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Ketogenic diet therapy in children with epilepsy caused by SLC2A1 mutations:a single-center single-arm retrospective study

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World Journal of Pediatrics 2024年第5期20卷 517-524页

作者： ying-yan Wang yun-Qing Zhou Li-Juan Luo Cui-Jin Wang Nan Shen Hao Li Ji-wen Wang Department of Neurology Shanghai Children’s Medical CenterSchool of MedicineShanghai Jiao Tong UniversityShanghaiChina Department of Infectious Diseases Shanghai Children’s Medical CenterSchool of MedicineShanghai Jiao Tong UniversityShanghaiChina Clinical Research Ward Clinical Research CenterShanghai Children’s Medical CenterSchool of MedicineShanghai Jiao Tong UniversityShanghaiChina

Background This retrospective study assessed the efficacy and safety of ketogenic diet therapies in children with epilepsy caused by SLC2A1 genetic mutations and glucose transporter type 1 deficiency *** Pediatric patients with epilepsy symptoms admitted to our medical center between January 2017 and October 2021 were included if they presented with an SLC2A1 genetic mutation on whole-exome *** analyzed the patients’convulsions and treatment with antiepileptic *** patients were followed up at different time periods after ketogenic diet *** Six patients with SLC2A1 mutations were included in this *** patients had seizures of different types and frequencies,and they took antiepileptic drugs to relieve their *** were then treated with a ketogenic diet for at least four *** analyzed epilepsy control rates at 1,2,3,6,and 12 months after ketogenic diet *** patients were seizure-free within a month of receiving the diet *** patients were followed up for six months,three were followed up for 12 months after the treatment,and there was no recurrence of epilepsy during this *** antiepileptic drug withdrawal,none of the patients experienced seizure relapse when receiving ketogenic diet treatment *** severe adverse events occurred during the *** Ketogenic diet therapy is very effective and safe for the treatment of epilepsy caused by SLC2A1 ***,patients with glucose transporter type 1 deficiency syndrome caused by SLC2A1 mutations should begin ketogenic diet treatment as soon as possible.

关键词： Epilepsy Glucose transporter type 1 Ketogenic diet SLC2A1

Diversification of interfaces triggered hydrogen storage properties enhancement

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Rare Metals 2024年第5期43卷 2324-2330页

作者： Jia-xin Zhang xin Ding Rui-Run Chen xiang-Feng Ma wen-Chao Cao yong Zhang Jing-Jie Guo National Key Laboratory for Precision Hot Processing of Metals Harbin Institute of TechnologyHarbin 150001China

Dual effects of grain refinement and alloying are achieved and the mechanism of"diverse interfaces reinforcement"for hydrogen storage Mg alloys is first *** interface reinforced Mg-y-Zn-Al alloy for hydrogen storage is *** work figures out that the adventurous Al-rich phase exhibits an ordered face-centered cubic(FCC)structure with composition of(31±2)at%y-(28±1)at%Zn-(41±3)at%Al,i.e.,y_(3)Zn_(3)Al_(4),and an incoherent interface between y3Zn3Al4phase and Mg substrate is observed.

关键词： hydrogen alloying alloy

Synergy of inside doped metals–Outside coated graphene to enhance hydrogen storage in magnesium-based alloys

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Journal of Magnesium and Alloys 2024年第6期12卷 2462-2471页

作者： Kun Zhang yu Chang Jingjing Lei Jing Chen Tingzhi Si xiaoli Ding Ping Cui Hai-wen Li Qingan Zhang yongtao Li School of Materials Science and Engineering&Low-Carbon New Materials Research Center Anhui University of TechnologyMaanshanAnhui 243002China Institute of Nuclear and New Energy Technology Tsinghua UniversityBeijing100084 Hefei General Machinery Research Institute HefeiAnhui 230031China Key Laboratory of Green Fabrication and Surface Technology of Advanced Metal Materials of Ministry of Education Anhui University of TechnologyMaanshan 243002China

