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Compound heterozygous mutation of AFG3L2 causes autosomal recessive spinocerebellar ataxia through mitochondrial impairment and MICU1 mediated Ca2+overload

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Science China(Life Sciences) 2025年第2期68卷 484-501页

作者： hongyu Li Qingwen Ma Yan Xue Linlin Cai Liwen Bao Lei hong Yitao Zeng Shu-Zhen huang Richard H.Finnell Fanyi Zeng Shanghai Institute of Medical Genetics Shanghai Children’s HospitalShanghai Jiao Tong University School of MedicineShanghai 200040China Department of Histo-Embryology Genetics and Developmental BiologyShanghai Jiao Tong University School of MedicineShanghai 200025China NHC Key Laboratory of Medical Embryogenesis and Developmental Molecular Biology Shanghai Key Laboratory of Embryo and Reproduction EngineeringShanghai 200040China Center for Precision Environmental Health Baylor College of MedicineHouston 77030USA School of Pharmacy Macao University of Science and TechnologyMacao 999078China

Autosomal recessive spinocerebellar ataxias(SCARs)are one of the most common neurodegenerative diseases characterized by progressive *** SCARs are known to be caused by mutations in multiple genes,there are still many cases that go undiagnosed or are *** this study,we presented a SCAR patient,and identified a probable novel pathogenic mutation(c.1A>G,p.M1V)in the AFG3L2 start *** 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 hi PSCs(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 ***,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.

关键词： AFG3L2 gene autosomal recessive spinocerebellar ataxia mitochondrial impairment Ca2+overload

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RGS22 maintains the physiological function of ependymal cells to prevent hydrocephalus

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Science China(Life Sciences) 2025年第2期68卷 441-453页

作者： Xue Pang Lin Gu Qiu-Ying Han Jia-Qing xing Ming Zhao Shao-Yi huang Jun-Xi Yi Jie Pan Hao hong Wen Xue Xue-Qing Zhou Zhi-Hui Su xin-Ran Zhang Li-Ming Sun Shao-Zhen Jiang Dan Luo Ling Chen Zheng-Jie Wang Yu Yu Tian Xia Xue-Min Zhang Ai-Ling Li Tao Zhou hong Cai Tao Li Nanhu Laboratory National Center of Biomedical AnalysisBeijing 100039China School of Basic Medical Sciences Fudan UniversityShanghai 200032China Department of Basic Medical Sciences School of MedicineTsinghua UniversityBeijing 100084China

Ependymal cells line the wall of cerebral ventricles and ensure the unidirectional cerebrospinal fluid(CSF)flow by beating their motile cilia *** ependymal denudation or ciliary dysfunction causes ***,we report that the deficiency of regulator of Gprotein signaling 22(RGS22)results in severe congenital hydrocephalus in both mice and ***,RGS22 is specifically expressed in ependymal cells within the *** conditional knock-out mice,we further demonstrate that the deletion of Rgs22 exclusively in nervous system is sufficient to induce ***,we show that Rgs22 deficiency leads to the ependymal denudation and impaired *** phenomenon can be attributed to the excessive activation of lysophosphatidic acid receptor(LPAR)signaling under Rgs22^(-/-)condition,as the LPAR blockade effectively alleviates hydrocephalus in Rgs22^(-/-)***,our findings unveil a previously unrecognized role of RGS22 in the central nervous system,and present RGS22 as a potential diagnostic and therapeutic target for hydrocephalus.

关键词： RGS22 hydrocephalus ependymal cells

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Regulating self-assembly of concomitant isotropic Ag_(78) and anisotropic Ag_(110)nanoclusters

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Science Bulletin 2025年第1期70卷 25-28页

作者： xin-Lan Peng Shuo huang Yu-Jing Zhao Xi-Ming Luo Hai-Yang Li Jia-Hua Hu Jia-hong huang Shuang-Quan Zang Henan Key Laboratory of Crystalline Molecular Functional Materials and College of Chemistry Zhengzhou UniversityZhengzhou 450001China

Owing to their inherently excellent geometric structure[1-3]and applications in catalysis,luminescence,and biology[4-6],as well as their importance in gaining insights into the embryonic period of metal nanoparticles,large-bulk(nuclearity>50)atomprecise Ag nanoclusters(NCs)[2,7]have garnered significant *** advancements in the use of different thiol precursors as low-activity sulfur sources and the selection of appropriate anions(such as polyoxometalates and tetrahedral anions)have contributed to the development of large-sized Ag-S NCs with excellent properties[2].

