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Compound heterozygous mutation of AfG3L2 causes autosomal recessive spinocerebellar ataxia through mitochondrial impairment and MICU1 mediated Caoverload

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Science China Life Sciences 2024年

作者： Hongyu Li Qingwen Ma Yan Xue Linlin Cai Liwen Bao Lei Hong Yitao zeng Shuzhen Huang Richard H.finnell fanyi zeng Shanghai Institute of Medical Genetics Shanghai Children's Hospital Shanghai Jiao Tong University School of Medicine Department of Histo-Embryology Genetics and Developmental Biology Shanghai Jiao Tong University School of Medicine NHC Key Laboratory of Medical Embryogenesis and Developmental Molecular Biology Shanghai Key Laboratory of Embryo and Reproduction Engineering Center for Precision Environmental Health Baylor College of Medicine School of Pharmacy Macau University of Science and Technology

Autosomal recessive spinocerebellar ataxias（SCARs） are one of the most common neurodegenerative diseases characterized by progressive ataxia. Although SCARs are known to be caused by mutations in multiple genes, there are still many cases that go undiagnosed or are misdiagnosed. In this study, we presented a SCAR patient, and identified a probable novel pathogenic mutation（c.1A>G, p.M1V） in the AfG3L2 start codon. the proband's genotype included heterozygous mutations of the compound AfG3L2(p.[M1V]; [R632X]（c.[1A>G];[1894.C>t]）), which were inherited from the father（c.1A>G, p.M1V） and mother（c.1894C>t, p.R632X）. functional studies performed on hiPSCs（human induced pluripotent stem cells） generated from the patients and HEK293t cells showed that the mutations impair mitochondrial function and the unbalanced expression of AfG3L2 mRNA and protein levels. furthermore, this novel mutation resulted in the degradation of the protein and the reduction of the stability of the AfG3L2 protein, and MCU（mitochondrial calcium uniporter） complex mediated Ca2+overload.

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Simultaneous enhancement of energy storage performance and thermal stability of NaNbO_(3)-based ceramics via multi-scale modulation

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Journal of Materiomics 2024年第3期10卷 670-681页

作者： Peng Nong Dafu zeng Yue Pan Qinpeng Dong Mingzhao Xu Xiang Wang Jiaming Wang Huanfu Zhou Xu Li Xiuli Chen Key Laboratory of Nonferrous Materials and New Processing technology Ministry of EducationSchool of Materials Science and EngineeringGuilin University of TechnologyGuilin541004China

More and more attention is being paid to the environmental friendliness of the dielectric capacitors,which have a rapid charging and discharging speed and steady ***,the low recoverable energy storage density(W_(rec))limits the practical application of dielectric capacitors in solid-state energy storage *** this paper,(1-x)(0.92NaNbO_(3)-0.08Bi(Mg_(0.5)ti_(0.5)O_(3))-xSrtiO_(3))(x=0.05,0.10,0.15,0.20)energy storage ceramics were fabricated based on various strategies such as nanodomain engineering,component and grain size *** excellent energy storage performance(W_(rec)~6.0 J/cm^(3),η~81.0%)was obtained in ***_(3)could enhance the internal disorder,improve the relaxation properties and increase the breakdown field strength of ***-cantly domain relaxor behavior,as evidenced by Vogel-fulcher(V-f)model,provides strong evidence for restraining early polarization *** addition,Pulse charge/discharge tests have illustrated that the sample has excellent and stable charge/discharge performance(CD~1,068.9 A/cm^(2),PD~96.1 MW/cm^(3),t0.9 z 0.99 ms)It is concluded that there is a huge prospect of NN-BMt-0.15St ceramics as a kind of pulsed-power-storing electronics.

关键词： NaNbO_(3) Breakdown field strength Lead-free ceramics Energy storage Power density

t6态ZL105-(Pr+Ce)合金的组织及力学性能研究

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热加工工艺 2024年第10期53卷 143-147页

作者：曾敏陈晖南昌工学院新能源车辆学院江西南昌330108

采用光学显微镜、扫描电子显微镜、EDS分析仪、布氏硬度与拉伸试验研究了混合稀土与t6热处理对ZL105合金的微观组织及力学性能的影响。结果表明,当添加0.5wt%的稀土(Pr+Ce)后,ZL105合金的α-Al相、共晶Si相及富fe相均被细化。ZL105-0.5w... 详细信息