Grain growth of magnesium(Mg)and its hydride is one of the main reasons for kinetic and capacity degradation during the hydrogen absorption and desorption *** solve this problem,herein we propose a novel method involving synergistic effect of inside embedded metals and outside coated graphene to limit the growth of Mg and its hydride *** graphene coated Mg-y-Al alloys were selected as a model system for demonstrating this positive effect where the Mg_(91)y_(3)Al_(6)alloy was first prepared by rapidly solidified method and then high-pressure milled with 5 wt%graphene upon 5 MPa hydrogen gas for obtaining in-situ formed yAl_(2)and yH_(3)embedded in the MgH_(2)matrix with graphene shell(denoted as MgH_(2)-y-Al@GR).In comparison to pure MgH_(2),the obtained MgH_(2)-y-Al@GR composites deliver much better kinetics and more stable cyclic *** instance,the MgH_(2)-y-Al@GR can release about 6.1 wt%H_(2)within 30 min at 300℃ but pure MgH_(2)only desorbs∼1.5 wt%H_(2).The activation energy for desorption of MgH_(2)-y-Al@GR samples is calculated to be 75.3±9.1 kJ/mol that is much lower than approximately 160 kJ/mol for pure MgH_(2).Moreover,its capacity retention is promoted from∼57%of pure MgH_(2)to∼84%after 50th cycles without obvious particle agglomeration and grain *** synergistic effect of outside graphene coating with inside embedded metals which could provide a huge number of active sites for catalysis as well as inhibit the grain growth of Mg and its hydride is believed to be responsible for these.

关键词： Energy Hydrogen storage Mg alloys Synergy effect

Clinical application value of long non-coding RNAs signatures of genomic instability in predicting prognosis of hepatocellular carcinoma

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World Journal of Gastrointestinal Surgery 2024年第8期16卷 2386-2392页

作者： xiao-wen xing xiao Huang Wei-Peng Li Ming-Ke Wang Ji-Shun yang Department of Disease Control and Prevention Naval Medical CenterNaval Medical UniversityShanghai 200052China Medical Care Center Naval Medical UniversityShanghai 200052China

Hepatocellular carcinoma(HCC)presents challenges due to its high recurrence and metastasis rates and poor *** current clinical diagnostic and prognostic indicators exist,their accuracy remains imperfect due to their biol-ogical ***,there is a quest to identify improved biomarkers for HCC diagnosis and *** combining long non-coding RNA(lncRNA)expression and somatic mutations,Duan et al identified five representative lncRNAs from 88 lncRNAs related to genomic instability(GI),forming a GI-derived lncRNA signature(LncSig).This signature outperforms previously re-ported LncSig and TP53 mutations in predicting HCC *** this editorial,we comprehensively evaluate the clinical application value of such prognostic evaluation model based on sequencing technology in terms of cost,time,and ***,we provide an overview of various prognostic models for HCC,aiding in a comprehensive understanding of research progress in pro-gnostic evaluation methods.

关键词： Hepatocellular carcinoma Prognosis Prognostic model Biomarkers Genomic instability long non-coding RNA Clinical application value

RsaL is a self-regulatory switch that controls alternative biosynthesis of two AHL-type quorum sensing signals in Pseudomonas aeruginosa PA1201

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mLife 2024年第1期3卷 74-86页

作者： ya-wen He Zi-Jing Jin ying Cui Kai Song Bo Chen Lian Zhou State Key Laboratory of Microbial Metabolism Joint International Research Laboratory of Metabolic and Developmental SciencesSJTU-NLBP Joint R&D Centre for Biopesticides and BiofertilizersSchool of Life Sciences and BiotechnologyShanghai Jiao Tong UniversityShanghaiChina Zhiyuan Innovative Research Centre Student Innovation CentreZhiyuan CollegeShanghai Jiao Tong UniversityShanghaiChina