关键词： clusters anisotropic precise

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IL-17A mediates inflammation-related retinal pigment epithelial cells injury via ERK signaling pathway

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International Journal of Ophthalmology(English edition) 2025年第1期18卷 15-27页

作者： Hui-Min Zhong Bing-Qiao Shen Yu-hong Chen Xiao-Huan Zhao Xiao-Xu huang Min-Wen Zhou Xiao-Dong Sun Department of Ophthalmology Shanghai General Hospital(Shanghai First People’s Hospital)Shanghai Jiao Tong UniversitySchool of MedicineShanghai 200080China National Clinical Research Center for Ophthalmic Diseases Shanghai 200080China National Clinical Research Center for Eye Diseases Shanghai 200080China Shanghai Key Laboratory of Fundus Diseases Shanghai 200080China Shanghai Engineering Center for Visual Science and Photomedicine Shanghai 200080China

AIM:To investigate whether interleukin-17A(IL-17A)gets involved in the mechanisms of inflammation-related retinal pigment epithelium(RPE)cells injury and its significance in age-related macular degeneration(AMD).MRTHODS:A sodium iodate(NaIO3)mouse model as well as IL-17A-/-mice were *** effects of inflammatory cytokines in RPE cells and retinal microglia before and after NaIO3 modeling in vivo and in vitro,were investigated using immunofluorescence,immunoprotein blotting,and quantitative real-time fluorescence polymerase chain reaction(qRT-PCR),*** using recombinant IL-17A protein(rIL-17A)or IL-17A neutralizing antibody(IL-17A NAb)were used to observe the subsequent differences in fundus,fundus photography and optical coherence tomography(OCT),cell viability,and expression of oxidative stress-related markers before and after modeling,and to screen for key signaling ***:In the scenario of NaIO3 stimulation,RPE cells obviously tended to *** proliferation and activation of retinal microglia was confirmed in NaIO3-stimulated mice,whereas such effects induced by NaIO3 were significantly ameliorated with IL-17A NAb intervention or in IL-17A-/-*** addition,IL-17A promoted the proliferation and activation of microglia as well as oxidative damage and the secretion of inflammatory cytokines alongside NaIO3-induced damage in RPE cells in vivo and ex ***,the extracellular signalregulated kinase(ERK)signaling pathway was shown to be participated in the regulation of NaIO3-induced RPE cells injury mediated by IL-17A in vivo and ex vivo,as IL-17A induced inflammatory cytokines release in the NaIO3 model was alleviated after blocking the ERK ***:IL-17A probably promotes the NaIO3-induced RPE cells injury through exacerbating inflammation in terms of retinal microglia activation and inflammatory cytokines release via ERK signaling *** of IL-17A may be a new potential ta

关键词： age-related macular degeneration retinal pigment epithelium NaIO3 microglia IL-17A

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Effect of h-BN rods on high-temperature properties of alumina-carbon refractory

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Journal of Iron and Steel Research International 2025年第1期32卷 115-128页

作者： Ya-ge Li Shuai-bing Wu Feng Liang Zhong huang Tu Long Long Dong Jun-yi Lv hong-juan Duan Biao Wen Yong Liu Hai-jun Zhang The State Key Laboratory of Refractories and Metallurgy Wuhan University of Science and TechnologyWuhan430081HubeiChina Sinosteel Luoyang Institute of Refractories Research Co. Ltd.Luoyang471039HenanChina

h-BN rods modified low-carbon alumina-carbon(Al_(2)O_(3)-C)refractories were prepared,and the effect of h-BN rod addition on the high-temperature properties was investigated and compared with commercial h-BN flake,carbon black,and carbon nanotubes *** results demonstrated that Al_(2)O_(3)-C refractories containing h-BN rods exhibited optimal high-temperature service performances,including 25%higher hot modulus of rupture,21.3%higher thermal shock strength residual ratio,20.9%lower in oxidation and 44.3%less in slag corrosion,compared to the counterpart specimens without ***,benefiting from the synergistic enhancement of the rod-like h-BN and in-situ generated SiC whiskers,the high-temperature service performances of h-BN rods containing specimens outperformed counterpart specimens containing commercial h-BN flake,carbon black,and carbon nanotubes,respectively.

关键词： Al_(2)O_(3)-C refractory h-BN rod Microstructure High-temperature service performance

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Temporal and Spatial Heterogeneity and Its Influencing Factors of Carbon Surplus and Deficit at County-Level Areas in Shaanxi Province

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Huanjing Kexue/Environmental Science 2025年第3期46卷 1502-1516页

作者： Ding, Yi-Meng Han, Ling huang, xin School of Land Engineering Chang'an University Xi'an710054 China

