and PACS2 were quite limited but located in different regions.A CNV destroying part of the PACS2 gene might also be *** findings may provide an important clue for further functional studies on the pathogenic mechanism of neurological disorders related to the PACS gene family.

关键词： Developmental delay Epilepsy Intellectual disability PACSI PACS2

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Highly efficient catalyst for 1,1,2-trichloroethane dehydrochlorination via BN_(3) frustrated Lewis acid-base pairs

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Nano Research 2024年第6期17卷 4773-4781页

作者： Yuxue yue Fangmin Zuo Bolin Wang Xiaoling xian Jun Tang Haifeng zhang Zilong zhang Qingping Ke Wei Chen School of Chemical Engineering Northeast Electric Power UniversityJilin 132012China Industrial Catalysis Institute of Zhejiang University of Technology Hangzhou 310014China Key Laboratory of Carbon Materials of Zhejiang Province College of Chemistry and Materials EngineeringWenzhou UniversityWenzhou 325035China College of Chemistry and Chemical Engineering Anhui University of TechnologyMaanshan 243002China

In this study,a novel non-metallic carbon-based catalyst co-doped with boron and nitrogen(B,N)was successfully *** precisely controlling the carbonization temperature of a binary mixed ionic liquid,we selectively modified the doping site structure,ultimately constructing a B,N co-doped frustrated Lewis acid-base pair *** catalyst exhibited remarkable catalytic activity,selectivity,and stability in the dehydrochlorination reaction of 1,1,2-trichloroethane(TCE).Detailed characterization and theoretical calculations revealed that the primary active center of this catalyst was the BN_(3)*** to conventional graphitic N structures,the BN_(3)structure had a higher p-band center,ensuring superior adsorption and activation capabilities for TCE during the *** the BN_(3)site,three negatively charged nitrogen atoms acted as Lewis bases,while positively charged boron atoms acted as Lewis *** synergistic interaction facilitated the specific dissociation of chlorine and hydrogen atoms from TCE,significantly enhancing the 1,1-dichloroethene *** this research,we not only explored the active site structure and catalytic mechanism of B,N co-doped catalysts in depth but also provided an efficient,selective,and stable catalyst for the dehydrochlorination of TCE,contributing significantly to the development of non-metallic catalysts.

关键词： B N co-doped sites dehydrochlorination frustrated Lewis acid-base pairs catalytic mechanism

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Full-domain collaborative deployment method of multiple interference sources and evaluation of its deployment effect

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Defence Technology（防务技术） 2024年第2期32卷 572-595页

作者： yue Wang Fuping Sun xian Wang Jinming Hao Kai Xiao School of Geospatial Information The PLA Information Engineering UniversityZhengzhou 450001HenanChina

This paper realizes the full-domain collaborative deployment of multiple interference sources of the global satellite navigation system(GNSS)and evaluates the deployment effect to enhance the ability to disturb the attacker and the capability to defend the GNSS during navigation *** evaluation indicators for the jamming effect of GNSS suppressive and deceptive jamming sources are first created,their evaluation models are built,and their detection procedures are sorted out,as the basis for determining the deployment *** principles for collaboratively deploying multi-jamming sources are developed to obtain the deployment structures(including the required number,structures in demand,and corresponding positions)of three single interference sources required by collaboratively ***,simulation and hardware-in-loop testing results are presented to determine a rational configuration of the collaborative deployment of multi-jamming sources in the set situation and further realize the full-domain deployment of an interference network from ground,air to *** evaluation indices for the deployment effect are finally developed to evaluate the deployment effect of the proposed configuration and further verify its reliability and rationality.