采用光学显微镜、扫描电子显微镜、EDS分析仪、布氏硬度与拉伸试验研究了混合稀土与t6热处理对ZL105合金的微观组织及力学性能的影响。结果表明,当添加0.5wt%的稀土(Pr+Ce)后,ZL105合金的α-Al相、共晶Si相及富fe相均被细化。ZL105-0.5wt%(Pr+Ce)合金经t6热处理后,α-Al相进一步细化,晶粒趋于圆整,共晶Si相也进一步细化,由长条状转变为短颗粒状,但是富fe相的形貌和尺寸没有发生明显变化。t6态ZL105-0.5wt%(Pr+Ce)合金的抗拉强度、伸长率及硬度分别为257.7 MPa、7.0%、98 HB。t6态ZL105-0.5wt%(Pr+Ce)合金的断口为典型的塑性断裂特征,断口表面出现了许多韧窝,主要以小韧窝为主,分布较均匀且密集。t6热处理后合金的力学性能得到了显著改善。

关键词： ZL105-(Pr+Ce)铝合金 t6热处理微观组织力学性能

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Beta-lactam antibiotic resistance among Escherichia coli isolates from patients with urinary tract infections in Vietnam:A systematic review and meta-analysis

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Asian Pacific Journal of tropical Medicine 2024年第6期17卷 243-255,I0001-I0003页

作者： Dat t Nguyen Princess Margaret Cancer Centre University Health NetworkTorontoOntarioCanada

Objective:to investigate the evolving patterns of antimicrobial resistance against beta-lactam antibiotics in Escherichia coli isolates from urinary tract infection patients in Vietnam,covering the period from 2005 to ***:29 Descriptive studies published between 2010 and 2022 in English and Vietnamese were included in the *** on resistance rates to beta-lactam antibiotics,including cephalosporins and carbapenems,were extracted and *** pooled resistance rates were calculated using random-effects *** trends in resistance were assessed using linear regression ***:Among the beta-lactam antibiotics studied,Escherichia coli exhibited varying levels of resistance,with cephalosporins showing higher resistance rates compared to *** pooled resistance rates were 66%for cefotaxime,65%for ceftriaxone,54%for ceftazidime,and 56%for *** contrast,carbapenems demonstrated lower resistance rates,with weighted pooled resistance rates ranging from 3%to 4%for meropenem,ertapenem,and *** rates were also observed for amoxicillin/clavulanate(35%),ticarcillin/clavulanate(26%),and piperacillin/tazobactam(12%).Significant annual increases in resistance were noted for imipenem(0.56%,Pthis study highlights the critical issue of antimicrobial resistance in urinary tract infections in Vietnam and emphasizes the importance of prudent antibiotic use and the regular monitoring of resistance *** insights are useful for guiding healthcare professionals in optimizing treatment strategies and for policymakers in formulating evidence-based clinical guidelines to combat antibiotic resistance effectively.

关键词： Antimicrobial resistance Urinary tract infection Vietnam

Impact of metabolic dysfunction-associated steatotic liver disease on the efficacy of immunotherapy in patients with chronic hepatitis B-related hepatocellular carcinoma

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Cancer Biology & Medicine 2024年第9期21卷 813-825页

作者： Jiaxin Han Wentao Kuai Liu Yang Xuemei taol Yuekui Wang Minghui zeng' Yuqin Lil Yuqiang Mi Ningning Zhang Wei Lu Liang Xu Clinical School of the tianjin Second People's Hospital Tianjin Medical UniversityTianjin 300192China Department of Hepatology Tianjin Second People's HospitalTianjin 300192China tianjin Institute of Hepatology Tianjin 300192China Department of Hepatobiliary Oncology Liver Cancer CenterTianjin Medical University Cancer Institute&HospitalNational Clinical Research Center for CancerKey Laboratory of Cancer Prevention and TherapyTianjinTianjin's Clinical Research Centerfor CancerTianjin300060China