Pseudomonas aeruginosa is a ubiquitous and metabolically versatile microorganism naturally found in soil and *** is also an opportunistic pathogen in plants,insects,animals,and *** response to increasing cell density,*** uses two acylhomoserine lactone(AHL)quorum-sensing(QS)signals(i.e.,N-3-oxo-dodecanoyl homoserine lactone[3-oxo-C12-HSL]and Nbutanoyl-homoserine lactone[C4-HsL]),which regulate the expression of hundreds of ***,how the biosynthesis of these two QS signals is coordinated remains *** studied the regulation of these two QS signals in the rhizosphere strain ***1201 sequentially produced 3-oxo-C12-HSL and C4-HSL at the early and late growth stages,*** highest 3-oxo-C12-HSL-dependent elastase activity was observed at the early stage,while the highest C4-HSL-dependent rhamnolipid production was observed at the late *** atypical regulator RsaL played a pivotal role in coordinating 3-oxo-C12-HSL and C4-HSL biosynthesis and QS-associated *** repressed las/transcription by binding the-10 and-35 boxes of the lasl *** contrast,RsaL activated rhll transcription by binding the region encoding the 5'-untranslated region of the rhll ***,RsaL repressed its own expression by binding a nucleotide motif located in the-35 box of the rsaL ***,RsaL acts as a molecular switch that coordinates the sequential biosynthesis of AHL QS signals and differential virulence in ***,C4-HSL activation by RsaL was independent of the Las and Pseudomonas quinolone signal(PQS)QS signaling ***,we propose a new model of the QS regulatory network in PA1201,in which RsaL represents a superior player acting at the top of the hierarchy.

关键词： N-3-oxo-dodecanoyl homoserine lactone N-butanoyl-homoserine lactone Pseudomonas aeruginosa quorum sensing RsaL

我国木质家具制造行业VOCs排放特征及环境影响

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环境科学 2024年第8期45卷 4470-4483页

作者：刘文文邵霞滕巍北京市生态环境保护科学研究院国家城市环境污染控制工程技术研究中心城市大气挥发性有机物污染防治技术与应用北京市重点实验室北京100037 生态环境部环境工程评估中心北京100012

为研究木质家具制造行业挥发性有机化合物(VOCs)排放特征及其环境影响,选取9家典型的木质家具制造企业,对其涂装工序排气筒废气进行样品采集和VOCs检测,并通过最大增量反应活性法(MIR)和二次有机气溶胶(SOA)生成潜势法量化其对臭氧(O_(... 详细信息

为研究木质家具制造行业挥发性有机化合物(VOCs)排放特征及其环境影响,选取9家典型的木质家具制造企业,对其涂装工序排气筒废气进行样品采集和VOCs检测,并通过最大增量反应活性法(MIR)和二次有机气溶胶(SOA)生成潜势法量化其对臭氧(O_(3))和SOA的生成贡献.结果表明:①不同类型涂料排气筒VOCs排放浓度差异较大,溶剂型涂料排气筒VOCs排放浓度显著高于水性涂料和辐射固化(UV)涂料排气筒废气VOCs排放浓度,其ρ(VOCs)范围分别为2.82~155.37、1.13~104.45和0.57~1.15 mg·m^(-3);②溶剂型涂料排气筒废气中VOCs以酯类为主,质量分数高达45.88%,质量分数最高的VOCs物种为乙酸丁酯(31.07%);水性涂料和UV涂料排气筒废气中VOCs均以醇类为主,质量分数分别为47.04%和35.10%,且质量分数最高的VOCs物种均为乙醇,质量分数分别为46.63%和34.32%;③溶剂型涂料、水性涂料和UV涂料排气筒排放VOCs的OFP值分别为149.23、50.90和1.87 mg·m^(-3),不同类型涂料排气筒OFP的首要贡献组分分别为间/对-二甲苯(26.61%)、乙醇(36.35%)和乙醇(23.98%);④溶剂型涂料、水性涂料和UV涂料排气筒排放VOCs的SOA值分别为0.76、0.25和0.01 mg·m^(-3),对SOA生成贡献率最高的VOCs组分均为芳香烃(96.35%~98.96%),关键活性物种为甲苯、乙苯和二甲苯;(5)将溶剂型涂料、水性涂料和UV涂料排气筒废气对环境影响进行比较,发现溶剂型涂料排气筒排放的VOCs所产生的OFP与SOA远高于水性涂料和UV涂料,从源头上采用水性涂料和UV涂料替代溶剂型涂料可以大幅度降低VOCs排放浓度,并减少OFP和SOA的生成.