关键词： Jamming effect Multiple interference sources Collaborative deployment Effect evaluation Defense capability

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Stabilization/solidification mechanisms of tin tailings and fuming slag-based geopolymers for different heavy metals

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Frontiers of Environmental Science & Engineering 2024年第5期18卷 43-55页

作者： xian Zhou Zhengfu zhang Hui Yang School of Materials Science and Engineering Kunming University of Science and TechnologyKunming650093China Kunming Metallurgical Research Institute Co. Ltd.Kunming650031China

Tin mine tailings(TMT)and fuming slag(FS)contain many heavy metals(As,Cr,Cu,Zn and Mn)that cause severe pollution to the ***,geopolymers were prepared using TMT,FS and flue gas desulfurization gypsum(FGDG)to immobilize heavy metals,and their compressive strength and heavy metal leaching toxicity were *** was first determined that T4F5(TMT:FS=4:5)sample exhibited the highest compressive strength(7.83 MPa).T4F5 achieved 95%immobilization efficiency for As and Cr,and nearly 100%for Cu,Zn and Mn,showing good immobilization performance.A series of characterization analyses showed that heavy metal cations can balance the charge in the geopolymer and replace Al in the geopolymer structure to form covalent *** addition,about 2%–20%of heavy metal Fe was immobilized in hydration products,heavy metal hydroxides and non-bridging Si–O and Al–O coordination with silica-aluminate ***_(3)^(3−) was oxidized into AsO_(4)^(3−),which may form Ca–As or Fe–As ***_(2)O_(7)^(2−)was converted to CrO_(4)^(2−)under alkaline environment and then combined with OH−to form Cr(OH)3 ***^(2+)may react directly with dissolved silicate to form Mn_(2)SiO_(4) and also form Mn(OH)_(2) *** unstable Mn(OH)_(2) can be further oxidized to MnO_(2).The heavy metal cations were immobilized in the silicoaluminate lattice,while the anions tended to form insoluble *** results may benefit the industry and government for better handling of TMT,FS and solid wastes containing the abovementioned five heavy metals.

关键词： Heavy metals Cementitious materials Tin tailings Stabilization/solidification Redox

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Impact of comorbid subthreshold depressive symptoms on cancerrelated fatigue and complications in adults with leukemia

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World Journal of Psychiatry 2024年第7期14卷 1009-1016页

作者： yue-xian Liu Juan Wang Department of Hematology Central Hospital Affiliated to Shandong First Medical UniversityJinan 250013Shandong ProvinceChina

BACKGROUND Patients not only experience symptoms caused by cancer but also suffer from the accompanying psychological ***,these patients do not have high quality of *** to the World Health Organization,the incidence of leukemia in China in 2020 was 5.1/100000,the mortality rate was 3.3/100000,and the prevalence rate was 16.7/***,it is important to examine the influence of comorbid subthreshold depressive symptoms on leukemia *** To determine the impact of comorbid subthreshold depressive symptoms on cancer-related fatigue and complications in leukemia patients,thereby providing a basis for early diagnosis and treatment in clinical *** A questionnaire survey was conducted among leukemia patients admitted to a tertiary hospital in Xi'an,Shaanxi Province,China,from August 2022 to December *** with a score>16 on the Chinese Classification of Mental Disorders(CCMD-3)and a Hamilton Depression Rating Scale score of 8-17 were classified as the subthreshold depressive group(n=95),while 100 leukemia patients admitted during the same period were classified as the control *** were collected using Epidata 3.1 software,and comparisons were made between the two groups regarding general clinical data,the Piper Fatigue Scale(PFS),the Pittsburgh Sleep Quality Index(PSQI),the Numeric Rating Scale for pain assessment,laboratory indicators,and the occurrence of *** In this survey,120 leukemia patients with depression were preliminarily screened,95 patients with subthreshold depression were ultimately selected as the subthreshold depression group,and 100 leukemia patients admitted during the same period were enrolled as the normal *** of basic clinical data between the two groups revealed no significant differences in age,sex,body mass index,cognitive function,or comorbidity with other chronic ***,there were statistically significant differences in the use of radiotherapy

关键词： Subthreshold depression Leukemia Cancer-related fatigue Complications Minor depression

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Product Form Solution of a Queuing-Inventory System with Lost Sales and Server Vacation

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Journal of Systems Science & Complexity 2024年第2期37卷 729-758页

作者： yue Dequan zhang Yuying XU Xiuli yue Wuyi School of Science Yanshan UniversityQinhuangdao 066004China School of Economics and Management Yanshan UniversityQinhuangdao 066004China The Kyoto College of Graduate Studies for Informatics Kyoto City 606-8225Japan

