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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

Enhancing Photocatalytic Hydrogen Evolution through Electronic Structure and Wettability Adjustment of ZnIn2S4/Bi2O3 S-Scheme Heterojunction

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物理化学学报 2024年第8期40卷 50-63页

作者： Linfeng Xiao Wanlu Ren Shishi Shen Mengshan Chen Runhua Liao Yingtang Zhou Xibao Li School of Materials Science and Engineering Jingdezhen Ceramic UniversityJingdezhen 333403Jiangxi ProvinceChina zhejiang Key laboratory of Petrochemical Environmental Pollution Control National Engineering Research Center for Marine AquacultureMarine Science and Technology CollegeZhejiang Ocean UniversityZhoushan 316022Zhejiang ProvinceChina School of Materials Science and Engineering Nanchang Hangkong UniversityNanchang 330063China

The production of renewable fuels through water splitting via photocatalytic hydrogen production holds significant ***,the sluggish kinetics of hydrogen evolution and the inadequate water adsorption on photocatalysts present notable *** this study,we have devised a straightforward hydrothermal method to synthesize Bi_(2)O_(3)(BO)derived from metal‐organic frameworks(MOFs),loaded with flower-like ZnIn_(2)S_(4)(ZIS).This approach substantially enhances water adsorption and surface catalytic reactions,resulting in a remarkable enhancement of photocatalytic *** employing triethanolamine(TEOA)as a sacrificial agent,the hydrogen evolution rate achieved with 15%(mass fraction)ZIS loading on BO reached an impressive value of 1610μmol∙h^(−1)∙g^(−1),marking a 6.34-fold increase compared to that observed for bare ***,through density functional theory(DFT)and ab initio molecular dynamics(AIMD)calculations,we have identified the reactions occurring at the ZIS/BO S-scheme heterojunction interface,including the identification of active sites for water adsorption and catalytic *** study provides valuable insights into the development of high-performance composite photocatalytic materials with tailored electronic properties and wettability.

关键词： S-scheme Hydrogen evolution Wettability Photocatalysis Electronic structure

Correlation of Oral Microbiota With Different Immune Responses to Antiretroviral Therapy in People Living With HIV

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Infectious Microbes & Diseases 2024年第2期6卷 85-92页

作者： Jingying Pan Xiaodi Zhang Danrong Shi Xuebin Tian Lijun Xu Xiangyun Lu Mingqing Dong Peng Yao Zhaoyi Pan Nanping Wu Hangping Yao State Key laboratory for Diagnosis and Treatment of Infectious Diseases National Clinical Research Center for Infectious DiseasesCollaborative Innovation Center for Diagnosis and Treatment of Infectious DiseasesThe First Affiliated HospitalSchool of MedicineZhejiang UniversityHangzhouChina Department of Infectious Disease Zhejiang Qingchun HospitalZhejiang UniversityHangzhouChina Jinan Microecological Biomedicine Shandong laboratory JinanChina

Both HIV infection and antiretroviral therapy(ART)affect the oral *** successful treatment with ART in people living with HIV(PLWH),which leads to a significant decline in viral loads and immune reconstitution,is associated with changes in or recovery of the oral microbiome remains ***,we performed a cross-sectional study of 118 PLWH receiving regular ART and 40 healthy controls(HCs).Among the 118 PLWH,18 immunological nonresponders(INRs;<200 CD4^(+)T cells/μL)and 30 immunological responders(IRs;≥500 CD4^(+)T cells/μL)were *** oral microbiota composition of all participants was analyzed using 16S rRNA gene sequencing of throat swab *** abundance of bacterial genera was compared between IRs and INRs,and Pearson correlations between bacterial abundance and peripheral blood immune cell counts were *** INR group showed lower alpha diversity than the IR and HC groups,which displayed similar alpha *** genera Alloprevotella,Prevotella and Neisseria were more abundant in PLWH than in HCs,whereas the genera Rothia,Streptococcus and Fusobacterium were more abundant in HCs than in *** genus Rothia was more abundant in the INR group,whereas Prevotella,Alloprevotella,Porphyromonas and Haemophilus were more abundant in the IR *** genera Rothia and Alloprevotella were negatively and positively associated with CD4^(+)T cell counts,***,an increased abundance of Rothia in the oral microbiome is associated with unfavorable outcomes regarding immune reconstitution in PLWH receiving regular ART,whereas Prevotella,Alloprevotella,Porphyromonas and Haemophilus are associated with favorable outcomes.