关键词：木质家具制造行业挥发性有机化合物(VOCs) 涂料类型排放特征环境影响

High-throughput discovery of highly selective reversible hMAO-B inhibitors based on at-line nanofractionation

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Acta Pharmaceutica Sinica B 2024年第4期14卷 1772-1786页

作者： yu Fan Jincai Wang Jingyi Jian yalei wen Jiahao Li Hao Tian Jacques Crommen Wei Bi Tingting Zhang Zhengjin Jiang Institute of Pharmaceutical Analysis/Guangdong Province Key Laboratory of Pharmacodynamic Constituents of TCM and New Drugs Research/International Cooperative Laboratory of Traditional Chinese Medicine Modernization and Innovative Drug Development of Ministry of Education(MOE)of China/College of Pharmacy Jinan UniversityGuangzhou 510632China KU Leuven-University of Leuven Pharmaceutical AnalysisDepartment of Pharmaceutical and Pharmacological SciencesLeuven 3000Belgium Laboratory of Analytical Pharmaceutical Chemistry Department of Pharmaceutical SciencesCIRMUniversity of LiegeLiege B-4000Belgium Department of Neurology the First Affiliated Hospital of Jinan University/Clinical Neuroscience Institutethe First Affiliated Hospital of Jinan UniversityGuangzhou 510630China

Human monoamine oxidase B(hMAO-B)has emerged as a pivotal therapeutic target for Parkinson's *** to adverse effects and shortage of commercial drugs,there is a need for novel,highly selective,and reversible hMAO-B inhibitors with good blood-brain barrier *** this study,a high-throughput at-line nanofractionation screening platform was established with extracts from Chuanxiong Rhizoma,which resulted in the discovery of 75 active compounds,including phenolic acids,volatile oils,and phthalides,two of which were highly selective novel natural phthalide hMAO-B inhibitors that were potent,selective,reversible and had good blood‒brain *** docking and molecular dynamics simulations elucidated the inhibition ***(IC_(50)=103 nmol/L;SI=645)and neocnidilide(IC_(50)=131 nmol/L;SI=207)demonstrated their excellent potential as hMAO-B *** offset the limitations of deactivating enzymes associated with irreversible hMAO-B inhibitors such as *** SH-Sy5y cell assays,sedanolide(EC_(50)=0.962μmol/L)and neocnidilide(EC_(50)=1.161μmol/L)exhibited significant neuroprotective effects,comparable to the positive drugs rasagiline(EC_(50)=0.896μmol/L)and safinamide(EC_(50)=1.079μmol/L).These findings underscore the potential of sedanolide as a novel natural hMAO-B inhibitor that warrants further development as a promising drug candidate.

关键词： Human monoamine oxidase B inhibitors High-throughput screening At-linenanofractionation Parkinson's disease Cellular neuroprotection Traditional Chinese medicines(TCMs)

A_(2)B_(7)-type La-Mg-Ni alloys prepared by Mg thermal diffusion for improved hydrogen storage performance

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Rare Metals 2024年第7期43卷 3260-3272页

作者： yong-xi Zhang Guan-Jiu Wu Jing Gu Hong-xing Kang yuan Li Di Zhou wen-Feng Wang Lu Zhang Shu-Min Han Hebei Key Laboratory of Applied Chemistry School of Environmental and Chemical EngineeringYanshan UniversityQinhuangdao 066004China Department of Chemistry and Biochemistry San Diego State UniversitySan DiegoCA92182-1030USA State Key Laboratory of Metastable Materials Science and Technology Yanshan UniversityQinhuangdao 066004China