Objective:to investigate the impact of metabolic dysfunction-associated steatotic liver disease(MASLD)on the efficacy of immune checkpoint inhibitor(ICI)-based therapy in patients with chronic hepatitis B(CHB)-related hepatocellular carcinoma(HCC).Methods:A total of 155 patients with CHB-related HCC who received ICI–based therapy(in the Department of Hepatology,tianjin Second People’s Hospital and Department of Hepatobiliary Oncology,tianjin Medical University Cancer Institute&Hospital)between April 2021 and December 2023 were *** were divided into two groups:MASLD concurrent with CHB[MASLD-CHB](n=38),and CHB(n=117).Results:the median progression-free survival(PfS,6.9 months vs.9.3 months;P=0.001),progressive disease(57.89%vs.37.61%;P=0.028),and disease control rate(42.11%vs.62.39%;P=0.028)in the MASLD-CHB group were significantly worse than the CHB *** median overall survival was not *** percentage of CD4+PD1+(17.56%vs.8.89%;Pt cells(10.50%vs.7.42%;P=0.005)in patient samples from the MASLD-CHB group were significantly higher than the CHB *** MASLD[hazard ratio(HR)=1.921;95%CI,1.138–3.245;P=0.015]and alpha-fetoprotein levels after 3 months of treatment(HR=2.412;95%CI,1.360–4.279;P=0.003)were independent risk factors for PfS in all ***:ICI-based therapy in patients with CHB-related HCC and concurrent MASLD resulted in poorer efficacy and shorter PfS compared to patients with CHB-related HCC alone.

关键词： Metabolic dysfunction-associated steatotic liver disease chronic hepatitis B hepatocellular carcinoma immunotherapy efficacy

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Development of red phosphor Li_(8)CaLa_(2)ta_(2)O_(13):Eu^(3+)for WLEDs and anti-counterfeiting applications

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ChemPhysMater

ChemPhysMater 2024年第2期3卷 194-203页

作者： Jingjing Ru fan zeng Bing Zhao Chenqing Ye tonghui Zhong feiyun Guo Jianzhong Chen College of Chemistry and Materials Fujian Province University Key Laboratory of Green Energy and Environment CatalysisNingde Normal UniversityNingde 352100China School of Environment and Resources School of Carbon Neutral and Modern IndustryFujian Normal UniversityFuzhou 350007China College of Information and Mechanical&Electrical Engineering Ningde Normal UniversityNingde 352100China College of Chemistry Fuzhou UniversityFuzhou 350108China

A set of novel red phosphors Li_(8)CaLa_(2)ta_(2)O_(13):Eu^(3+)(LCLtO:xEu^(3+))were successfully prepared using a solid-state reaction *** properties of the prepared samples,including phase purity,elemental composition,and morphology,were systematically investigated using X-ray diffraction,scanning electron microscopy,and diffuse reflectance spectroscopy *** 610 nm maximum emission peak is attributed to the^(5)D_(0)→^(7)f_(2)transition of Eu^(3+)ion under 394 nm *** all the LCLtO:xEu^(3+)phosphors,LCLtO:0.6Eu^(3+)showed the strongest emission intensity because of the concentration quenching effect of the electric dipole-dipole in-teraction among the Eu^(3+)ions,which was also demonstrated by the decay ***,the emission intensity of the optimal LCLtO:0.6Eu^(3+)phosphor,which exhibited a high internal quantum efficiency of 49.30%and excellent color purity of 96.79%,was approximately 2.29 times higher than that of commercial Y203:Eu^(3+)red *** thermal stability of the LCLtO:0.6Eu^(3+)sample with good color stability was meticulously *** fabricated white-light-emitting diode(WLED)exhibited a superior color-rendering index of Ra=82 and chromaticity coordinates of(0.3260,0.3639),suggesting that LCLtO:0.6Eu^(3+)has potential applicability in developing efficient and high-quality ***,the prepared LCLtO:0.6Eu^(3+)/PDMS composite film demonstrated exceptional flexural resistance and chemical stability,indicating considerable promise for practical anti-counterfeitingapplications.

关键词： Phosphor tantalate Luminescence LED device

Body composition and metabolic syndrome in patients with type 1 diabetes

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World Journal of Diabetes 2024年第1期15卷 81-91页

作者： Qiong zeng Xiao-Jing Chen Yi-ting He Ze-Ming Ma Yi-Xi Wu Kun Lin Department of Neurology The First Affiliated Hospital of Shantou University Medical CollegeShantou 515041Guangdong ProvinceChina Medical College Shantou University Medical CollegeShantou 515041Guangdong ProvinceChina Department of Endocrinology The First Affiliated Hospital of Shantou University Medical CollegeShantou 515041Guangdong ProvinceChina