关键词： HIV oral microbiome antiretroviral therapy immunological nonresponders immunological responders

Comprehensive analysis of advanced glycation end-products in commonly consumed foods:presenting a database for dietary AGEs and associated exposure assessment

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Food Science and Human Wellness 2024年第4期13卷 1917-1928页

作者： Qiaozhi Zhang Huatao Li Ruixing Zheng Lili Cao Shufen Zhang Shuifeng Zhang Huadong Sheng Yuhao Jiang Yanbo Wang Linglin Fu Food Safety Key laboratory of zhejiang Province School of Food Science and BiotechnologyZhejiang Gongshang UniversityHangzhou 310018China Ningbo Academy of Product and Food Quality Inspection(Ningbo Fibre Inspection Institute) Ningbo 315048China National Pre-packaged Food Quality Supervision and Inspection center(zhejiang) Zhejiang Fangyuan Test Group Co.Ltd.Hangzhou 310018China

Advanced glycation end-products(AGEs)are a group of heterogeneous compounds formed in heatprocessed foods and are proven to be detrimental to human ***,there is no comprehensive database for AGEs in foods that covers the entire range of food categories,which limits the accurate risk assessment of dietary AGEs in human *** this study,we first established an isotope dilution UHPLCQq Q-MS/MS-based method for simultaneous quantification of 10 major AGEs in *** contents of these AGEs were detected in 334 foods covering all main groups consumed in Western and Chinese populations.Nε-Carboxymethyllysine,methylglyoxal-derived hydroimidazolone isomers,and glyoxal-derived hydroimidazolone-1 are predominant AGEs found in most *** amounts of AGEs were high in processed nuts,bakery products,and certain types of cereals and meats(>150 mg/kg),while low in dairy products,vegetables,fruits,and beverages(<40 mg/kg).Assessment of estimated daily intake implied that the contribution of food groups to daily AGE intake varied a lot under different eating patterns,and selection of high-AGE foods leads to up to a 2.7-fold higher intake of AGEs through daily *** presented AGE database allows accurate assessment of dietary exposure to these glycotoxins to explore their physiological impacts on human health.

关键词： Advanced glycation end-products Maillard reaction Processed foods Dietary database Exposure assessment

Causality Between Gut Microbiota and Inflammatory Bowel Disease:A Bidirectional Mendelian Randomization Study

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Infectious Microbes & Diseases 2024年第2期6卷 93-99页

作者： Qi Zheng Yuetong Li Jiali Ni Yong Huang Jiajun Wu Xiang Xu Guoping Sheng Lanjuan Li State Key laboratory for Diagnosis and Treatment of Infectious Diseases National Clinical Research Center for Infectious DiseasesNational Medical Center for Infectious DiseasesCollaborative Innovation Center for Diagnosis and Treatment of Infectious DiseasesThe First Affiliated HospitalZhejiang University School of MedicineHangzhouChina Key laboratory of Artificial Organs and Computational Medicine in zhejiang Province Shulan(Hangzhou)Hospital Affiliated to Shulan International Medical CollegeZhejiang Shuren UniversityHangzhouChina zhejiang Chinese Medical University HangzhouChina

The exact relationship between inflammatory bowel disease(IBD)and gut microbiota(GM)is still *** study aimed to explore the cause-and-effect relationship between IBD and GM by Mendelian randomization *** IBD data used in this study were obtained from Genome-Wide Association *** GM data were from the Dutch Microbiome Project and included 207 taxa and 205 microbiota-associated *** Mendelian randomization analysis was performed to investigate the relationship between GM and *** results demonstrated that susceptibility to developing IBD is negatively correlated with class Clostridia(OR=0.80,P=0.003),family Sutterellacea(OR=0.87,P=0.014),genus Coprobacter(OR=0.90,P=0.009),order Clostridiales(OR=0.80,P=0.003),phylum Firmicutes(OR=0.82,P=0.002)and Coprobacter fastidiosus(OR=0.90,P=0.009).Conversely,the risk of developing IBD was positively correlated with the phosphopantothenate biosynthesis I pathway(OR=1.17,P=0.006),Bacteroides caccae(OR=1.14,P=0.021)and Bacteroides uniformis(OR=1.26,P=0.003).Reverse causality was found between the phosphopantothenate biosynthesis I pathway and IBD(OR=1.03,P=0.04),but not for the remaining specific GM *** conclusion,our study demonstrated a causal relationship between specific GM features and IBD.