A novel approach based on thermal diffusion was used to achieve controllable Mg content in A_(2)B_(7)-type La-Mg-Ni-based *** formation mechanism of the A_(2)B_(7)-type phase as a result of the thermal diffusion process and the effect of Mg content on hydrogen storage performance were investigated.x-ray diffraction(xRD)patterns and Rietveld refinement results showed that increased Mg transformed the LaNi_(5)phase in the La_(0.74)Sm_(0.03)y_(0.23)Ni_(4.32)Al_(0.04)precursor alloy into a superlattice *** electron microscopy(SEM)images showed that Mg was evenly distributed in the alloy *** in the superlattice significantly inhibited the phase decomposition of the superlattice structure during the hydrogen absorption/desorption *** A_(2)B_(7)-type La_(0.57)Sm_(0.02)y_(0.18)Mg_(0.23)Ni_(3.38)Al_(0.03)alloy composed of Gd_(2)Co_(7)and Ce_(2)Ni_(7)phases was successfully *** pressure-composition isotherm profiles showed that the alloy had a hydrogen storage capacity as high as 1.73 wt%,with good cycling *** 50 cycles of hydrogen absorption/desorption,the alloy retained a hydrogen storage capacity of 1.45 wt%,with a capacity retention rate of up to 84.28%.The Mg thermal diffusion process thus provides a new approach for the controlled preparation of La-Mg-Ni-based alloys.

关键词： La-Mg-Ni-based hydrogen storage alloy A_(2)B_(7)-type superlattice alloy Mg thermal diffusion process Phase transformation Hydrogen storage property

Optimization of performance of the KM2A full array using the Crab Nebula

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Chinese Physics C 2024年第6期48卷 169-178页