Carbon source and sink monitoring is an important prerequisite for realizing the dual-carbon target and the evolution of its spatial-temporal pattern and the spatial-temporal heterogeneity of the driving factors are the scientific basis for the implementation of the emission reduction and sink enhancement policy according to the local conditions，which is of great importance for the sustainable development of the region. Based on the carbon balance of payment relationship，the carbon surplus and deficit of Shaanxi Province counties were calculated in 2000，2010，and 2020 from land use，and a series of exploratory spatial and temporal analysis methods（ESTDA），including spatial autocorrelation，cold and hot spot analysis，standard deviation ellipse，and LISA-time pathway，were used to study the dynamics of carbon surplus and deficit in Shaanxi Province at different spatial-temporal scales. From the 21 indicators，six types of major driving factors were selected by principal component analysis，and the geographical spatio-temporal weighted regression model（GTWR）was used to identify their spatio-temporal heterogeneity to construct a comprehensive system of indicators to analyze the carbon deficit and its spatio-temporal heterogeneity in Shaanxi Province. The results showed that：① A carbon surplus of 8.56 million tons in 2000，a carbon deficit of 3 296 tons in 2010，and a deficit of 33.34 million tons occurred in 2020 in Shaanxi Province，and the growth rate of carbon emissions was much larger than that of carbon sinks，which indicates that it was gradually moving towards carbon peaks in Shaanxi Province;however，there is a long way to go to achieve the goal of carbon neutrality. ② The geographical distribution of the"north deficit and south surplus"phenomenon was visually represented. A carbon deficit was concentrated in the wind and sand area along the Great Wall and Guanzhong plain. The spatial-temporal leap characteristics were more stable. In conclusion，efforts aimed at emission red

关键词： Anthropogenic

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Stretchable All-Small-Molecule Organic Solar Cells Enabled by Polymer Elastomer Confinement

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Chinese Journal of Polymer Science 2025年第2期43卷 271-277页

作者： Chen-Yi Zhang Yu-Qiang Liu hong-Xiang Li xin-Yue Cui Zheng-Dong Wei Yue-Heng Liu Ming-Hua Li An-Dong Zhang Pei Cheng Zhi-Shan Bo College of Textiles&Clothing State Key Laboratory of Bio-fibers and Eco-textilesQingdao UniversityQingdao266071China State Key Laboratory of Polymer Materials Engineering College of Polymer Science and EngineeringSichuan UniversityChengdu610065China College of Materials Science and Engineering Qingdao UniversityQingdao266071China Beijing Key Laboratory of Energy Conversion and Storage Materials College of ChemistryBeijing Normal UniversityBeijing100875China

Intrinsic stretchability is a promising attribute of polymer organic solar cells(OSCs).However,rigid molecular blocks typically exhibit poor tensile properties,rendering polymers vulnerable to mechanical *** this study,we introduce a different approach utilizing all-small-molecule donors and acceptors to fabricate stretchable *** elastomer,styrene-b-ethylene-butylene-styrene(SEBS),was embedded to modulate film crystallization and *** effectively confines the growth process of donors and acceptors,leading to enhancement of the crystallization quality,thus contributing to enhanced device ***,SEBS can absorb and release mechanical stress during stretching,thereby preventing mechanical degradation of donors and *** mechanical properties of the OSCs were significantly improved by the incorporation of ***,the crack-onset strain increased from 1.03% to 5.99% with SEBS *** findings present a straightforward strategy for achieving stretchable OSCs using all small molecules,offering a different perspective for realizing stretchable devices.

关键词： All-small-molecule Organic solar cells Elastomer Stretchability Flexibility

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Chinese Medicine for Treatment of COVID-19:A Review of Potential Pharmacological Components and Mechanisms

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Chinese Journal of Integrative Medicine 2025年第1期31卷 83-95页

作者： XU Qian-qian YU Dong-dong FAN Xiao-dan CUI He-rong DAI Qian-qian Zhong Xiao-ying ZHANG xin-yi ZHAO Chen YOU Liang-zhen SHANG hong-cai Key Laboratory of Chinese Internal Medicine of Ministry of Education Dongzhimen HospitalBeijing University of Chinese MedicineBeijing100700China The Geriatrics Center First Affiliated HospitalAnhui University of Chinese MedicineHefei230031China School of Medical Information Engineering Guangzhou University of Chinese MedicineGuangzhou51006China Institute of Basic Research in Clinical Medicine China Academy of Chinese Medical SciencesBeijing100700China

Coronavirus disease 2019(COVID-19)is an acute infectious respiratory disease that has been prevalent since December *** medicine(CM)has demonstrated its unique advantages in the fight against COVID-19 in the areas of disease prevention,improvement of clinical symptoms,and control of disease *** review summarized the relevant material components of CM in the treatment of COVID-19 by searching the relevant literature and reports on CM in the treatment of COVID-19 and combining with the physiological and pathological characteristics of the novel *** the basis of sorting out experimental methods in vivo and in vitro,the mechanism of herb action was further clarified in terms of inhibiting virus invasion and replication and improving related *** aim of the article is to explore the strengths and characteristics of CM in the treatment of COVID-19,and to provide a basis for the research and scientific,standardized treatment of COVID-19 with CM.