关键词： gut microbiota inflammatory bowel disease Mendelian randomization

MSC-derived exosomes attenuate hepatic fibrosis in primary sclerosing cholangitis through inhibition of Th17 differentiation

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Asian Journal of Pharmaceutical Sciences 2024年第1期19卷 119-134页

作者： Wenyi Chen Feiyan Lin Xudong Feng Qigu Yao Yingduo Yu Feiqiong Gao Jiahang Zhou Qiaoling Pan Jian Wu Jinfeng Yang Jiong Yu Hongcui Cao Lanjuan Li State Key laboratory for the Diagnosis and Treatment of Infectious Diseases National Clinical Research Center for Infectious DiseasesCollaborative Innovation Center for Diagnosis and Treatment of Infectious DiseasesNational Medical Center for Infectious DiseasesThe First Affiliated HospitalZhejiang University School of MedicineHangzhou 310003China Jinan Microecological Biomedicine Shandong laboratory Jinan 250117China Key laboratory of Diagnosis and Treatment of Aging and Physic-chemical Injury Diseases of zhejiang Province Hangzhou 310003China

Primary sclerosing cholangitis(PSC)is an autoimmune cholangiopathy characterized by chronic inflammation of the biliary epithelium and periductal fibrosis,with no curative treatment available,and liver transplantation is inevitable for end-stage *** placentalmesenchymal stem cell(hpMSC)-derived exosomes have demonstrated the ability to prevent fibrosis,inhibit collagen production and possess immunomodulatory properties in autoimmune liver ***,we prepared hpMSC-derived exosomes(Exo^(MSC))and further investigated the anti-fibrotic effects and detailed mechanism on PSC based on Mdr2^(−/−)mice and multicellular organoids established from PSC *** results showed that Exo^(MSC) ameliorated liver fibrosis in Mdr2^(−/−)mice with significant collagen reduction in the preductal area where Th17 differentiation was inhibited as demonstrated by RNAseq analysis,and the percentage of CD4+IL-17A+T cells was reduced both in Exo^(MSC)-treated Mdr2^(−/−)mice(Mdr2^(−/−)-Exo)in vivo and Exo^(MSC)-treated Th17 differentiation progressed in ***,Exo^(MSC) improved the hypersecretory phenotype and intercellular interactions in the hepatic Th17 microenvironment by regulating PERK/CHOP signaling as supported by multicellular ***,our data demonstrate the antifibrosis effect of Exo^(MSC) in PSC disease by inhibiting Th17 differentiation,and ameliorating the Th17-induced microenvironment,indicating the promising potential therapeutic role of Exo^(MSC) in liver fibrosis of PSC or Th17-related diseases.

关键词： Mesenchymal stem cell Exosomes Primary sclerosing cholangitis Fibrosis Organoids Th17

Energy-absorbing porous materials:Bioinspired architecture and fabrication

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Nano Research 2024年第2期17卷 679-690页

作者： Junheng Zhao Meng Li Jiewei Chen Weiwei Gao Hao Bai State Key laboratory of Chemical Engineering College of Chemical and Biological EngineeringZhejiang UniversityHangzhou 310058China Department of Polymer Science and Engineering Zhejiang UniversityHangzhou 310058China

Energy-absorbing materials are widely used in transportations,sports,and the military ***,porous materials,including natural and artificial materials,have attracted tremendous attentions due to their light weight and excellent energy absorption *** review summarizes the recent progresses in the natural and artificial energy-absorbing porous ***,we review the typical natural porous materials including cuttlebone,bighorn sheep horn,pomelo peel,and sunflower stem *** architectures,energy absorption abilities,and mechanisms of these typical natural materials and their bioinspired materials are ***,we provide a review on the fabrication methods of artificial energy-absorbing porous materials,such as conventional foaming and three-dimensional(3D)***,we address the challenges and prospects for the future development of energy-absorbing porous *** importantly,our review provides a direct guidance for the design and fabrication of energy-absorbing porous materials required for various engineering applications.