BACKGROUND In recent years,the prevalence of obesity and metabolic syndrome in type 1 diabetes(t1DM)patients has gradually *** resistance in t1DM deserves *** is necessary to clarify the relationship between body composition,metabolic syndrome and insulin resistance in t1DM to guide clinical treatment and *** to assess body composition(BC)in t1DM patients and evaluate the relationship between BC,metabolic syndrome(MS),and insulin resistance in these *** A total of 101 subjects with t1DM,aged 10 years or older,and with a disease duration of over 1 year were *** impedance analysis using the tsinghua-tongfang BC Analyzer BCA-1B was employed to measure various BC *** and laboratory data were collected,and insulin resistance was calculated using the estimated glucose disposal rate(eGDR).RESULtS MS was diagnosed in 16/101 patients(15.84%),overweight in 16/101 patients(15.84%),obesity in 4/101(3.96%),hypertension in 34/101(33.66%%)and dyslip-idemia in 16/101 patients(15.84%).Visceral fat index(VfI)and trunk fat mass were significantly and negatively correlated with eGDR(both Pfemale patients exhibited higher body fat percentage and visceral fat ratio compared to male *** logistic regression analysis revealed that significant factors for MS included eGDR[P=0.017,odds ratio(OR)=0.109],VfI(P=0.030,OR=3.529),and a family history of diabetes(P=0.004,OR=0.228).Significant factors for hypertension included eGDR(Ptal muscle mass(P=0.003,OR=1.111).Significant factors for dyslipidemia included trunk fat mass(P=0.033,OR=1.202)and eGDR(P=0.037,OR=0.708).CONCLUSION Visceral fat was found to be a superior predictor of MS compared to conventional measures such as body mass index and waist-to-hip ratio in Chinese individuals with *** analysis,specifically identifying visceral fat(trunk fat),may play an important role in identifying the increased risk of

关键词： Body composition Metabolic syndrome Insulin resistance Visceral fat Estimated glucose disposal rate

A novel strategy for ionomer coating of Ag nanoparticles used for the electrochemical reduction of CO2 to CO in a membrane electrode assembly

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National Science Review 2024年第2期11卷 94-95页

作者： Alexis t.Bell Department of Chemical and Biomolecular Engineering University of California

the electrochemical reduction of the CO2reaction （CO2RR） offers an attractive means for converting the carbon content of CO2, released from a stationary source or the atmosphere, into fuels and chemicals [1]. Utiliz...

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A deep neural network based surrogate model for damage identification in full-scale structures with incomplete noisy measurements

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frontiers of Structural and Civil Engineering 2024年第3期18卷 393-410页

作者： tram BUI-NGOC Duy-Khuong LY tam t tRUONG Chanachai tHONGCHOM t.NGUYEN-tHOI Laboratory for Computational Mechanics Institute for Computational Science and Artificial IntelligenceVan Lang UniversityHo Chi Minh City 70000Vietnam faculty of Mechanical-Electrical and Computer Engineering School of TechnologyVan Lang UniversityHo Chi Minh City 70000Vietnam faculty of Civil Engineering School of TechnologyVan Lang UniversityHo Chi Minh City 70000Vietnam Department of Computer Science Aarhus UniversityAarhus 8000Denmark thammasat University research unit in structural and foundation engineering Department of Civil EngineeringThammasat UniversityPathumthani 12120Thailand Laboratory for Applied and Industrial Mathematics Institute for Computational Science and Artificial IntelligenceVan Lang UniversityHo Chi Minh City 70000Vietnam thammasat School of Engineering Thammasat UniversityPathumthani 12120Thailand

the paper introduces a novel approach for detecting structural damage in full-scale structures using surrogate models generated from incomplete modal data and deep neural networks(DNNs).A significant challenge in this field is the limited availability of measurement data for full-scale structures,which is addressed in this paper by generating data sets using a reduced finite element(fE)model constructed by SAP2000 software and the MAtLAB programming *** surrogate models are trained using response data obtained from the monitored structure through a limited number of measurement *** proposed approach involves training a single surrogate model that can quickly predict the location and severity of damage for all potential *** achieve the most generalized surrogate model,the study explores different types of layers and hyperparameters of the training algorithm and employs state-of-the-art techniques to avoid overfitting and to accelerate the training *** approach’s effectiveness,efficiency,and applicability are demonstrated by two numerical *** study also verifies the robustness of the proposed approach on data sets with sparse and noisy measured ***,the proposed approach is a promising alternative to traditional approaches that rely on fE model updating and optimization algorithms,which can be computationally *** approach also shows potential for broader applications in structural damage detection.

关键词： vibration-based damage detection deep neural network full-scale structures finite element model updating noisy incomplete modal data