关键词： Chinese medicine COVID-19 SARS-CoV-2 substance component mechanism experimental method

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Quantitative analysis of influencing factors to aerosol pH and its responses to PM_(2.5)and O_(3)pollution in a coastal city

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Journal of Environmental Sciences 2025年第5期151卷 284-297页

作者： Ke Xu Liqian Yin Qiaoling Chen Dan Liao Xiaoting Ji Keran Zhang Yu Wu Lingling Xu Mengren Li Xiaolong Fan Fuwang Zhang Zhi huang Jinsheng Chen Youwei hong Center for Excellence in Regional Atmospheric Environment Key Lab of Urban Environment and HealthInstitute of Urban EnvironmentChinese Academy of SciencesXiamen 361021China School of Life Sciences Hebei UniversityBaoding 071000China University of Chinese Academy of Sciences Beijing 100049China College of Resources and Environment Fujian Agriculture and Forest UniversityFuzhou 350002China College of Chemical Engineering Huaqiao UniversityXiamen 361021China College of Environment and Public Health Xiamen Huaxia UniversityXiamen 361024China Fujian Key Laboratory of Atmospheric Ozone Pollution Prevention Institute of Urban EnvironmentChinese Academy of SciencesXiamen 361021China Environmental Monitoring Center of Fujian Fuzhou 350003China Xiamen Institute of Environmental Science Xiamen 361021China

Aerosol acidity(pH)plays an important role in the multiphase chemical processes of atmospheric *** this study,we demonstrated the seasonal trends of aerosol pH calculated with the ISORROPIA-II model in a coastal city of southeast *** performed quantitative analysis on the various influencing factors on aerosol pH,and explored the responses of aerosol pH to different PM_(2.5)and O_(3)pollution *** results showed that the average aerosol pH was 2.92±0.61,following the order of winter>spring>summer>*** tests revealed that SO_(4)^(2−),NH_(x),T and RH triggered the variations of aerosol *** analysis results showed that T(37.9%-51.2%)was the main factors affecting pH variations in four seasons,followed by SO_(4)^(2−)(6.1%-23.7%),NH_(x)(7.2%-22.2%)and RH(0–14.2%).Totally,annual mean meteorological factors(52.9%)and chemical compositions(41.3%)commonly contributed the aerosolpH in the coastal *** concentrations of PM_(2.5)was positively correlated with aerosol liquid water content(R^(2)=0.53)and aerosol pH(R^(2)=0.26),indicating that the increase in pH was related with the elevated NH_(4)NO_(3)and decreased SO_(4)^(2−),and also the changes of T and *** O_(x)(O_(3)+NO_(2))was moderately correlated with aerosol pH(R^(2)=-0.48),attributable to the fact that the proportion of SO_(4)^(2−)increased under high T and low RH *** study strengthened our understanding of the contributions of influencing factors to aerosol pH,and also provided scientific evidences for chemical processes of atmospheric particles in coastal areas.

关键词： Aerosol acidity Aerosol liquid water Fine particular matter(PM_(2.5)) Ozone(O_(3)) Coastal city

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Model and Data Hybrid Driven Approach for Quantifying the Meteorology-Dependent Demand Flexibility of Building Thermal Loads

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CSEE Journal of Power and Energy Systems 2025年第1期11卷 394-405页

作者： Bo Hu xin Cheng Changzheng Shao Tao Niu Chunyan Li Yue Sun Wei huang Kaigui Xie State Key Laboratory of Power Transmission Equipment and System Security Chongqing UniversityChongqing400030China

Recently, the heat and electricity integrated energy system (HE-IES) has become a hot topic in both industry and academia. In the HE- IES, the potential flexibility of the buildings' thermal loads can be exploited to relax the heat power balance constraints and consequently allow a more flexible operation of the combined heat and power units. In this paper, model-driven and data-driven techniques are combined to quantify the demand flexibility of the buildings' thermal loads in a non-instructive way. First, the explicit analytical equivalent thermal parameter (ETP) model of the aggregated buildings is developed. The heat transfer coefficient (k) and thermal inertia coefficient (C) of the ETP model are designated to measure the potential demand flexibility. Second, the Particle Swarm Optimization optimized Radial Basis Function neural network (PSO-RBF) is used to identify the relationship between the values of k and C and the meteorological factors. To obtain the training data, an innovative two-stage regression method based on the adaptive temporal resolution is proposed to extract k and C values from the historical thermal load data. Finally, a flexible thermal load model is built based on the predictions of the meteorological factors, which can be conveniently incorporated into the online dispatch of the HE-IES. A comprehensive simulation environment is designed to verify the accuracy and availability of the proposed technique.

关键词： Flexibility of buildings'thermal loads heat and electricity integrated energy system meteorological factors model and data hybrid driven.

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