关键词： energy-absorbing porous materials natural materials natural structures bioinspired materials

Mechanism study of Cu(Ⅱ) adsorption from acidic wastewater by ultrasonic-modified municipal solid waste incineration fly ash

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Chinese Journal of Chemical Engineering 2024年第3期67卷 157-165页

作者： Shunda lin Yang Lu Lin Zheng Ling Long Xuguang Jiang Jianhua Yan State Key laboratory of Clean Energy Utilization Institute for Thermal Power EngineeringZhejiang UniversityHangzhou 310027China zhejiang University Qingshanhu Energy Research center Hangzhou 310027China Hangzhou Xiaoshan Jinjiang Green Energy Co. Ltd.XiaoshanHangzhou 310000China

High concentrations of copper ions(Cu(Ⅱ)) in water will pose health risks to humans and the ecological environment. Therefore, this study aims to utilize ultrasonic-cured modified municipal solid waste incineration(MSWI) fly ash for Cu(Ⅱ) adsorption to achieve the purpose of “treating waste by waste.” The effects of p H, adsorption time, initial concentration, and temperature on the modified MSWI fly ash’s adsorption efficiency were systematically studied in this article. The adsorption performance of the modified MSWI fly ash can be enhanced by the ultrasonic modification. At pH = 2, 3 and 4, the adsorption capacity of the modified MSWI fly ash for Cu(Ⅱ) increased by 2.7, 1.9 and 1.2 times, respectively. Furthermore, it was suggested that the adsorption process of the modified MSWI fly ash can be better simulated by the pseudo-second-order kinetic model, with a maximum adsorption capacity calculated by the Langmuir model of 24.196 mg.g-1. Additionally, the adsorption process is spontaneous,endothermic, and chemisorption-dominated from the thermodynamic studies(ΔH and ΔS > 0, ΔG < 0).Finally, the enhanced adsorption performance of the modified MSWI fly ash for Cu(Ⅱ) may be attributed to electrostatic interaction and chelation effects.

关键词： Ultrasound MSWI fly ash Cu(Ⅱ) Wastewater Adsorption

Enhanced thermal conductivity and mechanical properties of 2D C_(f)/SiC composites modified by in-situ grown carbon nanotubes

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Journal of Advanced Ceramics 2024年第1期13卷 19-33页

作者： Changhui Niu Qing Zhang Laifei Cheng Fang Ye Litong Zhang Min Huang Tao Yang Science and Technology on Thermostructure Composite Materials laboratory Northwestern Polytechnical UniversityXi’an 710072China Civil Aviation Department Zhejiang Institute of CommunicationsHangzhou 311112China zhejiang Provincial Key laboratory for Cutting Tools Taizhou UniversityTaizhou 318000China

In this work,the effects of carbon nanotubes(CNTs)on the microstructure evolution,thermal conductivity,and mechanical properties of C_(f)/SiC composites during chemical vapor infiltration(CVI)densification were investigated in *** with composites without CNTs,the thermal conductivity,flexural strength,flexural modulus,fracture toughness,interfacial shear strength,and proportional limit stress of specimens with CNTs of 4.94 wt%were improved by 117%,21.8%,67.4%,10.3%,36.4%,and 71.1%,*** improvement was attributed to the role of CNTs in the division of inter-layer pores,which provided abundant vapor growth sites for the ceramic matrix and promoted densification of the whole *** addition,the high thermal conductivity network formed by the overlap of CNTs and the rivet strengthening effect of CNTs were beneficial for synergistic improvement of thermal conductivity and mechanical properties of the ***,this study has practical significance for the development of thermal protection composite components with enhanced thermal conductivity and mechanical characteristics.

关键词： ceramic−matrix composites carbon nanotubes microstructure thermal conductivity mechanical properties