In this study,the authors consider an M/M/1 queuing system with attached inventory under an(s,S)control *** server takes multiple vacations whenever the inventory is *** is assumed that the lead time and the vacation time follow exponential *** authors formulate the model as a quasi-birth-and-dearth(QBD)process and derive the stability condition of the ***,the stationary distribution in product form for the joint process of the queue length,the inventory level,and the server’s status is ***,the conditional distributions of the inventory level when the server is on and operational,and when it is off due to a vacation,are *** the stationary distribution,the authors obtain some performance measures of the *** authors investigate analytically the effect of the server’s vacation on the performance ***,several numerical examples are presented to investigate the effects of some parameters on the performance measures,the optimal policy,and the optimal cost.

关键词： Cost function multiple vacations product form solution queuing-inventory system (s S)control policy

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Recent advances in vacancy engineering for reliable lithiumsulfur batteries

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Rare Metals 2024年第2期43卷 455-477页

作者： Wen-Dong Liu Xiao Tang Jun-An Feng Chao-yue zhang Hao Liu Chuan Shi Xiao-xian Zhao Jian-Jun Song College of Physics School of Chemistry and Chemical EngineeringQingdao UniversityQingdao266071China Faculty of Science Centre for Clean Energy TechnologySchool of Mathematical and Physical SciencesUniversity of Technology SydneySydneyNSW2007Australia Department of Chemistry College of ScienceHebei Agricultural UniversityBaoding071001China

The high-energy-density and low-cost features endow lithium-sulfur batteries with broad application ***,many drawbacks,especially the detrimental shuttle effect,have hindered the further development of *** response,a lot of new structures have been applied to suppress the shuttle effect and promote the development of ***,vacancy engineering has gained the attention of researchers due to its unique electronic *** review aims to analyze the application of vacancy engineering in ***,the electrochemistry of LSBs has been systematically discussed and the existing challenges as well as improvement tactics of LSBs have also been ***,the preparation methods and characterization technologies of various vacancies are summarized,including oxygen vacancies,sulfur vacancies,selenium vacancies,other anion vacancies,cation vacancies,*** latest applications of vacancy engineering in LSBs are also summarized in this ***,some prospects and insights for further investigation and practical application of vacancy engineering in LSBs are put forward.

关键词： Lithium-sulfur batteries(LSBs) Defects Vacancy engineering Electronic structure Shuttle effect

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Immobilizing Triphenylamine with Photoredox Inert Sr^(2+)Forming Sr-MOF with Controlled Electron Migration for Photocatalytic Oxidation of Thiols to Disulfides

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Chinese Journal of Chemistry 2024年第10期42卷 1093-1099页

作者： Ying-Xia Wang Fang Zheng Dong-Xia Song Bao-yue Niu Lu-Qian Deng xian-Ming zhang Key Laboratory of Magnetic Molecules and Magnetic Information Materials(Ministry of Education) Schoolof Chemistry and Materials ScienceShanxi Normal UniversityTaiyuanShanxi 030006China Key Laboratory of Interface Science and Engineering in Advanced Material(Ministry of Education) College of ChemistryTaiyuan University of TechnologyTaiyuanShanxi 030024China

The photocatalytic oxidative coupling of thiols is one of the most popular methods to synthesize the *** and its derivatives(TPAs)are promising for the above reaction,but suffer from the easy polymerization and difficult *** overcome these obstacles while controlling the photogenerated electrons transfer directly to target substrates,herein,we constructed one TPA-based metal-organic framework(MOF),(Me_(2)NH_(2))[Sr(TCBPA)]·DMA·3H_(2)O(1),by direct self-assembly of tris(4′-carboxybiphenyl)amine(H_(3)TCBPA)and photoredox inert strontium ion(Sr^(2+)).DFT calculations revealed that the valence band maximum(VBM)and the conduction band minimum(CBM)are mainly located on TCBPA^(3-),successfully inhibiting the undesirable electron migration to metal *** results indicated that 1 displays superior performance than homogeneous H_(3)TCBPA,which may result from the abundant π…π and C—H…π interactions between the well-arranged TCBPA^(3-)and the build-in electric field between the anionic framework and the Me_(2)NH^(2+).This work highlights that immobilizing TPAs into MOFs is one promising approach to designing heterogeneous photocatalysts for the synthesis of disulfides by oxidative coupling of thiols.