作者：曹臻 F.Aharonian 安琪阿西克古白云翔包逸炜 D.Bastieri 毕效军毕玉江蔡金庭曹晴曹文羽曹喆常进常劲帆陈尚明陈恩生陈亮陈林陈龙陈明君陈玛丽陈起辉陈素弘陈天禄陈阳程宁程耀东崔明阳崔树旺崔晓红崔昱东戴本忠代洪亮戴子高单增罗布 D.della Volpe 董绪强段凯凯樊军辉范一中方军方堃冯存峰封莉冯少辉丰晓婷冯有亮 S.Gabici 高博高川东高林青高启高卫高伟康葛茂茂耿利斯 G.Giacinti 龚光华苟全补顾旻皓郭福来郭晓磊郭义庆郭莹莹韩毅昂何会海贺昊宁何佳银何新波何钰 M.Heller 贺远强侯博文侯超侯贤胡红波胡铨胡世聪黄代绘黄天奇黄文俊黄性涛黄晓渊黄勇黄志成季筱璐贾焕玉贾康江琨姜晓巍姜泽军金敏康明铭柯通 D.Kuleshov.K.Kurinov 李兵兵李澄李骢李丹李飞李海波李会财李华阳李军李剑李捷李凯李文龙李文莲李秀荣李昕李一卓李哲黎卓梁恩维梁云峰林苏杰刘冰刘成刘栋刘虎刘海东刘佳刘江来刘金艳刘茂元柳若愚刘四明刘伟刘怡刘以农鲁睿罗晴吕洪魁马伯强马玲玲马欣华毛基荣闵振 W.Mitthumsiri 穆慧君南云程 A.Neronov 区子维庞彬宇 P.Pattarakijwanich 裴致远齐孟尧祁业情乔冰强秦家军 D.Ruffolo A.Sáiz D.Semikoz 邵澄宇邵琅 O.Shchegolev 盛祥东舒富文宋慧超 yu.V.Stenkin V.Stepanov 苏扬孙秦宁孙晓娜孙志斌谭柏轩唐庆文唐泽波田文武王超王昌贝王广威王洪光王惠惠王建成汪凯王利苹王玲玉王培汉王冉王为王祥高王祥玉王阳王玉东王岩谨王忠海王仲翔王振王铮韦大明魏俊杰魏永健文韬吴超勇吴含荣武莎吴雪峰吴雨生席邵强夏捷夏君集项光漫肖迪泫肖刚辛广广辛玉良邢祎熊峥徐东莲徐仁峰徐仁新徐伟立薛良闫大海颜景志颜田杨朝文杨帆杨冯帆杨何文杨佳盈杨莉莉杨明洁杨睿智杨深邦姚玉华姚志国叶一锰尹丽巧尹娜游晓浩游智勇于艳红袁强岳华曾厚敦曾婷轩曾玮查敏张彬彬张丰张海明张恒英张建立张丽霞张力张鹏飞张佩佩张瑞张少博张少如张寿山张潇张笑鹏张云峰张毅张勇赵兵赵静赵雷赵立志赵世平郑福周斌周浩周佳能周猛周平周荣周勋秀祝成光祝凤荣朱辉朱科军左雄 LHAASO Collaboration Key Laboratory of Particle Astrophyics&Experimental Physics Division&Computing Center Institute of High Energy PhysicsChinese Academy of SciencesBeijing 100049China University of Chinese Academy of Sciences Beijing 100049China Tianfu Cosmic Ray Research Center Chengdu 610000China Dublin Institute for Advanced Studies 31 Fitzwilliam Place2 DublinIreland Max-Planck-Institut for Nuclear Physics P.O.Box 10398069029 HeidelbergGermany State Key Laboratory of Particle Detection and Electronics China University of Science and Technology of China Hefei 230026China School of Physical Science and Technology&School of Information Science and Technology Southwest Jiaotong UniversityChengdu 610031China School of Astronomy and Space Science Nanjing UniversityNanjing 210023China Center for Astrophysics Guangzhou UniversityGuangzhou 510006China Hebei Normal University Shijiazhuang 050024China Key Laboratory of Dark Matter and Space Astronomy&Key Laboratory of Radio Astronomy Purple Mountain ObservatoryChinese Academy of SciencesNanjing 210023China Tsung-Dao Lee Institute&School of Physics and Astronomy Shanghai Jiao Tong UniversityShanghai 200240China Key Laboratory for Research in Galaxies and Cosmology Shanghai Astronomical ObservatoryChinese Academy of SciencesShanghai 200030China Key Laboratory of Cosmic Rays(Tibet University) Ministry of EducationLhasa 850000TibetChina National Astronomical Observatories Chinese Academy of SciencesBeijing 100101China School of Physics and Astronomy(Zhuhai)&School of Physics(Guangzhou)&Sino-French Institute of Nuclear Engineering and Technology(Zhuhai) Sun Yat-sen UniversityZhuhai 519000&Guangzhou 510275China School of Physics and Astronomy Yunnan UniversityKunming 650091China Département de Physique Nucléaire et Corpusculaire Facultéde SciencesUniversitéde Genève24 Quai Ernest Ansermet1211 GenevaSwitzerland Institute of Frontier and Interdisciplinary Science Shandong UniversityQingdao 266237China APC Universit'e Paris Cit'eCNRS/IN2P3CEA/IRFUObservat

The full array of the Large High Altitude Air Shower Observatory(LHAASO)has been in operation since July *** its kilometer-square array(KM2A),we optimized the selection criteria for very high and ultrahigh energyγ-rays using data collected from August 2021 to August 2022,resulting in an improvement in significance of the detection in the Crab Nebula of approximately 15%,compared with that of previous *** the implementation of these new selection criteria,the angular resolution was also significantly improved by approximately 10%at tens of *** aspects of the full KM2A array performance,such as the pointing error,were also calibrated using the Crab *** resulting energy spectrum of the Crab Nebula in the energy range of 10-1000 TeV are well fitted by a log-parabola model,which is consistent with the previous results from LHAASO and other experiments.

关键词： γ-ray Crab Nebula significance