关键词： Triphenylamine Metal-organic frameworks Strontium Photooxidation Thiols Disulfides

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Three-dimensional porous bimetallic metal–organic framework/gelatin aerogels: A readily recyclable peroxymonosulfate activator for efficient and continuous organic dye removal

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Chinese Journal of Chemical Engineering 2024年第7期71卷 193-202页

作者： Wenlong xiang xian zhang Rou Xiao Yanhui zhang College of Chemistry Chemical Engineering and EnvironmentFujian Province Key Laboratory of Morden Analytical Science and Separation TechnologyMinnan Normal UniversityZhangzhou 363000China Fujian Provincial University Key Laboratory of Pollution Monitoring and Control Minnan Normal UniversityZhangzhou 363000China

As promising catalysts for the degradation of organic pollutants,metal–organic frameworks(MOFs)often face limitations due to the particle agglomeration and challenging recovery in liquid-catalysis application,stemming from their powdery *** macroscopic structures from pulverous MOF is thus of great importance for broadening their practical *** this study,three-dimensional porous MOF aerogel catalysts were successfully fabricated for degrading organic dyes by activating peroxymonosulfate(PMS).MOF/gelatin aerogel(MOF/GA)catalysts were prepared by directly integrating bimetallic FeCo-BDC with gelatin solutions,followed by freeze-drying and low-temperature *** FeCo-BDC-0.15/GA/PMS system exhibited remarkable performance in degrading various organic dyes,eliminating 99.2%of rhodamine B within a mere 5 *** to the GA/PMS system,there was over a 300-fold increase in the reaction rate ***,high removal efficiency was maintained across varying conditions,including different solution pH,co-existing inorganic anions,and natural water *** trapping experiments and electron paramagnetic resonance analysis revealed that the degradation involved radical(SO_(4)^(-)·)and non-radical routes(^(1)O_(2)),of which ^(1)O_(2) was ***,even after a continuous 400-min reaction in a fixed-bed reactor at a liquid hourly space velocity of 27 h^(-1),the FeCo-BDC/GA composite sustained a degradation efficiency exceeding 98.7%.This work presents highly active MOF-gelatin aerogels for dye degradation and expands the potential for their large-scale,continuous treatment application in organic dye wastewater management.

关键词： Catalyst Environment Wastewater Metal–organic framework Gelatin aerogel Peroxymonosulfate

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Factors influencing Frey syndrome after parotidectomy with acellular dermal matrix

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World Journal of Clinical Cases 2024年第9期12卷 1578-1584页

作者： xian-Da Chai Huan Jiang Ling-Ling Tang Jing zhang Long-Fei yue Department of Oral and Maxillofacial Surgery People’s Hospital of AnshunAnshun 561000Guizhou ProvinceChina Department of General Practice People’s Hospital of AnshunAnshun 561000Guizhou ProvinceChina

BACKGROUND Frey syndrome,also known as ototemporal nerve syndrome or gustatory sweating syndrome,is one of the most common complications of parotid gland *** condition is characterized by abnormal sensations in the facial skin accompanied by episodes of flushing and sweating triggered by cognitive processes,visual stimuli,or *** To investigate the preventive effect of acellular dermal matrix(ADM)on Frey syndrome after parotid tumor resection and analyzed the effects of Frey syndrome across various surgical methods and other factors involved in parotid tumor *** Retrospective data from 82 patients were analyzed to assess the correlation between sex,age,resection sample size,operation time,operation mode,ADM usage,and occurrence of postoperative Frey *** Among the 82 patients,the incidence of Frey syndrome was 56.1%.There were no significant differences in sex,age,or operation time between the two groups(P>0.05).However,there was a significant difference between ADM implantation and occurrence of Frey syndrome(P<0.05).ADM application could reduce the variation in the incidence of Frey syndrome across different operation *** ADM can effectively prevent Frey syndrome and delay its onset.

关键词： Parotid gland tumor Frey syndrome Acellular dermal matrix Acellular allogenic dermal matrix

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